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Hematology Flashcards

1
Q

HML Embryology:
When does the liver make itself the major site of hematopoiesis?

A

Week 9

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2
Q

HML Embryology:
How long does the spleen remain exclusively a hematopoietic organ?
What occurs after that? (2 points)

A
  • 14 weeks
  • 15-18 weeks: T cell precursors
  • 23 weeks: B cell precursors enter. the spleen and form B- cell regions
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3
Q

HML Embryology:
Why does hemoglobin exhibit a sigmoid shape on the Oxygen dissociation curve?

A

Because it’s an allosteric molecule and exhibits positive cooperatively. Allowing for efficient loading and unloading of oxygen.

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4
Q

HML Embryology:
What causes a right shift in the Hb dissociation curve?

A

Elevation in H+, CO2, 2,3-bisphosphoglycerate, and temperature. Favoring unloading.

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5
Q

HML Embryology:
What is difference between the T and R form of Hb?

A
  • T (taut) is the deoxygenated form and has a lower affinity for oxygen to bind.
  • R (relaxed) is the oxygenated form and has 300x more affinity
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6
Q

HML Embryology:
Where is fetal hemoglobin made? What is made up of and why is it important?

A
  • Made in the fetal liver
  • made up of two alpha and 2 gamma chains
  • has higher affinity to oxygen than adult Hb.
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7
Q

HML Embryology:
When does the transition of fetal Hb to adult Hb complete?

A

6 months of age

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8
Q

HML Embryology:
What is the importance in the relationship between fetal Hb and 2,3-BPG?

A

Fetal Hb does not bind to 2,3-BPG, resulting in an increased affinity for oxygen.

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9
Q

HML Anatomy:
How much of the total blood volume is made up of plasma?

A

55%, blood cells (RBCs, WBCs, and platelets) occupy the rest of the 45%
Rich in proteins, hormones, electrolytes, and small molecules

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10
Q

HML Anatomy:
What is serum?

A

Plasma without the clotting factors.

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11
Q

HML Anatomy:
What is the effect of corticosteroids on polymorphonuclear neutrophil (PMN) migration?

A
  • inhibit PMN from the circulation into the periphery
  • causing benign leukocytosis. They also cause apoptosis of lymphocytes and sequestration of eosinophils in lymph nodes.
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12
Q

HML Anatomy:
Do RBCs utilize aerobic metabolism?

A

No

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13
Q

HML Anatomy:
How does the RBC utilize energy?

A

Source = glucose
90% anaerobically resulting into lactate,
10% hexose monophosphate HMP shunt to produce NADPH (reduced)

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14
Q

HML Anatomy:
What accounts for 0.5-1.5% of RBCs?

A

Reticulocytes

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15
Q

HML Anatomy:
What is normal range of WBCs?

A

4,000 to 11,000/úL

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16
Q

HML Anatomy:
What is the prevalence of the different WBC?

A

Mneumonic: ‘Never Let Monkeys Eat Bananas
- Neutrophils (54-62%)
- Lymphocytes (25-33%
- Monocytes (3-7%)
- Eosinophils (1-3%)
- Basophils (0-0.75%)

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17
Q

HML Anatomy:
How can you determine if an anemia is hypo/hyperproliferative?

A
  • Corrected HCT (hematocrit)
    = HCT/45; <2% then hypo, >3% is hyper
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18
Q

HML Anatomy:
What are the two types of abnormal neutrophils?

A

Immature (bands) and hypersegmented

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19
Q

HML Anatomy:
What conditions would you see “band” neutrophils?

A

Bacterial infections, leukemias, and other inflammatory conditions
- neutrophils are horseshoe shaped

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20
Q

HML Anatomy:
What conditions would you see hypersegmented neutrophils?

A

Macrocytic anemias associated with Vitamin B12 and folate deficiencies
- Neutrophils with more than five lobes

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21
Q

HML Anatomy:
What does hematocrit mean?

A

Represents the percentage of whole-blood volume composed of erythrocytes

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22
Q

HML Anatomy:
What does mean cell hemoglobin mean?

A

The average content of hemoglobin per RBC

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23
Q

HML Anatomy:
What does MCHC mean?

A
  • Mean corpuscular hemoglobin concentration
  • The average concentration of hemoglobin in a given volume of packed RBCs. The MCHC is low if the RBCs are hypochromic
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24
Q

HML Anatomy:
What does RDW mean?

A
  • RBC distribution width
  • The coefficient of variation of RBC volume. An increased RDW means that the RBCs vary greatly in size.
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25
Q

HML Anatomy:
What is the importance of lactoferrin in neutrophils?

A

Binds to iron so bacteria cannot utilize leading to bacterial death

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26
Q

HML Anatomy:
What are the five chemostatic agents in neutrophils?

A

C5a, IL-8, LTB4, kallikrein, and platelet-activation factor

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27
Q

HML Anatomy:
Where do B lymphocytes migrate towards after maturing in the bone marrow? 3

A

Peripheral lymphoid tissues (lymph node follicles, white pulp of spleen, and unencapsulated lymphoid tissue)

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28
Q

HML Anatomy:
Which immunoglobulins do B lymphocytes need to recognize first before plasma cell differentiation and antibody production?

A

IgM and IgD

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29
Q

HML Anatomy:
What do B lymphocytes function as?
3 points

A

Memory cells, APCs, and express MHC class II

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30
Q

HML Anatomy:
What are the CD markers for B lymphocytes?

A

CD19, CD20, CD21

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31
Q

HML Anatomy:
What T lymphocytes differentiate to?

A
  • Cytotoxic T cells
  • Helper T cells
  • Suppressor T cells
  • delayed hypersensitivity T cells
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32
Q

HML Anatomy:
Describe the mechanism how a T cells get activated:

A

First binds the antigen to the MHC complex, then sends a co-stimulatory signal via CD28/CD40L.

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33
Q

HML Anatomy:
Describe how you identify a plasma cell under a microscope:

A

Eccentric cells with purple “clock-faced” nuclei, cytoplasm has abundant blue rough endoplasmic reticulum and well developed Golgi apparatus.

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34
Q

HML Anatomy:
Which lymphocyte can mediate both adaptive and innate immunity responses?

A

Natural Killer cells (NK cells)

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35
Q

HML Anatomy:
What are the CD markers for NK Cells?

A

CD16 and CD56

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36
Q

HML Anatomy:
What is contained in the cytoplasm of the NK cell?

A
  • Small granules filled with perforin and granzyme
  • induces apoptosis to help target and kill cells lacking MCH I, including tumor-derived cells and cells infected with viruses.
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37
Q

HML Pathology:
Describe Leukocyte adhesion deficiency (LAD):

A

An autosomal recessive defect of integrant (CD18 subunits), resulting in delayed separation of the umbilical cord, increased circulating PMN leukocytes, recurrent bacterial infections (lack of pus formation), severe gingivitis, and poor wound healing.

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38
Q

HML Anatomy:
What are the five common causes of eosinophilia?

A

Mneumonic NAACP:
- Neoplasia
- Asthma
- Allergic processes
- Collagen vascular diseases
- Parasites

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39
Q

HML Anatomy:
What is the shape of the nucleus of a Monocyte?

A

Kidney shaped

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40
Q

HML Anatomy:
What is contained in the cytoplasm of the monocyte?

A

Azurphilic granules (lysosomes) and appear basophilic with a “frosted glass appearance.

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41
Q

HML Anatomy:
What accounts for 2-10% of all leukocytes?

A

Monocytes, they are the precursors for macrophages and APCs

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42
Q

HML Anatomy:
Describe how an eosinophil looks under a microscope:

A

Bilobed cell with large eosinophilic granules.

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43
Q

HML Anatomy:
What percentage of leukocytes do eosinophils comprise of?

A

1-6%

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44
Q

HML Anatomy:
How do eosinophils defend against parasitic infections?

A

Major basic protein

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45
Q

HML Anatomy:
Name the chemical mediators released by eosinophils:

A

Leukotrienes, prostaglandins E1 and E2, thromboxane B2, and platelet-activating factor

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46
Q

HML Anatomy:
How do eosinophils down regulate allergic reactions?

A

Histaminase inactivate the basophil-derived substrate histamine

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47
Q

HML Anatomy:
Which leukocyte has a causative role in allergic reactions, are bilobed, and have deep basophilic granules?

A

Basophils

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48
Q

HML Anatomy:
What percentage of leukocytes do basophils account for?

A

<0.5%

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49
Q

HML Anatomy:
What receptor is activated on basophils?
What is released?
What conditions would you see this in?

A
  • IgE receptors
  • release of histamine, heparin, prostaglandins, leukotrienes, and other vasoactive amines.
  • Seen in asthma, hay fever, and elevated in myeloproliferative diseases.
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50
Q

HML Anatomy:
Which Leukocyte is abundant near blood vessels and in tissue exposed to the external environment (eg skin, respiratory, GI, urogenital)?

A

Mast cells

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51
Q

HML Anatomy:
Which leukocyte is similar to basophils, express IgE receptors and counter parasitic infections and chronic allergic diseases?

A

Mast cells

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52
Q

HML Anatomy:
Describe the three ways Mast cells can be activated:

A
  1. Cross-linking of cell surface IgE by antigen
  2. Complement C3a/C5a
  3. Tissue trauma: Histamine (from immediate response) vs leukotrienes (delayed response)
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53
Q

HML Pharmacology:
Describe the action and clinical use for Cromolyn sodium:

A
  • Prevents mast cell degranulation, used for asthma prophylaxis.
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54
Q

HML Pharmacology:
Describe the action and clinical use for Omalizumab:

A

Inhibits IgE binding to mast cells. Used for severe allergic asthma.

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55
Q

HML Anatomy:
How would you describe a macrophage under a microscope?

A

Oval nuclei and blue-gray to pale cytoplasm

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56
Q

HML Anatomy:
Which leukocytes serve as tissue scavengers, are phagocytic, consumes bacteria, ages RBCs, cell debris, and has an APC property?

A

Macrophages

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57
Q

HML Anatomy:
How are macrophages activated?

A

Gamma interferon

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58
Q

HML Anatomy:
Describe four different derivatives of macrophages in different organs and tissues:

A
  • Kupffer cells in the liver
  • Microglial cells in the brain
  • Osteoclasts in the bone
  • Mesangial cells in the kidney (derived from monocytes)
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59
Q

HML Anatomy:
Which Leukocytes are the sentinels, adjuvants, and controllers of both innate and adaptive immunities?

A

Dendritic cells

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60
Q

HML Anatomy:
How do dendritic cells serve as APCs?

A

Expressing major histocompatibility complex (MHC) II and crystallizable fragment (Fc) receptors on their surface.

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61
Q

HML Anatomy:
Which leukocytes are the main inducers of the primary antibody response?

A

Dendritic Cells

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62
Q

HML Anatomy:
What are dendritic cells of the skin called?

A

Langerhans cells

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63
Q

HML Pathology:
Describe the relevant signs and symptoms for an anemia caused by hypoxia:

A

General weakness or fatigue, dyspnea or angina on exertion, or syncope, pale conjunctiva or skin, and koilonychias (spoon shaped nails)

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64
Q

HML Pathology:
Describe the relavant signs and symptoms for an anemia caused by an increased cardiac output:

A

Tachycardia or palpitation, systolic murmur, or high-output heart failure.

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65
Q

HML Pathology:
List the 5 categories that can cause a microcytic anemia:

A

Mneumonic: TAILS
1. Iron deficiency (late)
2. ACD (late)
3. Thalassemias
4. Lead poisoning
5. Sideroblastic anemia (copper deficiency can also cause this)

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66
Q

HML Pathology:
List the 4 circumstances where you would see a normocytic-nonhemolytic (reticulocyte count normal/decreased) anemia?

A
  1. Iron deficiency (early)
  2. ACD (Early)
  3. Aplastic Anemia
  4. Chronic Kidney disease
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67
Q

HML Pathology:
List the 5 circumstances where you would see a normocytic-hemolytic (reticulocyte count increase) anemia in an intrinsic matter:

A
  1. RBC membrane defect: hereditary spherocytsosis
  2. RBC enzyme deficiency: G6PD, pyruvate kinase
  3. HbC disease
  4. Paroxysmal nocturnal hemoglobinuria
  5. Sickle cell anemia
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68
Q

HML Pathology:
List the 4 circumstances where you see a normocytic-hemolytic (reticulocyte count increase) anemia in an extrinsic matter:

A

Mneumonic: AIIMM
1. Autoimmune
2. Microangiopathic
3. Macroangiopathic
4. Infections

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69
Q

HML Pathology:
List the 3 circumstances where you see a macrocytic-megaloblastic anemia:

A
  1. Folate deficiency
  2. B12 deficiency
  3. Orotic aciduria
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70
Q

HML Pathology:
List the 3 circumstances where see a macrocytic-nonmegaloblastic anemia:

A
  1. Liver disease
  2. Alcoholism
  3. Diamond-Blackfan anemia
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71
Q

HML Pathology:
List 2 common conditions affiliated with acanthocytes: RBCs with spiny processes protruding from cell surface

A

Abetalipoproteinemia and liver disease

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72
Q

HML Pathology:
List 2 common conditions affiliated with basophilic stipping: RBCs with numerous small purplish dots

A

Lead poisoning, thalassemias

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73
Q

HML Pathology:
What is a common associated condition relating with Dacrocyte? (“teardrop cell”)

A

Myelofibrosis

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74
Q

HML Pathology:
What is a common condition associated with Degmacyte? (“bite cell”)

A

Glucose-6-phosphate dehydrogenase deficiency

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75
Q

HML Pathology:
List 2 common conditions associated with Elliptocytes:

A

Hereditary elliptocytosis and Iron deficiency

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76
Q

HML Pathology:
What is a common condition associated with Heinz body?

A

Glucose-6-phosphate dehydrogenase deficiency

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77
Q

HML Pathology:
List 2 common conditions associated with Howell-Jolly bodies:

A

Asplenia and functional hyposplenia

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78
Q

HML Pathology:
What is a common condition associated with macro-ovalocyte?

A

Megaloblastic anemia

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79
Q

HML Pathology:
What is a common condition associated with ringed sideroblast?

A

Lead poisoning

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80
Q

HML Pathology:
What is a common conditions associated with schistocyte?
2 points

A
  1. Disseminated intravascular coagulation
  2. Thrombotic thrombocytopenia purpura
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81
Q

HML Pathology:
List 2 common condition associated with Spherocytes:

A
  1. Hereditary spherocytosis
  2. Autoimmune hemolysis
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82
Q

HML Pathology:
List 4 common condition associated with Target cells:
4 points

A

(HALT mneumonic)
1. Thalassemia
2. Liver disease
3. Hemoglobin C disease
4. Aspenia

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83
Q

HML Pathology:
List 4 common causes of GI bleeding:

A
  1. Endometrial polyps in adult female
  2. Peptic ulcer in adult male
  3. Colon polyps/carcinoma in elderly
  4. Hookworm in developing countries (Ancylostoma Duodenale, Necator Americanus)
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84
Q

HML Pathology:
How can Iron deficiency be caused? 3 categories

A
  1. Increased requirement: Pregnancy, infants, and preadolescents
  2. Dietary deficiency: Exclusively breast-fed infants after 6 months of age, elderly
  3. Chronic blood loss: Menorrhagia, gastrointestinal bleeding
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85
Q

HML Pathology:
Describe the triad for Plummer-Vinson syndrome:

A
  1. Upper esophageal web (dysphagia)
  2. Iron deficiency
  3. Atrophic glossitis
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86
Q

HML Pathology:
How can you diagnose an Iron deficiency anemia?

A
  1. Decreased hemoglobin and hematocrit
  2. Decreased serum iron
  3. Increased total iron-binding capacity (TIBC)
  4. Decreased ferritin
  5. PBS will show microcytic, hypochromic RBCs
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87
Q

HML Pathology:
What are 4 common country population are Thalassemias among?

A
  1. African
  2. Indian
  3. Southeast Asian
  4. Mediterranean
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88
Q

HML Pathology:
How many types of thalassemias are there?

A

2; alpha and beta

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89
Q

HML Pathology:
What is the underlying mechanism that makes beta-thalassemia develop?

A

Point mutation in either the promoter region or the splicing sites on chromosome 11, forming a premature stop or reduced production.

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90
Q

HML Pathology:
What is an alternate name for beta-thalessemia?

A

Mediterranean/ Cooley anemia

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91
Q

HML Pathology:
How many different forms of beta-thalassemia are there?

A

3; Minor (Beta/Beta+), Major (B0/B0), HbS/Beta-thalassemia

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92
Q

HML Pathology:
Describe beta thalassemia minor:

A

Heterozygote, clinically asymptomatic, lab studies show elevated HbA2

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93
Q

HML Pathology:
Describe beta thalassemia major:

A

Homozygote with absent Beta globin chain, resulting in severe anemia

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94
Q

HML Pathology:
Describe HbS/Beta-thalassemia:

A
  • Combo of sickle cell and beta-thalassemia
  • Mc in USA and Mediterrannean
  • Mild to moderate presentation depending on Beta global production
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95
Q

HML Pathology:
What does alpha-aggregation mean?

A
  • clumping of alpha-globin chains in beta-thalassemia major (lacks HbA)
  • leads to decreased RBC life span, apoptosis of its precursors, then ineffective erythropoeisis
  • Fetal Hb in increased but not adequate
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96
Q

HML Pathology:
How does beta thalassemia present: 3 S’s

A
  • anemia: severe anemia months after birth
  • splenomegaly: extra medullary hematopoiesis (increase EPO)
  • Skeletal deformities: thinning of cortical bone nan peripheral new bone formation, “crew cut” and “chipmunk face”
  • HemoSiderosis: iron overload from repeated transfusions
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97
Q

HML Pathology:
What are patients with beta thalassemia at increased risk for?

A

Aplastic crisis due to parvovirus B19 (ssDNA non enveloped, dx: fecal testing)

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98
Q

HML Pathology:
How do you diagnose and treat beta-thalassemia?

A

Dx:
- Hb electrophoresis
- PBS: Target cells, microcytosis, and hypochromic
Tx:
- blood transfusions (increased risk for secondary hemochromatosis

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99
Q

HML Pathology:
What is the prognosis for beta-thalassemia?
4 points

A
  • growth retardation
  • death at an early age
  • cardiac failure
  • other organ damage from hemosiderosis
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100
Q

HML Pathology:
What is the mechanism behind the development of alpha-thalassemia?

A
  • gene mutation or deletion in one or more of the four alpha-globing genes on chromosome 16
  • relative excess of other chains including beta, gamma, and delta
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101
Q

HML Pathology:
How does alpha-thalassemia present, in the silent carrier state?

A

Only one alpha globin gene affected; asymptomatic

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102
Q

HML Pathology:
How does alpha-thalassemia present, with the alpha-thalassemia trait?

A
  • two genes deleted
  • similar to beta-thalassemia minor with minimal anemia
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103
Q

HML Pathology:
How does alpha-thalassemia present, in the HbH state?

A
  • three genes affected
  • tetramers of beta-globin chains form which have a high affinity for oxygen, and also damages RBCs, leading to hemolysis, anemia disproportionate to the amount of Hb
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104
Q

HML Pathology:
How does alpha-thalassemia present, in the Hb Bart state?

A
  • deletion of all four genes
  • stable tetramers of gamma-globin chains form with extremely high affinity for oxygen
  • severe anemia
  • leads to intrauterine death (Hydrops Fetalis) unless intrauterine transfusion is performed
  • fetus shows edema, pallor, and hepatosplenomegaly
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105
Q

HML Pathology:
How would you diagnose alpha-thalassemia?

A
  • Hb Electrophoresis
  • PBS: microcytic and hypochromic
  • molecular genetic testing showing alpha-thalassemia traits
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106
Q

HML Pathology:
What is worse for alpha-thalassemia trait: the cis deletion or the trans?

A
  • Cis deletion on same chromosome is worse, common in asian populations, there is an increased risk of spontaneous abortion and severe thalassemia in offspring
  • Trans deletion occurs on separate chromosomes, common in African American populations. No increased risk.
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107
Q

HML Pathology:
What two enzymes are inhibited when exposed to lead?

A
  1. ALAD: aminolevulinic acid dehydratase
  2. Ferrochelatase
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108
Q

HML Pathology:
What occupations or settings are we concerned about lead poisoning?
3 points

A
  • Miners
  • Industrial workers (exposure to lead batteries)
  • houses built on or before 1978
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109
Q

HML Pathology:
How does lead poisoning present?

A
  • abdominal pain
  • peripheral neuropathy (eg wrist and foot drop)
  • encephalopathy
  • characteristic Burton lines on gingiva and metaphases of long bone
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110
Q

HML Pathology:
How would you diagnose lead poisoning?

A
  • PBS: basophilic stippling (aggregated rRNA)
  • hypochromic microcytic anemia or sideroblastic anemia
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111
Q

HML Pharmacology:
How would you treat lead poisoning?

A
  • Children: Succimer
  • Dimercarprol and ethylenediaminetetraacetic acid (EDTA)
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112
Q

HML Pathology:
Describe sideroblastic anemia:

A
  • defective protoporphyrin synthesis
  • genetic (congenital defects of aminolevulinic acid synthetase (ALAS)
  • X-linked
  • acquired causes (eg, alcoholism, lead poisoning, and vitamin B6 deficiency)
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113
Q

HML Pathology:
How do you diagnose sideroblastic anemia?

A
  • special staining of bone marrow aspirate for iron showing ringed sideroblasts (trapped iron in mitochondria)
  • Lab studies: increased ferritin, decreased TIBC, increased serum iron saturation
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114
Q

HML Pharmacology:
How do you treat sideroblastic anemia?

A

Pyridoxine (vitamin B6) cofactor for Aminolevulinic acid synthase (ALAS)

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115
Q

HML Pathology:
What is the pathophysiology underlying megaloblastic anemia?

A

Deficiency in vitamin B12 or folate (coenzymes in DNA synthesis) leads to delayed DNA replication, cytoplasmic maturation is normal

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116
Q

HML Pathology:
What are some causes of nonmegaloblastic, macrocytic anemia?
4 points

A

Mneumonic: HAAL
- Alcoholism
- liver disease
- hypothyroidism
- anticancer drugs (5-FU)

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117
Q

HML Pathology:
How can vitamin B12 deficiency be caused?

A
  • Decreased intake (vegans)
  • Impaired absorption (PPIs, pancreatic insufficiency, pernicious anemia, gastrectomy, malabsorption, ill resection, Diphyllobothrium datum /fish tapeworm infection, blind loop syndrome, broad spectrum antibiotics
  • Increased requirement (pregnancy, hyperthyroidism)
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118
Q

HML Pathology:
How can folic acid deficiency be caused?
5 points

A
  • Decreased intake (alcoholics and elderly)
  • impaired absorption (sprue, phenytoin, oral contraceptives)
  • increased loss (hemodialysis)
  • increased requirement (pregnancy, infancy, increased hematopoiesis)
  • folic acid antagonist chemotherapy (methotrexate)
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119
Q

HML Pathology:
Describe subacute combined degeneration:

A

Vitamin B12 deficiency leads to demyelination of the dorsal and lateral columns of the spinal cord. Results in ataxia (spinocerebellar tract), hyperreflexia (lateral corticospinal tract), and decreased potion and vibration sensation (dorsal column)

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120
Q

HML Pathology:
How does a megaloblastic anemia present?

A

Anemia, glossitis, and
subacute combine degeneration (vitamin 12, impaired myelination form elevated toxic methylmalonic acid buildup)

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121
Q

HML Pathology:
If you have a child pt with a megaloblastic anemia that is not responding to folate or vitamin B12 supplementation, what should you suspect?

A

Orotic aciduria:
- a genetic mutation in uridine monophosphate synthase (converts orotic acid to uridine in pyrimidine synthesis). Orotic acid in the urine.
- Tx: Uridine monophosphate

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122
Q

HML Pathology:
How would you diagnose and treat a megaloblastic anemia?

A
  • PBS: pancytopenia, oval macrocytosis, and hyperhsegmented neutrophils (> 5 lobes)
  • Bone marrow: megaloblastic hyperplasia
  • Labs for folate deficiency: Decreased serum folate, elevated homocysteine, and normal methylmalonic acid
  • Labs for vitamin b12 deficiency: decreased vitamin b12, elevated homocysteine, and increased methylmalonic acid
  • Tx: Vitamin B12 with or without Folate
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123
Q

HML Pathology:
How does a pernicious anemia develop?

A

anti-intrinsic factor antibodies

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124
Q

HML Pathology:
What is a Schilling test used for?
5 points

A

To confirm/correct a vitamin b12 deficiency caused by either:
1. Impaired absorption: oral Vitamin B12 after saturation via IM
2. Pernicious anemia: oral Vitamin B12 + IF
3. Bacterial overgrowth: B12 + antibiotics
4. Pancreatic insufficiency: B12 + pancreatic enzymes (R-binder)

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125
Q

HML Pathology:
What is the inheritance pattern of Glucose-6-phosphate dehydrogenase deficiency?

A

XLR, leads to abnormally folded enzyme subjected to proteolysis.

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126
Q

HML Pathology:
What ethnic populations is G6PD deficiencies more prevalent in?
3 points

A

Black, Middle Eastern/ Mediterranean populations

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127
Q

HML Pathology:
What kind of hemolysis is seen in G6PD deficiency?

A

Both intravascular and extravascular

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128
Q

HML Pathology:
How does G6PD deficiency present? 5 points

A
  • often symptomatic
  • hx of neonatal jaundice and cholelithiasis
  • episodic fatigue, pallor, hemoglobinuria, and back pain (Hb Nephrotoxicity) days after oxidant stress
  • PE shows jaundice and splenomegaly
  • Hemolysis from exposure to oxidative stress
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129
Q

HML Pathology:
How does hemolysis occur in G6PD deficiency? 3 categories

A
  • exposure to oxidative stress from:
    – Drugs: Sulfonamides, dapsone, primaquine, chloroquine, and nitrofurantoin, etc.
    – Infections: viral hepatitis, typhoid fever, pneumonia
    – Others: Fava beans
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130
Q

HML Pathology:
How would you diagnosis G6PD deficiency? 6 points

A
  • Measure G6PD enzyme activity after resolved hemolytic episodes
  • increased indirect bilirubin, decreased serum haptoglobin
  • decreased hematocrit and hemoglobinemia in CBC
  • Heinz bodies and bite cells (splenic removal)
  • Hemoglobinuria in urinalysis
  • splenomegaly and gallstones in abdominal US (increased indirect bilirubin)
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131
Q

HML Pathology:
How do you treat G6PD deficiency? 4 points

A
  • avoid precipitating factors
  • provide oxygen and rest during episodes
  • exchange transfusions if severe
  • phototherapy in infants
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132
Q

HML Pathology:
What is the prognosis in G6PD deficiency?

A
  • healthy if avoiding predicating factors
  • complicated by neonatal jaundice leading to kernicterus
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133
Q

HML Pathology:
Describe Chediak-Higashi syndrome: 3 points

A
  • autosomal recessive
  • lysosomal trafficking defect characterized by impaired phagolysosome formation
  • results in increased pyogenic infection, neutropenia, albinism, defective primary hemostasis, and peripheral neuropathy
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134
Q

HML Pathology:
Describe Chronic Granulomatous disease: 4 points

A
  • XLR inheritance
  • defect in NADPH
  • Poor O2-dependent killing, resulting in recurrent infection and granuloma formation with catalase-positive organisms.
  • Nitroblue tetrazolium test result is negative
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135
Q

HML Pathology:
What is the inheritance pattern of Hereditary Spherocytosis?

A

Autosomal dominant

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136
Q

HML Pathology:
What is deficient in Hereditary Spherocytosis?

A
  • Spectrin, ankyrin, and other membrane tethering cytoskeletal proteins (vertically) like band 3, and protein 4.2, decreasing RBC fragility
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137
Q

HML Pathology:
What is the difference between Hereditary spherocytosis and hereditary elliptocytosis?

A

Hereditary elliptocytosis has a defect in tethering proteins involving horizontal interactions (spectral, protein 4.1, and glycophorin)

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138
Q

HML Pathology:
How does Hereditary Spherocytosis present? 4 points

A
  • anemia, splenomegaly, and jaundice
  • predominantly extravascular hemolysis
  • may develop cholelithiasis (bilirubin gallstones)
  • positive family history
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139
Q

HML Pathology:
How is hereditary spherocytosis diagnosed? 4 points
What are the differentials? 4 points

A
  • RBC lysis in hypotonic salt (osmotic fragility test)
  • Increase MCHC (pathognomonic, but also seen in cold-agglutinin anemia) due to dehydration
  • PBS: Spherocytes
  • minimal anemia with reticulocytosis in CBC, and increased indirect bilirubin
  • DDX includes anemia, biliary disease, hyperbilirubinemia, and AIHA (also have spherocytes)
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140
Q

HML Pathology:
How do you treat hereditary spherocytosis? 4 points

A
  • splenectomy
  • transfusions if severe
  • phototherapy in infants
  • supplemental folic acid and iron for increased RBC turnover
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141
Q

HML Pathology:
What is the prognosis of Hereditary Spherocytosis? 4 points

A
  • aplastic crisis triggered by parvovirus B19
  • infections after splenectomy
  • cholelithiasis (bilirubin gallstones)
  • hemosiderosis from multiple blood transfusions
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142
Q

HML Pathology:
Describe Myeloperoxidase deficiency disease:

A
  • results from defective conversion from H2O2 to HOCl, leading to an increased risk for Candida infections. However, most patients remain asymptomatic (vs CGD)
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143
Q

HML Pathology:
What are the three types of immunohemolytic anemias?

A
  • Warm antibody
  • Cold agglutinin
  • erythroblastosis fetalis
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144
Q

HML Pathology:
What is the mnemonic to distinguish between Warm antibody vs Cold agglutinin?

A
  • Warm is GGGreat (IgG)
  • Cold ice cream MMM (IgM)
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145
Q

HML Pathology:
What is the shape and consequence of the RBCs in Warm Antibody Hemolytic anemia?

A
  • Spherocytes
  • Sequestered then phagocytose in the spleen, a form of extravascular hemolysis
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146
Q

HML Pathology:
How does Warm antibody hemolytic anemia present? 3 points

A
  • elevated bilirubin (jaundice, pigment gallstones)
  • reticulocytosis
  • splenomegaly
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147
Q

HML Pathology:
What conditions are associated with warm antibody hemolytic anemia? 4 points

A

Memory Aid: Rule of L’s
- SLE
- Hodgkin lymphoma
- chronic lymphocytic leukemia (CLL)
- Drugs: alpha-methyldopa, PCN, cephalosporins

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148
Q

HML Pathology:
How do you diagnose warm antibody hemolytic anemia? 2 points

A
  • spherocytosis
  • positive direct Coombs test
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149
Q

HML Pathology:
How do you treat warm antibody hemolytic anemia? 3 points

A
  • drug cessation
  • steroids
  • splenectomy if necessary
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150
Q

HML Pathology:
Where is intravascular hemolysis seen in Cold Agglutinin IHA?

A

Distal body parts

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151
Q

HML Pathology:
How does extravascular hemolysis happen in Cold Agglutinin IHA?

A

IgM is released when the cells are warm, leaving C3b bound to the membrane

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152
Q

HML Pathology:
How does Cold Agglutinin IHA present? 5 points

A
  • Episodic hyperbilirubinemia: hemoglobinemia and hemoglinuria
  • positive Coombs test
  • elevated C3
  • Acute: in recovery phase after infectious Mononucleosis or mycoplasma pneumonia
  • Chronic: associated with lymphoproliferative neoplasms; may be associated with Raynaud phenomenon due to vascular obstruction
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153
Q

HML Pathology:
How is cold agglutinin IHA treated? 3 points

A
  • steroids
  • IVIG
  • plasmapheresis
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154
Q

HML Pathology:
What two settings can Erythroblastosis Fetales occur?

A
  • Mother is Rh- gives birth to Rh+ baby
  • ABO incompatibility: mother is type O and baby is type A or B
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155
Q

HML Pathology:
How does Erythroblastosis Fetalis present?

A

Fetal hemolytic anemia

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156
Q

HML Pathology:
How do you diagnose Erythoblastosis Fetalis?

A

Test maternal and fetal blood for presence of antibodies

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157
Q

HML Pathology:
What is the prognosis for Erythroblastosis fetalis? 3 points

A
  • Stillbirth
  • hydrops fetalis (fetal heart failure)
  • kernicterus
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158
Q

HML Pathology:
What is kernicterus?

A

Unconjugated bilirubin damaging the basal ganglia and other CNS structures, leading to neurological damage

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159
Q

HML Pathology:
How do you prevent Erythroblastosis fetalis?

A

Treat mother with anti-D Ig (RhoGAM) during and after each pregnancy

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160
Q

HML Pathology:
How is Anemia of Chronic Disease characterized? 2 points

A
  • marrow hypoproliferation as a result of impaired responsiveness to EPO
  • impaired iron utilization
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161
Q

HML Pathology:
What are the common causes of Anemia of Chronic Disease? 4 points

A
  • Chronic infections: Osteomyelitis and bacterial endocarditis, etc
  • Chronic immune disorders: rheumatoid arthritis, SLE
  • Renal disease
  • Neoplasms: Hodgkin disease, lung carcinoma, etc
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162
Q

HML Pathology:
What are the mechanisms behind anemia of chronic disease? 2 points

A
  • Increased hepcidin (produced from liver) limits iron release from macrophage to erythroid precursors and suppresses EPO release
  • Increased hepcidin sequesters iron to keep it away from microorganisms
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163
Q

HML Physiology:
What is the role of Hepcidin?

A

Master regulator in iron metabolism, normally limits iron transport by binding to ferroportin (iron transporter present in intestinal mucosal cells and macrophages)

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164
Q

HML Pathology:
How does Anemia of chronic disease present?

A

Symptoms of the chronic disease along with mild anemia (fatigue, pallor)

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165
Q

HML Pathology:
How do you diagnose Anemia of chronic disease? 6 points

A
  • decreased TIBC
  • decreased serum iron
  • increase serum ferritin (iron stores in macrophages
  • nornocytic, normochromic RBCs in the early stage
  • Microcytic and hypochromic RBCs in the late stage
  • Decrease hemoglobin and hematocrit in CBC
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166
Q

HML Pathology:
How do you treat an anemia of chronic disease? 2 points

A
  • administer EPO
  • treat underlying condition
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167
Q

HML Pathology:
What are three ways an intrinsic hemolytic anemia can develop?

A
  • complement fixation
  • mechanical injury
  • or toxins
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168
Q

HML Pathology:
What do you look for in an intravascular hemolytic anemia? 8 points

A
  • hemoglobinemia, methemalbuminemia
  • mild jaundice/ elevated unconjugated bilirubin
  • hemoglobinuria (early stage)
  • hemosiderinuria
    methemoglobinuria
  • decreased serum haptoglobin
  • hemosiderosis of renal tubules (chronic stage)
  • increased fecal urobilin
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169
Q

HML Pathology:
What causes an extravascular hemolytic anemia?
4 points

A
  • Due to RBC injury
  • antibody attachment
  • structural or membrane defects leading to abnormal shape
  • decreased ability to leave cords of Billroth (red pulp) and enter sinusoids of spleen
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170
Q

HML Pathology:
Where does hemolysis usually occur in extravascular hemolytic anemia?

A

Inside of mononuclear phagocytic cells in the spleen (RES)

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171
Q

HML Pathology:
What do you look for in extravascular hemolytic anemia? 4 points

A
  • jaundice/ elevation in unconjugated bilirubin (increased risk of bilirubin gallstones_
  • some decrease in haptoglobin
  • Splenomegaly
  • Minimal hemoglobinemia and hemoglobinuria
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172
Q

HML Pathology:
What are some hereditary circumstances that cause an intrinsic hemolytic anemia, that is categorized and intravascular and extravascular?
2 points

A

Enzyme deficiencies:
- HMP shunt: G6PD, glutathione synthetase
- Glycolytic enzymes: pyruvate kinase, hexokinase

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173
Q

HML Pathology:
What are some hereditary circumstances that cause an intrinsic hemolytic anemia, that is categorized as purely extravascular? 2 categories

A

Membrane disorders:
- elliptocytosis
- spherocytosis
Hemoglobin synthesis derangements:
- Thalassemias
- Sickle cell anemia (can also be Intravascular)

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174
Q

HML Pathology:
What are some acquired circumstances that cause an intrinsic hemolytic anemia, that is categorized as purely extravascular?

A
  • membrane disorders (EV)
  • Paroxysmal nocturnal hemoglobinuria
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175
Q

HML Pathology:
What are some acquired circumstances that cause an extrinsic hemolytic anemia, that is categorized as purely intravascular? 4 categories

A

Antibody mediated
- transfusions reactions, erythroblastosis fetalis, SLE, malignancies, mycoplasma infection, mononucleosis, drugs, idiopathic

Mechanical injury:
- MAHA: TTP (thrombotic thrombocytopenia purpura), DIC (disseminated intravascular coagulation
- Cardiac traumatic hemolytic anemia

Infections:
- Malaria, babesiosis

Chemical injury:
- lead poisoning

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176
Q

HML Pathology:
What are some acquired circumstances that cause an extrinsic hemolytic anemia, that is categorized as purely extravascular?

A

Hypersplenism

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177
Q

HML Pathology:
What is the pathogenesis of Sickle cells disease?

A

Point mutation (substitution of glutamic acid to valine at position 6) in the beta-chain gene leading to abnormal hemoglobin, HbS

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178
Q

HML Pathology:
Carriers of the sickle cell trait have protection against what?

A

Plasmodia falciparum malaria

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179
Q

HML Pathology:
Describe the sickle cell variant: Sickle cell trait: 5 points

A
  • heterozygotes with an HbA and an HbS allele (HbSA)
  • usually asymptomatic without anemia and only present with microscopic hematuria
  • increased risk of renal microinfarctions and renal papillary necrosis
  • labs positive for metabisulfate
  • Hb electrophoresis shows 55% HbA, 43% HbS, 2% HbA2
180
Q

HML Pathology:
Describe the sickle cell variant: Sickle cell disease

A

Homozygotes have two HbS alleles (HbSS)

181
Q

HML Pathology:
Describe the sickle cell variant: HbSC

A

Heterozygotes with an HbS and HbC (glutamic acid to lysine substitution allele

182
Q

HML Pathology:
What is the order of severity of the different sickle cell variants?

A

HbSS > HbSC > HbSA

183
Q

HML Pathology:
Describe the sickle cell variant: Sickle Beta-Thalassemia

A

Heterozygotes with an HbS and Beta-thalassemia (HbS/[beta]Th)

184
Q

HML Pathology:
What causes the sickle cell shape?

A

Aggregation and polymerization of HbS when deoxygenated
- hypoxia and fall in pH can also cause sickling

185
Q

HML Pathology:
What is a consequence of the sickle cell shape?

A
  • subject to splenic destruction
  • makes the blood hyper viscous which can lead to microvascular occlusion
186
Q

HML Pathology:
What can cause the MCHC to increase?

A

Dehydration

187
Q

HML Pathology:
What prevents polymerization of HbS in a newborn before 6 months?

188
Q

HML Pathology:
What are the four different groups of symptoms a patient with sickle cell can have?
Mneumonic: SHAVe

A
  1. Vasoocclusion symptoms
  2. Sequestration symptoms
  3. Hemolytic symptoms
  4. Aplastic symtoms
189
Q

HML Pathology:
How do the vaso-occlusion symptoms present in sickle cell disease? 8 points

A
  • pallor
  • Strokes
  • acute chest syndrome (associated with fever, hypoxia, and pulmonary infiltrate)
  • Dactylics
  • Aseptic necrosis of femoral or humeral head
  • Pain episodes in joints, abdomen, viscera, lung, liver, and penis
  • increased susceptibility to encapsulated bacteria including Salmonella osteomyelitis secondary to asplenia
  • Renal papillary necrosis, which leads to gross hematuria and proteinuria
190
Q

HML Pathology:
How do the sequestration symptoms present in sickle cell disease? 2 points

A
  • acute pooling of erythrocytes in the spleen after encapsulated infection
  • acute splenomegaly and septic shock-like state and hypovolemia
191
Q

HML Pathology:
How do the Hemolytic symptoms symptoms present in sickle cell disease? 2 points

A
  • intravascular hemolysis
  • massive erythroid hyperplasia due to expansion of hematopoiesis into skull (“crewcut”), facial bone (“chipmunk face”), and extra medullary hematopoiesis with hepatomegaly
192
Q

HML Pathology:
How do the aplastic symptoms present in sickle cell disease?

A

increase risk of Parvovirus B19 infection

193
Q

HML Pathology:
How do you diagnose sickle disease? 6 points

A
  • Hb Electrophoresis
  • Metabisulfate screen: Positive in both sickle cell trait and disease
  • CBC: anemia, reticulosytosis, leukocytosis, thrombocytosis
  • PBS: Sickled cells and Howell-Jolly Bodies (basophilic nuclear remnants due to autosplenectomy)
  • serum bilirubin and fecal/urinary urobilinogen are elevated (dues to extravascular hemolysis; low haptoglobin level (due to intravascular hemolysis)
  • skull radiograph shows “crew cut” pattern due to extramedulary hematopoiesis
194
Q

HML Pathology:
How do you treat sickle cell disease?

A
  • hydration
  • folic acid supplementation
  • blood transfustion
  • PCN prophylaxis
  • Vaccination against pneumococcal, H influenza, and meningococcal
  • pain control
  • Bone marrow transplantation
  • Hydroxyurea (increases concentration of HbF
195
Q

HML Pathology:
What is the inheritance pattern of pyruvate kinase deficiency?

A

Autosomal recessive

196
Q

HML Pathology:
How does a chronic anemia occur in the setting of Pyruvate Kinase deficiency? 2 points

A
  • chronic decreased ATP, leading to membrane damage because the Na+/K+ ATPase cannot be maintained
  • there is also a buildup of 2,3-BPG inducing a right shift, promoting oxygen unloading in the peripheral tissue
197
Q

HML Pathology:
How does a Pyruvate Kinase Deficiency present? 3 points

A
  • neonatal jaundice
  • extravascular hemolysis
  • splenomegaly
198
Q

HML Pathology:
What gene is mutated in Paroxysmal Nocturnal Hemoglobinuria?
What does it lead to pathophysiologically?
(Total 3 points)

A
  • PIGA
  • synthesis of glycosylphosphatidylinositol (GPI) anchors used for surface anchor protein attachment
  • without GPI no inactivation of complement, subjecting RBCs to lyse by endogenous compliment, platelets and granulocytes can be affected also
199
Q

HML Pathology:
How does Paroxysmal Nocturnal Hemoglobinuria (PNH) present?
5 points

A
  • episodic hemoglobinuria on awakening (mild respiratory acidosis from shallow breathing at sleep increases CO2 and activates complement)
  • hemosiderinuria can lead to iron deficiency
  • Pancytopenia (cell lysis)
  • Increased risk of venous thrombosis
  • increased risk for acute myeloid leukemia
200
Q

HML Pathology:
How would diagnose PNH? 3 points

A
  • clinical presentation
  • flow cytometry to detect lack of CD55 decay-accelerating factor (DAF) on blood cells
  • sucrose test or acidified serum test (activates complement)
201
Q

HML Pathology:
What is the prognosis of PNH?

A
  • aplastic anemia
  • acute leukemia
202
Q

HML Pharmacology:
How would you treat PNH?

A

Eculizumab and Ravulizumab (terminal compliment inhibitor)

203
Q

HML Pathology:
What is Microangiopathic Hemolytic Anemia?

A

Mechanical trauma caused buy narrowed vessels leading to intravascular hemolysis

204
Q

HML Pathology:
What conditions would you see a Microangiopathic Hemolytic Anemia? 7 points

A
  • Disseminated intravascular coagulation (DIC)
  • thrombotic thrombocytopenic purpura (TTP
  • hemolytic-uremic syndrome (HUS)
  • SLE
  • malignant HTN
  • prosthetic heart valves
  • aortic stenosis
205
Q

HML Pathology:
How would you diagnose microangiopathic hemolytic anemia?

A

PBS: schistocytes

206
Q

HML Pathology:
What is the cause of cardiac traumatic hemolytic anemia?

A

Turbulent flow and abnormal pressures occurring is prosthetic heart valves

207
Q

HML Pathology:
What is underlying problem in Aplastic Anemia?

A

Failure or destruction of multipotent myeloid stem cells leads to inadequate production or release of differentiated cell lines

208
Q

HML Pathology:
What some common causes of Aplastic Anemia? 6 points

A
  • chemicals: Benzene
  • Drugs: CHloramphenicol, sulfonamides, alkylating agents, antimalarial drugs, antimetabolites
  • Fanconi anemia
  • Idiopathic (immune-mediated or primary stem cell defect
  • radiation
  • Viral agents: Parvovirus B19, EBV, HIV, hepatitis C Virus
209
Q

HML Pathology:
How does aplastic anemia present?

A

Onset is gradual
- anemia
- thrombocytopenia
- neutropenia

210
Q

HML Pathology:
How is an aplastic anemia diagnosed?

A
  • CBC
  • Marrow biopsy: Hypocellular bone marrow and fatty infiltration
  • Peripheral smear: pancytopenia
    *no reticulocytosis and no splenomegaly
211
Q

HML Pathology:
What is the treatment for an aplastic anemia? 5 points

A
  • withdrawal of offending agent
  • immune therapy: antithymocyte globulin, cyclosporine
  • allogenic bone marrow transplantation
  • RBC and platelet transfusion
  • Granulocyte colony-stimulating factor (G-CSF), granulocyte- macrophage colony-stimulating factor (GM-CSF)
212
Q

HML Pathology:
What is the prognosis of aplastic anemia?

A

Unless it’s idiopathic, withdrawal of offending agent may lead to recovery

213
Q

HML Pharmacology:
What is common adverse effect of chloramphenicol?

A

Aplastic anemia

214
Q

HML Pathology:
What can porphyria be defined as?

A

A group of diseases that result from the accumulation of heme intermediates

215
Q

HML Pathology:
What is the inheritance patter of acute intermittent porphyria?

A

Autosomal dominant

216
Q

HML Pathology:
What enzyme is deficient in AIP?

A

Porpholbilinogen deaminase

217
Q

HML Pathology:
What is accumulated in AIP? What does it lead to? 2 points

A
  • Porphobilinogen
  • leads to degeneration of myelin
218
Q

HML Pathology:
How does AIP present? 6 points

A
  • medication induced (anything that increases heme synthesis: sulfa drugs, barbiturates, P450 inducers
    Symptoms:
  • dark, foul-smelling urine
  • abdominal pain
  • hallucinations
  • blurred vision
  • peripheral motor neuropathy (eg, foot drop) MIMICING GBS
219
Q

HML Pathology:
How would you diagnose AIP? 3 points

A
  • Genetic testing
  • increased urinary secretion of porphobilinogen and porphyrins
  • no sun-induced lesions
220
Q

HML Pathology:
How would treat AIP? 2 points

A

Goal is to decrease heme synthesis to reduce porphyrin precursor production
- high-carb diet during attacks
- severe attacks should be treated with hematin (inhibits ALA synthase)

221
Q

HML Pathology:
What enzyme has deficient activity in Porphyria Cutanea Tarda?

A

Urophorphyrinogen decarboxylase (UROD)

222
Q

HML Pathology:
What is elevated in Porphyria Cutanea Tarda?

A

Memory Aid: PCT increases your chances of getting a UTI
- Uroporyphyrin (large, insoluble molecule and photosensitive)
- Iron
- Transferrin

223
Q

HML Pathology:
How does Porphyria Cutanea Tarda present? 5 points

A
  • Cutaneous bullous
  • skin lesions that form after sun exposure
  • organ damage from siderosis affecting the liver progressing to fibrosis
  • urine turns dark upon standing
  • disease exacerbates by consuming of alcohol, iron, and estrogens
224
Q

HML Pathology:
How is Porphyria Cutanea Tarda diagnosed?

A

Plasma and urine analysis for elevated uroporphyrin (RBC’s can also be tested too)

225
Q

HML Pathology:
How is Porphyria Cutanea Tarda treated? 3 points

A
  • low dose antimalarial medications
  • phlebotomy
  • avoid alcohol, sun exposure, estrogens, and iron
226
Q

HML Pathology:
What is a consequence of severe thrombocytopenia?

A

Life-threatening intracranial bleeding

227
Q

HML Physiology:
What does von Willobrand Factor bind to?

228
Q

HML Physiology:
What releases von Willowbrand factor?

A
  • Weibel-Palade bodies of endothelial cells
  • alpha granules of platelets
229
Q

HML Pathology:
What labs would you expect in a pt with vessel abnormalities? 5 points

A
  • Normal platelet count
  • increase bleeding time
  • normal PT
  • normal PTT
  • normal thrombin time/ fibrinogen
230
Q

HML Pathology:
What labs would you expect in a pt with thrombocytopenia? 5 points

A
  • decreased platelet count
  • increased bleeding time
  • normal PT
  • normal PTT
  • normal thrombin time/ Fibrinogen
231
Q

HML Pathology:
What labs would you expect in a pt with qualitative platelet defects? 5 points

A
  • normal platelet count
  • increased bleeding time
  • Normal PT
  • normal PTT
  • normal thrombin time/ fibrinogen
232
Q

HML Pathology:
What labs would you expect in a pt with Hemophilia A or B?

A
  • normal platelet count
  • normal bleeding time
  • normal PT
  • increased PTT
  • normal thrombin time/ fibrinogen
233
Q

HML Pathology:
What labs would you expect in a pt with vitamin K deficiency?

A
  • normal platelet count
  • normal bleeding time
  • increased PT
  • increased PTT
  • normal thrombin time/ fibrinogen
234
Q

HML Pathology:
What labs would you expect in a pt with von Willebrand disease?

A
  • normal platelet count
  • increased bleeding time
  • normal PT
  • normal/increased PTT
  • normal thrombin time/ fibrinogen
235
Q

HML Pathology:
What labs would you expect in a pt with DIC?

A
  • decrease platelet count
  • increased bleeding time
  • increased PT
  • increased PTT
  • increased thrombin time/ fibrinogen
236
Q

HML Pathology:
What labs would you expect in a pt with Liver disease?

A
  • decreased platelet count
  • increased bleeding time
  • increased PT
  • increased PTT
  • increased thrombin time/fibrinogen
237
Q

HML Pathology:
What labs would you expect in a pt with multiple transfusions?

A
  • decreased platelet count
  • increased bleeding time
  • increased PT
  • increased PTT
  • increased thrombin time/ fibrinogen
238
Q

HML Pathology:
What is the treatment for Idiopathic thrombocytopenia purpura?

A
  • Initial treatment is corticosteroids
  • IVIG for symptomatic bleeding
  • splenectomy for refractory cases
239
Q

HML Pathology:
How does thrombotic microangiopathies occur?

A

Result from hyaline micro thrombi (platelet aggregates surrounded by fibrin) leading to thrombocytopenia and MAHA.

240
Q

HML Physiology:
what is a normal bleeding time?

A

2 to 9 minutes

241
Q

HML Pathology:
What causes a prolonged PT (prothrombin) time?

A

Deficiency in factors V, VII, or X, prothrombin, or fibrinogen

242
Q

HML Pathology:
What causes a prolonged PTT (Partial thromboplastin time)?

A

Heparin therapy

243
Q

HML Pathology:
What enzyme is lacking in Thrombotic Thrombocytopenia Purpura (TTP)?

A

metalloproteinase ADAMTS13

244
Q

HML Pathology:
What symptoms does TTP portray?
5 points

A
  • Microangiopathic hemolytic anemia
  • thrombocytopenia
  • renal failure
  • fever
  • neurological symptoms
245
Q

HML Pathology:
What population would you see TTP?

A

Adult females

246
Q

HML Pathology:
What platelet count would you consider to be pathological?

A

under 100,000

247
Q

HML Pathology:
What platelet count would you consider a patient to have spontaneous bleeding?

A

under 20,000

248
Q

HML Pathology:
What is the only lab value that is decreased in DIC?

A

Platelet count

249
Q

HML Pathology:
What is the inheritance of von Willebrand disease?

A

Autosomal dominant

250
Q

HML Physiology:
What is the role of von Willebrand Factor?

A

Carry and stabilize factor 8

251
Q

HML Pathology:
What is the inheritance pattern of Bernard-Soulier disease?

A

Autosomal recessive

252
Q

HML Pathology:
What is the problem inBernard- Soulier disease?

A

platelets are abnormally large and lack platelet-surface glycoprotein IB, leading to defective platelet adhesion

253
Q

HML Physiology:
What is the product of cyclooxygenase?

A

Thromboxane A2, a platelet aggregate

254
Q

HML Pathology:
What is deficient in Glanzmann thrombasthenia?

A

glycoprotein IIB and IIIA, required for forming fibrinogen bridges between platelets during platelet aggregation

255
Q

HML Pathology:
What is the inheritance in classic hemophilia?

A

X-linked deficiency of factor VIII (hemophilia A)

256
Q

HML Pathology:
What lab change would you expect in classic hemophilia?

A

PTT normalizes in mixing studies

257
Q

HML Pathology:
How do you treat classic hemophilia?

A

Factor VIII and Desmopressin to release additional Factor VIII from the endothelial stores

258
Q

HML Pathology:
What is the problem in Christmas Disease?

A

X linked deficiency of factor IX, mixing studies does not correct after factor VIII

259
Q

HML Pathology:
What is the inheritance and problem in Hemophilia C?

A

Autosomal Recessive deficiency of factor XI affecting PTT

260
Q

HML Pathology:
What vitamin is absent in breastmilk?

261
Q

HML Physiology:
What enzyme activate vitamin K?

A

Epoxide reductase

262
Q

HML Pathology:
What kind of symptoms would you see in Liver disease? 3 points

A
  • hemarthroses (bleeding into joints)
  • easy bruising
  • hematomas rather than petechiae
263
Q

HML Pathology:
What components are ‘consumed’ in disseminated intravascular coagulation DIC? 2 points

A
  • platelets
  • coagulation factors (II, V, VIII, Fibrinogen)
264
Q

HML Pathology:
Describe the mechanism that causes DIC? 2 points

A
  • release of tissue thromboplastin, or
  • activation of the intrinsic pathway
265
Q

HML Pathology:
What are the common causes of DIC? 7 points

A
  • obstetric complication
  • gram negative sepsis
  • transfusion, trauma
  • malignancy ( esp. adenocarcinoma of the lung, pancreas, prostate, and stomach)
  • acute pancreatitis
  • nephritic syndrome
  • acute promyelocytic leukemia (degranulation)
266
Q

HML Pathology:
What risk is increased with Ehlers-Danlos syndrome?

A
  • aortic dissection and berry aneurysm
267
Q

HML Pathology:
What is the cause of vessel wall abnormalities in Cushing Syndrome?

A

Protein wasting

268
Q

HML Pathology:
What kind of vessel wall abnormalities are associated with Henoch-Schönlein purpura?

A
  • Hypersensitivity vasculitis, often associated with URI.
  • Symptoms include palpable purpura, polyarthraligias, fever, painful focal GI hemorrhages, and acute glomerulonephritis
269
Q

HML Pathology:
What is the inheritance pattern of Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)?

A

Autosomal dominant

270
Q

HML Pathology:
Why does Waldenström macroglobinulinemia cause vessel wall abnormalities?

A

Hyperviscous blood

271
Q

HML Pathology:
What test has replaced bleeding time tests?

A

Platelet function assay (PFA)-100

272
Q

HML Pathology:
How does hypercortisolism create eosinopenia?

A

Sequesters eosinophils inside of lymph nodes

273
Q

HML Pathology:
What causes a neutrophilic leukocytosis? 2 points

A
  • Bacterial infection
  • tissue necrosis
274
Q

HML Pathology:
What number is considered a lymphopenia? 2 points

A
  • < 1500 in adults
  • < 3000 in children
275
Q

HML Pathology:
What number is considered leukocytosis? 2 points

A
  • > 4000 in adults
  • > 8000 in children
276
Q

HML Pathology:
What are 5 key facts about Hodgkin lymphomas?

A
  • Reed-Sternberg cells (CD15 CD30)
  • single groups of axial nodes
  • contiguous spread
  • constitutional symptoms
  • bimodal distribution
277
Q

HML Pathology:
What are the four histological variants of Hodgkin lymphoma?

A
  • nodular sclerosis
  • mixed cellularity
  • lymphocyte predominant
  • lymphocyte depleted
278
Q

HML Pathology:
What viral infection is commonly associated with Hodgkins Lymphoma?

279
Q

HML Pathology:
What are six key facts about non-hodgkin lymphomas?

A
  • No Reed-Sternberg cells
  • multiple groups of peripheral nodes
  • noncontiguous spread
  • fewer constitutional symptoms
  • usually seen in those 2–40 years old
  • associated with HIV and autoimmune diseases
280
Q

HML Pathology:
What is the translocation for CML?

A

t(9;22) Ph chromosome

281
Q

HML Pathology:
What is the translocation for Burkitt lymphoma?

A

t(8;14) c-myc

282
Q

HML Pathology:
What is the translocation for Follicular lymphoma?

A

t(14;18) bcl-2

283
Q

HML Pathology:
What is the translocation for AML M3 type?

A

t(15;17) APL: RARA

284
Q

HML Pathology:
What is the translocation for Ewing Sarcoma?

285
Q

HML Pathology:
What is the translocation for Mantle cell lymphoma?

286
Q

HML Pathology:
What determines the prognosis of Hodgkin Lymphomas?
3 points

A
  • Staging, the best predictor of prognosis.
  • Amount of lymphoid aggregates and dissemination.
  • Also chemotherapy and radiation therapy can induce secondary cancers.
287
Q

HML Physiology:
What three actions does Bradykinin do?

A

Increase vasodilation, permeability, and pain

288
Q

HML Pathology:
What type of non-hodgkin lymphoma is the only nodular type?

A

Follicular Lymphoma

289
Q

HML Pathology:
Describe Hodgkin Lymphoma type: Nodular Sclerosing (7 points)

A
  • Women > Men
  • Cells grouped into nodule surrounded by “fibrous bands”
  • usually located in cervical, supraclavicular, or mediastinal nodes
  • Lacunar cells (Reed-Sternberg nucleus surrounded by empty space
  • can be related to EBV infection
  • Most common prevalence (70%)
  • Excellent prognosis
290
Q

HML Pathology:
Describe histology of Hodgkin Lymphoma: Mixed cellularity (7 points)

A
  • typically seen in young men
  • also often related to EBV infection
  • complete effacement, multiple nodes
  • Eosinophils, plasma cells, histiocytes, and Reed-Sternberg cells with regions of fibrosis
  • 2nd most common (25%)
  • most amount of Reed-Sternberg cells
  • intermediate prognosis
291
Q

HML Pathology:
Describe histology of Hodgkin Lymphoma: Lymphocyte predominant (6 points)

A
  • Lymphocytes and histiocytes
  • <35 year old men
  • popcorn cells (Reed-Sternberg cells)
  • 6% prevalence
  • most amount of lymphocytes involved
  • excellent prognosis
292
Q

HML Pathology:
Describe histology of Hodgkin Lymphoma: Lymphocyte depleted (6 points)

A
  • few lymphocytes with large number of Reed-Sternberg cells and fibrosis
  • older men with disseminated disease, HIV patients
  • most rare prevalence
  • Higher number of RS cells relative to lymphocytes
  • Least amount of lymphocytes involved
  • poor prognosis
293
Q

HML Pathology:
In terms of Cutaneous T-cell Lymphomas, what is the difference between Mycosis fungoides and Sézary syndrome?

A

Mycosis fungoides is in the skin (dermis and epidermis), Sézary syndrome is in the blood

294
Q

HML Pathology:
How would you diagnose Cutaneous T-cell Lymphoma? 2 points

A
  • Bx showing CD4+ T cells with cerebriform nuclei proliferating in the dermis
  • focal concentrations of neoplastic cells in the epidermis are called Pautrier microabscesses
295
Q

HML Pathology:
Describe the prognosis of Cutaneous T-cell lymphomas? 3 points

A
  • indolent (minimal pain)
  • average survival 8-9 years
  • can progress to diffuse large-cell lymphoma
296
Q

HML Pathology:
What organs/structures are affected in a MALToma? 4 points

A
  • Salivary glands
  • Small intestines
  • Large intestines
  • Lungs
297
Q

HML Pathology:
What bacterial infection is associated with MALToma?

A

Helicobacter Pylori

298
Q

HML Pathology:
What common chronic inflammatory conditions are associated with MALTomas? 2 points

A
  • Hashimoto Thyroiditis
  • Sjögren syndrome
299
Q

HML Pathology:
Describe the histological type and its characteristics of Non-Hodgkin lymphoma: Small lymphocytic lymphoma
5 points

A
  • Histology: Small, mature looking lymphocytes; lymph nodes are effaced
  • affects B cells in adults
  • low grade
  • appears as a focal mall of CLL
  • “Richter syndrome” or prolymyphocytic transformation into diffuse B-cell lymphoma occurs in a quarter of cases
300
Q

HML Pathology:
Describe the histological type and its characteristics of Non-Hodgkin lymphoma: Follicular lymphoma
8 points

A
  • Histology: arranged in nodules
  • complication include progression to diffuse large B-cell lymphoma
  • Genetics: t(14;18), BCL2 to Ig Heavy chain locus on chromosome 12 leads to over expression and prevents apoptosis (bad thing in this case)
  • Low grade
  • B cells affected
  • affects adults
  • Presentation is painless “waxing and waning” lymphadenopathy
  • Tx is low dose chemotherapy or rituximab
301
Q

HML Pathology:
Describe the histological type and its characteristics of Non-Hodgkin lymphoma: Diffuse Large cell
8 points

A
  • Histology: Large B cells that grow diffusely in sheets
  • Genetics: associated with BCL-6
  • intermediate grade
  • 80% B cells, 20% mature T cells involved
  • 80/20 % split for adult and children involved
  • occurs most commonly
  • often presents with extra nodal mass
  • aggressive, 50% cure rate
302
Q

HML Pathology:
Describe the histological type and its characteristics of Non-Hodgkin lymphoma: Mantle cells lymphoma
6 points

A
  • Histology: resemble cells in the mantle zone of lymph nodes
  • Genetics: t(11’14) - cyclin D1 (11) and Ig heavy chain (14) leads to over expression of cyclin D1, which promotes G1/S transition
  • B cells affected
  • adults affected
  • poor prognosis
  • GI tract often involved causing lymphomatoid polyposis
303
Q

HML Pathology:
Describe the histological type and its characteristics of Non-Hodgkin lymphoma: Lymphoblastic lymphoma
7 points

A
  • Histology: cell nuclei appear convoluted; arise from thymic lymphocytes
  • High grade
  • Immature T cells involved most commonly in children but adults too
  • 80% cure rate, but high relapse rate
  • presents with ALL and mediastinal mass
304
Q

HML Pathology:
Describe the histological type and its characteristics of Non-Hodgkin lymphoma:
9 points

A
  • Histology: starry-sky appearance (sheets of lymphocytes with interspersed nonneoplastic macrophages), high mitotic index
  • Genetics: t(8;14) - translocation of c-myc (8) and Ig Heavy chain.
  • High grade
  • B cells affected
  • children and adults affected
  • associated with EBV, HPV
  • very rapid growing mass
  • jaw involved common in endemic affray form
  • pelvic/abdomoninal involvement often occurs in sporadic form
305
Q

HML Pathology:
Where can you expect for relapses to occur in acute lymphoblastic leukemia?

A

CNS and testes

306
Q

HML Pathology:
How does acute lymphoblastic leukemia present?
2 points

A
  • adolescent with a mediastinal mass that may spread other trachea, esophagus, and SVC
  • Marrow failure, lymphadenopathy, splenomegaly hepatomegaly also important findings
307
Q

HML Pathology:
What are the common leukemias and the leukemias associated?
4 points

A
  • < 15 yrs: ALL
  • 15-39 yrs: AML
  • > 40-60 yrs: AML, CML
  • 60+: CLL
308
Q

HML Pathology:
What tests would you order to diagnose a leukemia?
3-4 points

A
  • Flow cytometry
  • PBS
  • bone marrow biopsy
    Would see monomorphic cels with condensed chromatin, minimal cytoplasm, and no granules
309
Q

HML Pathology:
How can you differentiate an Acute myeloid leukemia from T cell and B cell?

A
  • Surface markers
    TdT+ marks for pre-T and pre-B; CD10+ is a marker for pre-B only.
310
Q

HML Pathology:
What is often associated with acute lymphoblastic leukemia?
What labs would you expect to see?
2 points

A
  • Tumor lysis syndrome
  • hyperuricacidemia, hyperphosphatemia, hyperkalemia
311
Q

HML Pathology:
What cell marker would you expect to see in Chromic Lymphocytic Leukemia?

A

CD5+ B cells

312
Q

HML Pathology:
Do the proliferating B cells mature to plasma cells and secrete antibodies in CLL?

313
Q

HML Pathology:
How does chronic lymphocytic leukemia present?
5 points

A
  • 60 yr old pts or older
  • lymphadenopathy and hepatosplenomegaly are common
  • bacterial infection common due to hypogammaglobunemia
  • auto bodies can form and cause warm antibody autoimmune hemolytic anemia or thrombocytopenia
  • prolymphocytic transformation into diffuse B-cell lymphoma occurs in 25% of cases (Richter transformation)
314
Q

HML Pathology:
How can you diagnose CLL?
2 points

A
  • Peripheral smears and bone marrow bx will show smudge cells
  • some pts may show a monoclonal Ig “spike”
315
Q

HML Pathology:
What is another name for Acute myelogenous Leukemia (AML)?

A

Acute granulocytic leukemia

316
Q

HML Pathology:
What is the pathophysiology of APML?
2 points

A
  • Translocation of the retinoid acid receptor-alpha gene from chromosome 17 to the promyelocytic leukemia gene on chromosome 15
  • t(15;17)(q22;q12) - prevents cell differentiation and maturation
317
Q

HML Pathology:
What age population would you expect AML to come from myelodysplasia?

318
Q

HML Pathology:
What symptoms does AML present with?
4 points

A
  • fatigue
  • infection
  • bleeding (anemic, neutropenia, thrombocytopenia)
  • cutaneous lesions (leukemia cutis, gingival hypertrophy, and chloromas)
319
Q

HML Pathology:
What are some risk factors for AML?
4 points

A
  • Hx of alkylating agents
  • hx of topoisomerase inhibitors
  • radiation
  • myeloproliferative disorders
320
Q

HML Pathology:
How would you diagnose AML?
6 points

A
  • peripheral smeal
  • bone marrow biopsy
  • biopsy reveals >20% myeloid blasts in marrow
  • myeloblasts will be peroxidase-positive and have abundant cytoplasm compared with lymphoblasts
  • Aeur rods present (peroxidase positive cytoplasmic inclusions)
  • The lack of TdT and CD10 surface markers to differentiate from ALL
321
Q

HML Pathology:
How can you compare CLL and SLL (small)?

A

same except CLL proliferates in the blood and BM vs SLL in the nodal and extra nodal masses

322
Q

HML Pharmacology:
How do you treat AML?
2 points

A
  • chemotherapy:
    7 + 3 + 3 - cytarabine, daunorubicin, etoposide
  • high dose all- trans retinoid acid for APML (APL) with t(15;17)
323
Q

HML Pharmacology:
What is the danger in treating APL?

A

Tx can release large numbers of Auer rods, which can result in DIC

324
Q

HML Pathology:
Which leukemia is also a myeloproliferative disorder?

A

Chronic Myelogenous Leukemia

325
Q

HML Pathology:
What is the pathophysiology of Chronic Myelogenous Leukemia?

A

Translocation 9;22 cause BCR-ABL fusion, this has tyrosine kinase activity which enhances cellular proliferation, inhibiting normal cell production due to overcrowding. Associated with the Ph (Philadelphia) chromosome.

326
Q

HML Pathology:
Describe the how CML presents?
4 points

A
  • insidious onset with nonspecific symptoms
  • splenomegaly is common
  • accelerated phase with increasing anemia and thrombocytopenia occurring after around 3 years
  • all untreated pts eventually enter a blast crisis with > 20% blasts. 2/3 progress to AML, 1/3 ALL
327
Q

HML Pathology:
What are the risk factors for developing Chronic Myelogenous Leukemia?
2 points

A
  • ionizing radiation
  • Benzene
328
Q

HML Pathology:
How can you diagnose CML?
3 points

A
  • chromosomal analysis or PCR to detect BCR-ABL fusion gene
  • Bone marrowy biopsy and peripheral smear also show leukocytosis with mixed neutrophils, metamyelocytes, and absolute basophilia
  • normal leukocyte ALP is no elevated (vs leukemoid reaction)
329
Q

HML Pharmacology:
How do you treat chronic myelogenous leukemia?
2 points

A
  • Gleevec (imatinib mesylate, a small- molecule inhibitor of the BCR-ABL tyrosine kinase): induces apoptosis of leukemic cells
  • Allogenic bone marrow transplantation: cures 75%, most effective in the stable phase
330
Q

HML Pathology:
Which leukemia has cells that are CD103+ and TRAP+ (tartrate resistant acid phosphatase)?

A

Hairy cell leukemia

331
Q

HML Pathology:
What can Hairy cell leukemia progress to?

A

Marrow fibrosis, bone marrow bx will show “dry tap”

332
Q

HML Pathology:
How do you treat Hairy cell leukemia?
2 points

A
  • Cladribine
  • adenosine deaminase inhibitor
333
Q

HML Pathology:
How is Adult T-cell Leukemia/Lymphoma caused?

A
  • infection with a retrovirus HTLV-1 (human T-cell leukemia virus type 1)
334
Q

HML Pathology:
How is Adult T-cell leukemia/lymphoma characterized?
5 points

A
  • skin lesions
  • generalized lymphadenopathy
  • HS-megaly
  • hypercalcemia
  • elevated leukocyte count with multi lobed CD4 lymphocytes
335
Q

HML Pathology:
What can hairy cell leukemia progress to?

A

Demyelination of the CNS

336
Q

HML Pathology:
How many Plasma Cell disorders are there?
Name them

A

3
- Multiple myeloma
- Waldenström macroglobulinemia
- Monoclonal gammopathy

337
Q

HML Pathology:
What is the M component?

A

Monoclonal Ig

338
Q

HML Pathology:
Why are Bence Jones proteins made?

A

There is a loss of balance between the heavy and light chains, causing excess free light chains to escape in the urine.

339
Q

HML Pathology:
Who is responsible for the myeloma proliferation and its survival in Multiple Myeloma?

340
Q

HML Pathology:
What is the mnemonic for the clinical manifestations in Multiple Myeloma?
Name each component

A

CRAB
- hyperCalcemia
- Renal involvement
- Anemia
- Bone lytic lesions/ Back pain

341
Q

HML Pathology:
How does multiple myeloma present?
8 points

A
  • 50-60 yr
  • punched out bone lesion in vertebrae or skull (osteoclast activating factor, IL-1 on RANK receptors)
  • Hypercalcemia
  • myeloma kidney, or renal insufficiency with azotemia. Bence Jones proteins can cause tubular casts
  • Marrow failure (anemia > leuko/thrombocytopenia
  • Infections (Staph A, Strep Pneumonia, E. coli)
  • Amyloidosis from IgG light chain
  • Hyperviscosity syndrome (rare)
342
Q

HML Pathology:
How can you diagnose Multiple Myeloma?
6 points

A
  • rouleaux formation of RBCs
  • Electrophoresis reveal increase Ig in blood
  • Bence Jones proteins in the urine
  • M spike: IgG 50% of the time, IgA 25% of the time
  • punched out round skeletal lesions
  • cells have a “fried egg” appearance
343
Q

HML Pathology:
How can you treat Multiple Myeloma?
3 points

A
  • Chemotherapy (remission in 50%)
  • Bisphosponates
  • Bone marrow transplant (improve survival but not curative)
344
Q

HML Pathology:
What is Waldenström Macroglobulinemia?

A

Neoplasm of plasmacytoid lymphocytes (cells between B lymphocytes and plasma cells in terms of maturity) with monoclonal IgM secretion

345
Q

HML Pathology:
How does Waldenström Macroglobulinemia present?
5 points

A
  • affects older individuals
  • nonspecific symptoms (fatigue and weight loss) are common
  • lymphadenopathy, hepatomegaly, and splenomegaly
  • anemia due to marrow failure and IgM cold antibody AI hemolysis
  • hypervisocity syndrome presenting as visual impairment via retinal vascular dilation, neurological issues, bleeding, and Raynaud phenomenon
346
Q

HML Pathology:
How can you diagnose Waldenström Macroglobulinemia?

A
  • Serum electrophoresis reveals clonal IgM spike
  • 10% of cases show Bence Jones proteinuria
  • no bone lesions
347
Q

HML Pathology:
What is the prognosis of Waldenström Macroglobulinemia?
2 points

A
  • incurable and progressive, median survival of 4 years. Plasmapheresis for hemolysis and hyperviscosity syndrome.
  • Rarely transforms to large-cell lymphoma
348
Q

HML Pathology:
Describe MGUS:
4 points

A
  • Monoclonal Gammopathy of Uncertain Significance
  • Mostly benign M proteins, asymptomatic
  • some can develop into multiple myeloma or other plasma cell pathologies after a decade
  • Amyloidosis can also develop
349
Q

HML Pathology:
How many different forms are there of Langerhans Cell Histiocytosis?
Name them:

A

Four
1. Letterer-Siwe disease
2. Hand-Schüller-Christian disease
3. Multifocal Langerhans cell histocytosis
4. Eosinophilic granuloma

350
Q

HML Pathology:
Describe Letterer-Siwe disease:
6 points

A
  • before 2 yrs of age
  • acute disseminated histiocytosis
  • cutaneous lesions on trunk and scalp
  • marrow failure
  • hepatosplenomegaly
  • osteolytic lesions
351
Q

HML Pathology:
Describe Hand-Schüller-CHristian disease:

A

Triad of calvarial lesions, DI, and exophthalmos

352
Q

HML Pathology:
Describe Langerhans cell histocytosis:
6 points

A
  • affects children
  • fever
  • eruptions on the scalp and ear canals
    -recurrent infections
  • HS-megaly
  • DI from posterior pituitary involvement
353
Q

HML Pathology:
Describe an Eosinophilic Granuloma:

A
  • Unifocal or multifocal expansion of Langerhans cell, usually in the marrow space and occasionally in lung
  • asymptomatic and benign
  • may present with fracture in adolescence (without skin involvement)
  • bx shows lagerhands cell with increased eosinophils and mixed inflammatory cells
354
Q

HML Pathology:
How would you diagnose Langerhans Cell Histiocytosis?

A

Electron microscopy reveals Birbeck granules (CD1a and S-100 positive) in the cytoplasm, which appear like tennis rackets

355
Q

HML Pathology:
What are the different kinds of myeloproliferative syndromes?

A
  • Chronic Myelogenous Leukemia
  • Polycythemia vera
  • essential thrombocythemia
356
Q

HML Pathology:
What cells do you expect to produce in excess in Polycythemia vera?
3 points

A
  • Erythrocytes
  • Granulocytes
  • megakaryocytes
357
Q

HML Pathology:
How does Polycythemia Vera present?
8 points

A
  • insidious consent around 60 yrs
  • cyanosis
  • bleeding and thrombosis risks can lead to a DVT, MI, stroke, Budd-Chiari syndrome, splenic and mesenteric infarction
  • headache
  • pruritus after hot shower due to increase basophils
  • “spent phase” showing BM fibrosis, resulting in extra medullary hematopoiesis
  • Myelofibrosis with myeloid metaplasia
  • Splenomegali
358
Q

HML Pathology:
How do you diagnose Polycythemia Vera?

A
  • increased hematocrit
  • decreased EPO (JAK2 V617F mutation)
  • bone marrow is hyper cellular until the spent phase
  • Leukocyte ALP are elevated
359
Q

HML Pathology:
How do you treat Polycythemia vera?

A

Phlebotomy; increased survival up to 10 years

360
Q

HML Pathology:
Describe how relative polycythemia/erythrocythemia develops:

A

Due to decreased plasma volume (volume contraction) cause by H20 deprivation

361
Q

HML Pathology:
Describe how absolute polycythemia/erythrocythrmia develops:

A

Increased total RBC mass due to PCV, increased sensitivity to EPO receptor, increased levels of EPO, physiologic changes, or EPO secreting tumors

362
Q

HML Pathology:
Describe the plasma volume, RBC mass, SAO2, a EPO levels in Relative polycythemia/erythrocythemia:

A
  • decreased Plasma volume
  • no chance in RBC mass, SAO2, and EPO levels
363
Q

HML Pathology:
Describe the plasma volume, RBC mass, SAO2, a EPO levels in Absolute polycythemia erythrocythemia?

A
  • increase in plasma volume
  • increase in RBC mass
  • no change in SAO2
  • decrease EPO levels
364
Q

HML Pathology:
Describe the plasma volume, RBC mass, SAO2, a EPO levels in Hypoxia:

A
  • no change in plasma volume
  • increase in RBC mass
  • decrease in SAO2
  • increase in EPO levels
365
Q

HML Pathology:
Describe the plasma volume, RBC mass, SAO2, a EPO levels in RCC (with ectopic EPO secretion:

A
  • no change in plasma volume
  • increase in RBC mass
  • no change in SAO2
  • increase in EPO levels
366
Q

HML Pathology:
What is the prognosis of Polycythemia Vera?

A

Death within months without treatment

367
Q

HML Pathology:
Describe the pathogenesis of Myelofibrosis with Myeloid Metaplasia:

A

Meoplastic changes in multipoint stem cells leading to. proliferation of megakaryocytes. Megakaryocytes release platelet-derived growth factor and transforming growth factor beta, which grows fibroblasts. Fibroblasts release lot of collagen leading to fibrosis. Similar to the spent stage in PCV.

368
Q

HML Pathology:
How does Myelofibrosis with Myeloid Metaplasia present?
4 points

A
  • 60+ yr
  • anemia, bleeding, thrombosis, and recurrent infections
  • hyperuricemia
  • hepatosplenomegaly
369
Q

HML Pathology:
How would you diagnose Myelofibrosis with Myeloid Metaplasia?
3 points

A
  • Peripheral smear shows leukoerythroblastosis, increased numbers of nucleated erythroid progenitors and early granulocytes
  • Dacrocytes/ teardrop erythrocytes
  • normochromic normocytic anemia and thrombocytopenia as the disease advances
370
Q

HML Pharmacology:
How would you treat myrlofibrosis with myeloid metaplasia?

A

JAK2 kinase inhibitors

371
Q

HML Pathology:
How does Essential Thrombocythemia present?

A

Indolent course, usually asymptomatic except for episodes of prominent thrombosis and hemorrhage

372
Q

HML Pathology:
How would you diagnose Essential thrombocythemia?

A
  • JAK2 mutations
  • abnormally large megakaryocytes and platelets
373
Q

HML Pharmacology:
How would you treat essential throbocythemia?

A

Aspirin and hydroxyurea for high-risk patients (older individuals, history of thrombosis)

374
Q

HML Pathology:
Describe tumor grading:

A
  • graded 1-3/4
  • based on differentiation; poorly differentiated tend to be highly aggressive
375
Q

HML Pathology:
Describe tumor staging:

A
  • determines treatment and prognosis based on spread
  • TNM staging system:
    Tumor: is the extent of growth.
    Nodes: which and how many lymph nodes are involved
    Metastasis: has the tumor metastasized
376
Q

HML Pathology:
Which virus can develop to T-cell leukemia and lymphoma?

A

RNA oncogenic virus Human T-cell leukemia virus type 1

377
Q

HML Pathology:
Which virus can develop into Burkitt lymphoma, AIDS-related lymphoma, Hodgkin Lymphoma, nasopharyngeal carcinoma?

A

DNA oncogenic virus Epstein-Barr virus

378
Q

HML Pathology:
Which virus can develop into cervical carcinoma?

A

DNA oncogenic virus Human papillomavirus

379
Q

HML Pathology:
Which virus can develop Kaposi sarcoma?

A

DNA oncogenic virus Human herpesvirus-8

380
Q

HML Pathology:
Which virus can develop hepatocellular carcinoma?

A

DNA oncogenic virus Hepatitis B virus

381
Q

HML Pathology:
Which cancer as associated with paraneoplastic syndrome: Cushing syndrome?

A

small cell lung cancer, pancreatic carcinoma

382
Q

HML Pathology:
Which cancer as associated with paraneoplastic syndrome: SIADH?

A

small cell lung cancer, intracranial neoplasm

383
Q

HML Pathology:
Which cancer as associated with paraneoplastic syndrome: Hypercalcemia?

A

squamous cell cancer of the lung, breast cancer, renal cell carcinoma

384
Q

HML Pathology:
Which cancer as associated with paraneoplastic syndrome: Myasthenia gravis?

385
Q

HML Pathology:
Which cancer as associated with paraneoplastic syndrome: Hypertrophic osteoarthropathy?

A

Lung cancer

386
Q

HML Pathology:
Which cancer as associated with paraneoplastic syndrome: Migratory venous thrombosis (Trousseau syndrome)

A

Pancreatic carcinoma

387
Q

HML Pathology:
Which cancer as associated with paraneoplastic syndrome: Cancer cachexia?

A

Generally it’s a progressive loss of lean body mass and body fat, weakness, anorexia, and anemia

388
Q

HML Pharmacology:
Name the three Nitrosoureas:

A

Carmustine, Lomustine, Streptozocin

389
Q

HML Pharmacology:
What is the mechanism of Nitrosoureas?

A

Interfere with DNA and RNA synthesis by alkylation and protein modification. Requires bioactivation, they are lipid soluble and can cross the BBB.

390
Q

HML Pharmacology:
Name the different categories of chemotherapy drugs:
4 points

A
  • Tomopisomerase inhibitors
  • Antimetabolites
  • Microtubule inhibitors
    -Cell Cycle independent drugs: Platinum agent and Alkylating agents
391
Q

HML Pharmacology:
What are the side effects of using nitrosoureas?
5 points

A
  • CNS toxicity (dizziness and ataxia)
  • myelosuppression
  • dose related nephrotoxicity
  • hepatotoxicity
  • pulmonary toxicity (infiltrates or fibrosis)
392
Q

HML Pharmacology:
Name the platinum agents

A

Cisplatin, carboplatin, oxaliplatin

393
Q

HML Pharmacology:
Describe the mechanism of platinum agents:

A

Crisslinks DNA strands inhibiting replication

394
Q

HML Pharmacology:
Which cancers can you use platinum agents?
4 points

A

Testicular, bladder, ovary, and lung carcinomas

395
Q

HML Pharmacology:
What are the side effects of using platinum agents?
5 points

A
  • anaphylactic-like reactions*
  • nephrotoxicity*
  • neurotoxicity
  • ototoxicity
  • vomiting
396
Q

HML Pharmacology:
How can you prevent nephrotoxicity when using platinum agents?

A

Amifostine (free radical scavenger) and chloride (saline) diuresis.

397
Q

HML Pharmacology:
Describe the mechanism of Busulfan:

A

Alkylates DNA

398
Q

HML Pharmacology:
When can you use Busulfan?
2 points

A
  • leukemias/lymphomas
  • also to ablate BM before transplant
399
Q

HML Pharmacology:
What are the side effects of Busulfan?
4 points

A
  • pulmonary fibrosis*
  • hyperpigmentation
  • seizures
  • severe myelosuppression
400
Q

HML Pharmacology:
Which anticancer drugs are cell cycle specific?

A

Antimetabolites, predominantly target the S phase

401
Q

HML Pharmacology:
Describe the mechanism of Methotrexate:

A

Folic antimetabolite inhibiting dihydropfolate reductase, impairing DNA and protein synthesis

402
Q

HML Pharmacology:
Name the uses for methotrexate:
10 points

A
  • leukemias/lymphomas
  • choriocarcinomas
  • sarcomas
  • abortion
  • ectopic pregnancy
  • Rheumatoid arthritis
  • Crohn disease
  • psoriasis
  • IBD
  • Vasculitis
403
Q

HML Pharmacology:
Describe the side effects of methotrexate:
6 points

A
  • Myelosuppression
  • fatty change to the liver similar to ethanol and amiodarone
  • skin rash
  • mucositis (mouth ulcer)
  • nephrotoxicity
  • pulmonary fibrosis
404
Q

HML Pharmacology:
How can you reverse the myelosuppression when taking methotrexate?

A

Leucovorin (folinic acid)

405
Q

HML Pharmacology:
Describe the mechanism of action of Pemetrexed:

A
  • pyrrolopyrimidine antifolaxe analog that requires a reduced folate carrier and activation. Inhibits Thymidylate Synthase.
406
Q

HML Pharmacology:
When can you use Pemetrexed?

A

Mesothelioma and other lung cancers

407
Q

HML Pharmacology:
Describe the side effects of Pemetrexed:
5 points

A
  • Myelosuppression
  • skin rash
  • mucositosis
  • diarrhea
  • fatigue
    *Reduce toxicities with folic acid and vitamin B12 supplementation.
408
Q

HML Pharmacology:
Describe the MOA of 6-MP:

A

Blocks purine synthesis. Must be activated by Hypoxanthine guanine phosphoribosyl transferase (HGPRTase)

409
Q

HML Pharmacology:
Describe the uses of 6-MP:

A
  • Leukemias/lymphomas
  • prevents organ rejection
  • tx for RA, IBD, and SLE
410
Q

HML Pharmacology:
Describe the side effects of 6-MP:
5 points

A
  • myelosuppression
  • hepatotoxicity
  • nausea
  • vomitting
    *6-MP is metabolized by xanthine oxidase, so toxicity increases if inhibited (eg allopurinol)
411
Q

HML Pharmacology:
Describe the MOA of antimetabolite Cytarabine:

A

Pyrimidine antagonist, terminates chain elongation. Also inhibits DNA polymerase

412
Q

HML Pharmacology:
When can you use antimetabolite Cytarabine?

A

Leukemias/Lymphomas

413
Q

HML Pharmacology:
Describe the side effects of antimetabolite Cytarabine:

A
  • potent myelosuppression
    Leukopenia, thrombocytopenia, megaloblastic anemia, neurotoxicity, and nephrotoxicity
414
Q

HML Pharmacology:
Describe the antimetabolite 5-FU:

A

Pyrimidine analog that is bioactivated to 5F-dUMP. 5F-dUMP bind to flic acid inhibiting thymidylate synthase, thus inhibiting nucleic acid synthesis.

415
Q

HML Pharmacology:
Describe the uses of antimetabolite 5-FU:

A

Solid tumors (eg, colon cancer, pancreatic cancers). Used topically for basal cell carcinoma of the skin.

416
Q

HML Pharmacology:
Describe the side effects of antimetabolite 5-FU:

A

‘Hand-foot syndrome’ (dermopathy after extended use), mucositis, GI toxicity (diarrhea)

417
Q

HML Pharmacology:
Describe the MOA of Etoposide and Teniposide:

A

Inhibits topoisomerase II (increases DNA damage)

418
Q

HML Pharmacology:
Describe the uses of Etoposide and Teniposide:

A

Solid tumors (particularly testicular and small cell lung cancer), leukemias, and lymphomas

419
Q

HML Pharmacology:
Describe the side effects of Etoposide and Teniposide:

A
  • Myelosuppression
  • GI upset
  • alopecia
420
Q

HML Pharmacology:
Describe the MOA of Irinotecan and Topotecan:

A

Inhibit topoisomerase I (prevents DNA unwinding and replication)

421
Q

HML Pharmacology:
Describe the use of Irinotecan and Topotecan:
2 points

A
  • Colon cancer (irinotecan)
  • Ovarian and small cell lung cancers (topotecan)
422
Q

HML Pharmacology:
Describe the side effects of Irinotecan and Topotecan:
2 points

A
  • Severe myelosuppression
  • diarrhea
423
Q

HML Pharmacology:
Describe the MOA of Vinca Alkaloids (Vincristine, Vinblastine):

A

Prevents microtubule formation by interfering with tubule binding. Mitotic spindle does not form, M phase does not proceed.

424
Q

HML Pharmacology:
Describe the uses of Vinca Alkaloids (Vincristine, Vinblastine):

A

Leukemias/lymphomas and solid tumors

425
Q

HML Pharmacology:
Describe the side effects of Vinca Alkaloids (Vincristine, Vinblastine):
- 2 points

A
  • ViNcristine causes Neurotoxicity
  • VinBlastine causes bone marrow toxicity
426
Q

HML Pharmacology:
Describe the MOA of Taxanes (Paclitaxel and Docetaxel):

A

Prevent microtubule breakdown by stabilizing tubilin already bound in mitotic spindles. M phase does not complete.

427
Q

HML Pharmacology:
Describe the uses of Taxanes (Paclitaxel and Docetaxel):

A

Solid tumors (eg, ovarian and breast carcinomas)

428
Q

HML Pharmacology:
Describe the side effects of Taxanes (Paclitaxel and Docetaxel):
3 points

A
  • Myelosuppression
  • alopecia
  • hypersensitivity
429
Q

HML Pharmacology:
Describe the MOA of Ixabepilone and Epothilone:

A

Microtubule inhibitors active in the M phase of the cell cycle.

430
Q

HML Pharmacology:
Describe the uses of Ixabepilone and Epothilone:

A

Breast cancers

431
Q

HML Pharmacology:
Describe the side effects of Ixabepilone and Epothilone:
3 points

A
  • Myelosuppression
  • hypersensitivity reactions
  • neurotoxicity (peripheral sensory neuropathy)
432
Q

HML Pharmacology:
Describe the MOA of Cetuximab and Panitumumab:

A
  • Cetuximab is an antibody directed against the extracellular domain of the EGFR, decreasing cellular growth, proliferation, invasion, metastasis, and angiogenesis
  • Panitumumab is a human monoclonal antibody directed against the EGFR and blocks signaling
433
Q

HML Pharmacology:
Describe the uses for Cetuximab and Panitumumab:

A

Colorectal cancer and some head and neck cancers

434
Q

HML Pharmacology:
Describe the side effects of Cetuximab and Panitumumab:

A
  • Cetuximab can cause acneiform skin rash, hypersensitivity infusion reaction, and hypomagnesemia
  • Infusion related reactions less likely with Panitumumab
435
Q

HML Pharmacology:
Name the Anti-BCR-ABL agents:

A

Imatinib, Dasatinib, and Nilotinib

436
Q

HML Pharmacology:
Describe the MOA of anti-BCR-ABL agents:

A
  • Imatinib inhibits BCR-ABL tyrosine kinase specific to CML by blocking the binding site of ADP substrate
  • Nilotinib is a more potent BCR-ABL inhibitor
  • Dasatinib inhibits both BCR-ABL and Src kinases
437
Q

HML Pharmacology:
Describe the uses of anti-BCR-ABL agents:

A

CML with the t(9;22) Ph chromosomal translocation and GI stroll tumors (GISTs)

438
Q

HML Pharmacology:
Describe the side effects of anti-BCR-ABL agents:

A

Mild. Potential interactions exist with other drugs, grapefruit, and St. John’s wort, which are also metabolized by the CYP3A4 system

439
Q

HML Pharmacology:
Describe the uses of Rituximab:
4 points

A
  • Non-hodgkin Lymphoma
  • CLL
  • inflammatory bowel disease
  • RA
440
Q

HML Pharmacology:
Describe the side effects of Rituximab:
5 points

A
  • Infusion reactions
  • tumor lysis syndrome
  • skin and mouth reactions
  • infections
  • increased risk of progressive multifocal leukoencephalopathy
441
Q

HML Pharmacology:
Describe the MOA of anti proteasome, Bortezomib:

A

Interferes with proteasome, which normally control the degradation of proteins regulating cell proliferation. Causes apoptosis in tumor cells.

442
Q

HML Pharmacology:
Describe the uses of anti proteasome, Bortezomib:

A

Multiple myeloma, mantle cell lymphoma

443
Q

HML Pharmacology:
Describe the side effects of anti proteasome, Bortezomib:
4 points

A
  • GI effects
  • asthenia
  • peripheral neuropathy
  • myelosuppression
444
Q

Uworld HML Pathophysiology:
What would you see in peripheral blood smear in hemolytic anemia?

A
  • Reticulocytosis
  • Spherocytes
  • Nucleated RBCs
445
Q

Bootcamp: PNH Pharmacology/ Microbiology
When treating a pt with PNH, what media does the organism more susceptible as result of the medication grow on?

A

Thayer-Martin agar
- a culture medium containing vancomycin, trimethoprim, colistin, and nystatin
- Neisseria spp. would grow

446
Q

Pathophysiology: Abnormal Oxygen Levels
Describe Methemoglobinemia:

A
  • Life threatening condition where the Ferrous (Fe2+)/ heme iron is oxidized to the ferric (Fe3+) state
  • congenital or acquired
  • agents that can cause include primaquine, dapsone, nitrites, and local anesthetics.
  • results in conformational changes causing increased oxygen affinity, reducing oxygen delivery to peripheral tissues, reduces the partial pressure of oxygen (P50) seen as a leftward shift of the Oxygen-Hb dissociation curve
  • signs and symptoms include AMS, cyanosis, and dark brown “chocolate” appearance of the blood after exposure to an oxidizing agent
  • Tx: reducing agents like methylene blue or ascorbic acid (Vitamin C) to convert ferric iron back to ferrous state

Subject Cards (17 decks)

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