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UWorld Incorrects > Hematology > Flashcards

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?

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
  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
  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 condition associated with schistocyte?

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:

A
  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
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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

99
Q

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

A
  • growth retardation
  • death at an early age
  • cardiac failure
  • other organ damage from hemosiderosis
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
101
Q

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

A

Only one alpha globin gene affected; asymptomatic

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
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
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
105
Q

HML Pathology:
How would you diagnose alpha-thalassemia?

A
  • Hb Electrophoresis
  • PBS: microcytic and hypochromic
  • molecular genetic testing showing alpha-thalassemia traits
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.
107
Q

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

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

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

A
  • Miners
  • Industrial workers (exposure to lead batteries)
  • houses built on or before 1978
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
110
Q

HML Pathology:
How would you diagnose lead poisoning?

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

HML Pharmacology:
How would you treat lead poisoning?

A
  • Children: Succimer
  • Dimercarprol and ethylenediaminetetraacetic acid (EDTA)
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
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
114
Q

HML Pharmacology:
How do you treat sideroblastic anemia?

A

Pyridoxine (vitamin B6) cofactor for ALAS

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

116
Q

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

A

Alcoholism, liver disease, hypothyroidism, and anticancer drugs (5-FU)

117
Q

HML Pathology:
How can vitamin B12 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)
118
Q

HML Pathology:
How can folic acid deficiency be caused?

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)
119
Q

HML Pathology:
Describe subacute combines degeneration:

A

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

120
Q

HML Pathology:
How does a megaloblastic anemia present?

A

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

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

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
123
Q

HML Pathology:
How does a pernicious anemia develop?

A

anti-intrinsic factor antibodies

124
Q

HML Pathology:
What is a Schilling test used for?

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)

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.

126
Q

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

A

Black, Middle Eastern/ Mediterranean populations

127
Q

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

A

Both intravascular and extravascular

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
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
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)
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
132
Q

HML Pathology:
What is the prognosis in G6PD deficiency?

A
  • healthy if avoiding predicating factors
  • complicated by neonatal jaundice leading to kernicterus
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
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
135
Q

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

A

Autosomal dominant

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
137
Q

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

A

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

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
139
Q

HML Pathology:
How is hereditary spherocytosis diagnosed? 5 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)
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
141
Q

HML Pathology:
What is the prognosis of Hereditary Spherocytosis? 3 points

A
  • aplastic crisis triggered by parvovirus B19
  • infections after splenectomy, cholelithiasis (bilirubin gallstones)
  • hemosiderosis from multiple blood transfusions
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)
143
Q

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

A
  • Warm antibody
  • Cold agglutinin
  • erythroblastosis fetalis
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)
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
146
Q

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

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

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

A
  • SLE
  • Hodgkin lymphoma
  • chronic lymphocytic leukemia (CLL)
  • Drugs: alpha-methyldopa, PCN, cephalosporins
148
Q

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

A
  • spherocytosis
  • positive direct Coombs test
149
Q

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

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

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

A

Distal body parts

151
Q

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

A

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

152
Q

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

A
  • Episodic hyperbilirubinemia: hemoglo binemia 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
153
Q

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

A
  • steroids
  • IVIG
  • plasmapheresis
154
Q

HML Pathology:
What two settings can Erythroblastosis Fettles occur?

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

HML Pathology:
How does Erythroblastosis Fetalis present?

A

Fetal hemolytic anemia

156
Q

HML Pathology:
How do you diagnose Erythoblastosis Fetalis?

A

Test maternal and fetal blood for presence of antibodies

157
Q

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

A
  • Stillbirth
  • hydros fetalis (fetal heart failure
  • kernicterus
158
Q

HML Pathology:
What is kernicterus?

A

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

159
Q

HML Pathology:
How do you prevent Erythroblastosis fetalis?

A

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

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
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
162
Q

HML Pathology:
What are the mechanisms behind anemia of chronic disease? 3 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
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)

164
Q

HML Pathology:
How does Anemia of chronic disease present?

A

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

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
166
Q

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

A
  • administer EPO
  • treat underlying condition
167
Q

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

A
  • complement fixation
  • mechanical injury
  • or toxins
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
169
Q

HML Pathology:
What causes an extravascular hemolytic anemia?

A

Due to RBC injury, antibody attachment, structural or membrane defects leading to abnormal shape, and decreased ability to leave cords of Billroth and enter sinusoids of spleen

170
Q

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

A

Inside of mononuclear phagocytic cells in the spleen (RES)

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
172
Q

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

A

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

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)

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
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

176
Q

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

A

Hypersplenism

177
Q

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

A

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

178
Q

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

A

Plasmodia falciparum malaria

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?

A

HbF

188
Q

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

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? 4 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 (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 is 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 caused of Aplastic Anemia? 6 points

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

A
  • Porphobilinogen
  • urinary coporphobilinogen
  • delta- aminolevulinic acid (ALA)
  • 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
  • Uroporyphyrin (large, insoluble molecule and photosensitive)
  • Iron
  • Transferrin
223
Q

HML Pathology:
How does Porphyria Cutanea Tarda? 5 points

A
  • Cutaneous bull
  • 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?

A

collagen

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
A

UWorld Incorrects (4 decks)

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