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HemOnc > Pathology: RBC > Flashcards

Pathology: RBC Flashcards

1
Q

What is in a complete blood count (CBC)?

10

A 
WBC count,
RBC count,
[Hb],
Hematocrit (Hct),
Mean Corpuscular Volume (MCV), 
Mean Corpuscular Hemoglobin (MCH),
Mean Corpuscular Hemoglobin Concentration (MCHC),
Red cell Distribution Width (RDW),
Platelet count,
Mean Platelet Volume (MPV)
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2
Q

Define: WBC count, RBC count, Hct

A

WBC Count: nucleated cells in blood’

RBC Count:  absolute number per microliter (mm3)
                    # of RBC cells in a sample

Hematocrit (Hct): packed red cell volume

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3
Q

Define: MCV, MCH, MCHC

A

Mean Corpuscular Volume (MCV): average size of all RBCs

MCH: average amount of hemoglobin per RBC

MCHC: % per red blood cell
useful if spherocytes in peripheral blood

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4
Q

What is a CBC with differential?

A

Percentage of each WBC found in the peripheral blood

- Wright/Giemsa stained peripheral blood smear used

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5
Q

What is found in the CBC w/ differential using an AUTOMATED counter?

A

In addition to CBC the automated counter gives % and absolute # of:

  • neutrophils
  • eosinophils
  • basophils
  • lymphocytes
  • monocytes
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6
Q

A MANUAL counter is used to find what in a CBC w/ diff?

A

Automated doesn’t separate immature neutrophils, use manual to find:

  • bands
  • metamyelocytes
  • myelocytes
  • promyelocytes
  • blasts
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7
Q

What you’ll find in a peripheral blood smear

A

platelets - little small dots

neutrophils - multi-lobed nucleus; mediate killing for bacteria and fungi

lymphocyte

basophils

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8
Q

More interesting things found in peripheral blood smear

A

**eosinophils - fighting parasites, allergies, asthma, etc… (he likes this one so make sure you know how it looks like)

neutrophilic band - immature neutrophil; if there’s a lot, maybe an infection occurring (“Left Shift”); granules present

monocytes - will have vacuoles present (different from neutrophil band which has granules)

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9
Q

What is Anisocytosis?

A

variability in RBC size

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10
Q

What is Poikilocytosis?

A

variability in RBC shape

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11
Q

What is a Spherocyte?

A

round cells

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12
Q

What is a Dacrocyte?

A

tear-drop cells

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13
Q

What is an Elliptocyte?

A

oblong cells

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14
Q

What is Polychromasia?

A

immature RBCs

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15
Q

What is a Blast?

A

primitive multipotent cells

e.g found in leukemia

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16
Q

In Hematopoiesis, what is the pluripotent stem cell?

A

CD34+

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17
Q

CD34+ pluripotent stem cell divides into:

A

Granulocyte-Monocyte lineage

Lymphoid lineage

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18
Q

What is under the Granulocyte-Monocyte lineage?

A

Myeloid lineage:

  • Erythrocytes (RBCs)
  • Granulocytes (Neutrophils, Eosinophils and Basophils)
  • Megakaryocytes (platelets)

Monocytes

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19
Q

What is under the Lymphoid lineage?

A

Lymphoid cells in Thymus Gland:
CD3+ T-Lymphocytes:
-CD 4+ Helper T cells
-CD8+ Cytotoxic T cells

Lymphoid cells in “Bursa Equivalent”
B-Lymphocytes

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20
Q

Define Anemia?

A

Reduction of [RBC] below normal limits

-decreased [Hb] (

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21
Q

Is anemia symptomatic or asymptomatic?

A

Both!

symptoms include: fatigue, dyspnea

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22
Q

What are the two types of anemia?

A

increased destruction or loss of blood cells: blood loss
hemolytic anemias

problems in the production of blood cells:
decreased hematopoiesis

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23
Q

Anemia of blood loss: What is involved in acute blood loss?

A

NO TIME FOR COMPENSATION

  • Hemorrhage
  • May lead to Hypovolemic Shock
  • Loss of total blood volume more important than acute loss of hemoglobin
  • CBC is usually INITIALLY NORMAL
  • (CBC) Hemoglobin drops over time and also when IVF are given (dilutional effect)
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24
Q

Anemia of blood loss: What is involved in chronic blood loss?

A

Body CAN COMPENSATE if able to

  • Usually GI tract or uterine bleeding
    - (e.g. elderly diverticulosis, menopause)
  • Causes anemia ONLY if blood loss greater than the capacity of bone marrow to replace lost RBCs
    - Intrinsic bone marrow diseases OR nutritional deficiencies:
    - Vitamin B12, Folate, Iron, Pyridoxine, Protein imbalance
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25
Q

Differential Dx for Anemia of decreased hematopoiesis

A

Iron Deficiency** - leading cause (not enough heme being made)
Vitamin B12 Deficiency
Folic Acid/Folate Deficiency
Anemia of Chronic Disease
Anemia of Bone Marrow Stem Cell Failure
-Primary failure (of bone marrow)
-Secondary failure from malignancy (causing bone marrow to be deficient)

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26
Q

Microcytic/Hypochromatic Anemia: slide contains:

A

Neutrophilic bands
neutrophils
hypochromatic cells (Fe deficiency until proven otherwise)
microcytic cells (can tell bc normal RBC is a little bit smaller than a lymphocyte)

Normal RBC might mean anemic pt received blood transplant

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27
Q

MIcrocytic anemia

A

small RBC

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28
Q

Hypochromic anemia

A

pale RBC

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29
Q

What is the gold standard for evaluating blood problems?

A

Bone marrow biopsy

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30
Q

Is a bone marrow biopsy necessary?

A

Not usually

Only if >= 2 cell lines are decreased on a lab result (e.g. RBC, WBC, Plt)

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31
Q

What kind of procedure is a bone marrow biopsy?

A

outpatient that last < 1 hr

not as bad as most ppl think

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32
Q

What can be found in a bone marrow aspirate slide?

A

made from liquid part of bone marrow

Very immature cells
Huge nucleus

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33
Q

What can be found in a bone marrow stain?

A

if there are only a few cells –> bone marrow isn’t healthy

same for too many cells

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34
Q

What is the most common etiology of microcytic/hypochromic anemia?

A

Iron Deficiency (a type of anemia of diminished erythropoiesis)

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35
Q

Iron loss from chronic bleeding:

A 
Menstrual blood loss (reproductive age women)
GI bleeding (age >50 in man or woman)
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36
Q

What are the other etiologies of microcytic/hypochromic?

A
  • Thalassemia
  • Hemoglobinopathy
  • Anemia of Chronic Disease
  • Sideroblastic Anemia
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37
Q

What is the most common nutritional disorder throughout the world?

A

Iron deficiency

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38
Q

Where are Fe2+ found in the body?

A
  • 80% of Fe2+ found in Hb

- 20% found in myoglobin, cytochromes, catalase, others

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39
Q

Name the two Fe storage pool

A

Ferritin and Hemosiderin

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40
Q

What is Ferritin?

A

Protein-Fe complex found mostly in liver, spleen, bone marrow and skeletal muscle

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41
Q

What is Hemosiderin?

A

Brown pigment in macrophages

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42
Q

What is the histologic stain for Fe?

A

PRUSSIAN BLUE

Darker blue spots mean that there is a concentration of Fe in that area

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43
Q

What are the lab test for Fe?

A

Serum Fe: concentration on transferrin (transfers iron in the body)

Serum Ferritin: proportionate to total body Fe stores

Total Iron-Binding Capacity: amount or iron carried by blood
–INDIRECT measure of transferrin

Transferrin Saturation: iron/TIBC x 100

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44
Q 
What are results for Iron Deficiency anemia?
Serum Fe: 
Serum Ferritin:
Serum TIBC:
Transferrin Saturation:
A

Serum Fe: DECREASED
Serum Ferritin: DECREASED
Serum TIBC: INCREASED
Transferrin Saturation: LOW

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45
Q

If you have microcytic anemia + esophageal webs, what’s your dx?

A

Plummer-Vinson Syndrome:

Microcytic, Hypochromic Anemia, Atrophic Glossitis and Esophageal Webs (Upper GI)

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46
Q

What type of anemia is Megaloblastic Anemia?
and
Name the two deficiency

A

Vitamin Deficiencies

Vitamin B12 Deficiency
Folic acid/Folate Deficiency

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47
Q

What is Megaloblastic anemia?

A

Macrocytic

Results from inhibition of DNA synthesis during red blood cell production

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48
Q

What does Vit B12/Cobalamin require for absorption and where is it absorbed?

A

INTRINSIC FACTOR

It’s secreted by PARIETAL CELLS IN STOMACH

INTRINSIC FACTOR/VITAMIN B12 complex is absorbed in the terminal ileum (last 100cm)

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49
Q

What are some problems that impair Vit B12 absorption?

A

Crohn’s Disease
stomach ca
just not enough B12

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50
Q

What two conditions lead to Folic Acid/Folate Deficiency?

A

Alcoholism (Usually a nutritional problem)

Pregnancy (supplementation necessary to avoid neural tube defects)

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51
Q

What causes Vit B12 deficiency?

A

Nutritional deficiency
GI tract malabsorption:
-Surgical rearrangement
-Autoimmune (pernicious anemia–> attack on parietal cells)
-Parasitic infections (fish tapeworm –> eats all the B12)

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52
Q

What are some characteristics of Vit B12 deficiency?

A

Megaloblastic anemia

hypersegmented neutrophils –> nucleus inapporpriately develops

Neuropathy (irreversible)

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53
Q

What causes Anemia of Chronic Disease?

A

Chronic inflammatory diseases may impair the production of erythrocytes in myeloid bone marrow

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54
Q

What are the 3 Major Categories of Inflammatory Diseases that cause Anemia of Chronic Disease?

A

Chronic Infectious Diseases: osteomyelitis, bacterial endocarditis, lung abscess

Chronic Autoimmune Disorders: collagen vascular diseases, inflammatory bowel disease

Malignant Neoplasms

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55
Q

Anemia of Chronic Disease:

Defect of iron incorporation into Hb molecules during erythropoiesis:

A

Problem in mobilizing Fe from storage pool

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56
Q 
What are results are seen in Anemia of Chronic Disease?
Serum Fe: 
Serum Ferritin:
Serum TIBC:
Transferrin Saturation:
A 
Serum Fe:  DECREASED/NORMAL 
Serum Ferritin: NORMAL/INCREASED (inflammation - during acute inflammatory phase ferrtin will go up)
**Serum TIBC:  DECREASED**
-limited ability to "grab up" Fe
Transferrin Saturation: NORMAL

** used to distinguish btwn this and Fe deficiency anemia

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57
Q

What is Pancytopenia?

A

An abnormal reduction in the number of erythroid (RBCs), myeloid granulocytes (WBCs), and megakaryocytes (platelets) in the blood

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58
Q

What is Aplastic anemia?

A

Pancytopenia due to the body’s bone marrow not making enough new blood cells

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59
Q

Etiologies of Aplastic anemia?

A

Mostly drug and chemical exposures: idiosyncratic

Autoimmune

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60
Q

How do you treat aplastic anemia?

A

Recovery doesn’t always occur –> bone marrow transplant

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61
Q

What are the characteristics of idiopathic aplastic anemia?

A

Mechanism of stem cell failure poorly understood

May occur at any age and no gender preference

  • HYPOCELLULAR BONE MARROW
  • ALMOST NO MATURING MYELOID CELLS—JUST SCATTERED LYMPHOCYTES AND PLASMA CELLS
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62
Q

What is Fanconi’s anemia and what anemia does it fall under?

A

Autosomal recessive disease of defects in DNA repair leading to stem cell failure

Aplastic Anemia

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63
Q

What is PNH (paroxysmal nocturnal hemoglobinuria)?

A

Abnormal stem cell produces RBCs that are deficient in proteins attached by Glycosyl phosphatidyl inositol (GPI) anchor
- Loss of CD55/CD59

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64
Q

How do you treat PNH (paroxysmal nocturnal hemoglobinuria)?

A

Eculizumab is treatment

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65
Q

A rare form of bone marrow failure leading to erythroid aplasia only

A

Pure Red Cell Aplasia
- granulopoiesis and thrombopoiesis aren’t effected

Type of Aplastic anemia

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66
Q

What disease is associated with Aplastic anemia?

A

Mostly idiopathic but…

THYMOMAS of anterior mediastinum

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67
Q

What is Myelophthisic Anemia?

A

(Overcrowding inside bone marrow)
Bone Marrow space replaced by other components
-Neoplasm
-Fibrosis

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68
Q

What is seen on slides for Myelophthisic Anemia?

A

Target cells and Dacrocytes

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69
Q

Differential Dx of Myelophthisic Anemia?

A
  • Metastatic carcinoma
  • Myelofibrosis with myeloid metaplasia,
  • Plasma cell myeloma
  • Malignant lymphoma
  • Osteosclerosis
70
Q

What are the two types of organ failure associated with anemia of diminished erythropoiesis?

A

Chronic Renal Failure:

  • Lack of erythropoietin production
  • Often proportional to degree of renal failure

Diffuse liver disease:

  • end-stage liver cirrhosis
  • Infections (usually viral hepatitis)
  • Toxins (acetaminophen, mushrooms)
71
Q

What is hemolytic anemia?

A

Condition in which red blood cells are destroyed and removed from the bloodstream before their normal lifespan is over.

72
Q

What are schistocytes?

A

Fragmented RBC cuased by physical damage to the circulating cell

on slides looks like “dented helmets”
seen in hemolytic anemia

73
Q

Increased destruction of RBCs is…

A

Hemolysis

RBC usually live for 120 days

74
Q

What are the causes of Hemolytic anemia?

A
  • Mechanical Injury (valves)
  • Autoimmune complement fixation on surface membrane
  • Exogenous toxic Factors
  • RBC membrane abnormalities
  • Enzyme abnormalities
75
Q

Clinical characteristics of intravascular hemolysis?

A 
Anemia symptoms (fatigue, dyspnea)
Jaundice (destruction of Hb-->bilirubin)
76
Q

What is Intravascular hemolysis?

A

When something is wrong with the RBC itself

77
Q

How do the lab results look like for Intravascular hemolysis?

A 
DECREASED Hb
DECREASED haptoglobin (binds Hb to prevent toxicity)
INCREASED LDH (lactate dehydrogenase)
INCREASED Reticulocytes (immature RBCs)
78
Q

What is extravascular hemolysis?

A

RBC is “chewed up” in organ

e.g Liver, spleen –> sinusoidal organs

79
Q

Clinical characteristics of Extravascular hemolysis?

A
  • Anemia (fatigue, dyspnea)
  • Sometimes hepatomegaly, splenomegaly
  • Less hemoglobinuria
  • Fewer laboratory abnormalities
  • Other comorbidities
80
Q

Intrinsic causes of hemolytic anemia

A
  • Defects in RBC membranes: hereditary
  • Defects in Hb production: sickle-cell, thalassemia
  • Defective RBC metabolism: G6PD deficiency, pyruvate kinase deficiency
81
Q

Extrinsic causes of hemolytic anemia

A
  • Infections (mycoplasma pneumonia)
  • Autoimmune (SLE, RA, Hodgkin’s disease, CLL, PNH, HUS, TTP)
  • Hypersplenism
  • Toxins (lead, heavy metals)
  • Trauma
    • -Defective heart valves
    • -“March hemoglobinuria”
82
Q

What is Hereditary Spherocytosis?

A

Inherited disorder of erythrocytes

Abnormal membranes and shape of RBCs
-Mutated Spectrin (most common), ankyrin, band 3 protein, or protein 4.2

Seen most commonly in Northern European descendants (1 in 5,000)
-Usually autosomal dominant

83
Q

What is a spherocyte?

A
  • RBC with little of no central pallor.
  • No longer biconcave.
  • Smallest possible surface area-to-volume ratio.
  • Less deformable
    • -therefore more likely to be sequestered in splenic sinusoids
  • Premature destruction
84
Q

What is the gold standard lab test and result for Hereditary Spherocytosis?

A

Osmotic fragility

Abnormally increased

85
Q

What are the clinic characteristics of Hereditary Spherocytosis?

A
  • Anemia
  • Splenomegaly
  • Jaundice

Severity differs among pts

86
Q

What is Hemolytic Crisis and what condition is it associated with?

A

Sudden onset of massive hemolysis, fever, abdominal pain, jaundice, low blood pressure, shock

Hereditary Spherocytosis

87
Q

What is Hereditary Spherocytosis: Aplastic Crisis?

A
  • Temporary suppression (shut down) of bone marrow production
  • Worsening of anemia
  • Later: Disappearance of reticulocytes from peripheral blood
88
Q

What is Aplastic Crisis associated with?

A

Parvovirus Infection

89
Q

What is Polychromasia and which condition does it appear in the lecture?

A

disorder where there is an abnormally high number of RBC found in the bloodstream as a result of being PREMATURELY released from the bone marrow during blood formation
- sign of immature RBC –> Reticulocytes are increased

has purple specs –> nuclear material

90
Q

Is Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD deficiency) an intrinsic or extrinsic defect?

A

Intrinsic defect of hemolytic anemia usually caused by infection and drug exposure

  • Anti-malarial
  • Sulfa-drugs
91
Q

What does G6PD do?

A

Key enzyme in the hexose monophosphate shunt (HMP) –> HMP produces NADPH needed for glutathione reductase

–NADPH removes reactive oxygen species from RBCs

92
Q

What does G6PD deficiency do?

A 
Causes oxidation of globin chains which leads to DENATURED HEMOGLOBIN (Heinz bodies) 
and 
after 2-3 days of oxidative stress 
- acute intravascular hemolysis
- decreased hemoglobin
93
Q

In what population does G6PD deficiency affect?

A

Deficiency is present in ~ 10% of African-Americans or those of Mediterannean ancestry

  • Autosomal recessive inheritance
  • Variable phenotypic expression of the disease
94
Q

Heinz Bodies cause …

A

splenic macrophages to “bite” out section of RBC membrane

- ultimately leading to formation of spherocytes

95
Q

Hemolytic Anemia: Intrinsic defects

What is Hemoglobinopathies?

A

Structural abnormalities in alpha or beta hemoglobin

- Most common in beta-globin chain

96
Q

What are the two clinically significant Hemoglobinopathies?

A

Thalassemia, Sickle-cell Anemia

97
Q

What and where is the mutation for Sickle Cell Disease and Trait?

A

Glutamate –> Valine substitution at position #6 of b-globin chain.

Autosomal Recessive

98
Q

What group of people do Sickle Cell Disease mostly affect?

A

Predominant in African-American population

- Confers resistance to malaria (if heterozygous)

99
Q

Heterozygous Sickle Cell Trait or Disease

A

Trait

< 50% of hemoglobin is hemoglobin S

100
Q

Homozygous Sickle Cell Trait or Disease

A

Disease

> 50% of hemoglobin is hemoglobin S

101
Q

In Sickle Cell anemia HbS undergoes …, in … condition?

A

polyermerization… low oxygen tension (ie. Denver)

102
Q

HbS polyererization does what exactly in low O2 tension?

A

Converts normal hemoglobin from freely flowing liquid to viscous gel
- causes RBC membrane to undergo “Sickle” malformation

At first reversible but later irreversible

103
Q

When does Chronic Hemolytic Anemia occur in relation to sickle cell?

A

Presence of irreversibly sickled RBCs

104
Q

Irreversible sickled RBCs also results in what?

A

Occlusion of small blood vessels resulting in ischemic tissue damage

  • this is why pt are in pain
  • “gumming up” of vessels
105
Q

When does the first sickling episode begin?

A

occurs in homozygote after “SWITCH” from producing normal hemoglobin F to attempting to produce normal hemoglobin A1 (but this can’t be made)
- at about 5-6 months after birth

106
Q

What facilitates sickling?

A

Dehydration and acid pH

- high altitiudes

107
Q

What occurs in Sickle Cell Disease and Trait?

A
  • Elevated peripheral destruction of RBCs
  • Average RBC survival in peripheral blood directly proportional to number of sickled RBCs
      • approximately 20 days
  • Hyperplastic Bone Marrow with Erythroid Hyperplasia with extramedullary hematopoiesis
      • splenomegaly –> trying to get rid of sickle cells, it autoinfarcts
108
Q

Clinical features of Sickle cell Disease and trait?

A
  • Splenomegaly in early course of disease in children
  • Erythrostasis in spleen results in repeated infarction of spleen: “AUTO-SPLENECTOMY”
  • Infarction secondary to RBC occlusion often in bone, brain, kidney, liver and retina – vaso-occulsive complication
  • Chronic cutaneous leg ulcers in adults
  • Pigment calculi (gallstones) in gallbladder
  • Hyperbilirubinemia during active hemolysis
109
Q

Which two bacterial infections are the most common etiology of DEATH in CHILDREN with HOMOZYGOUS sickle cell anemia?

A

Streptococcus pneumoniae
Haemophilus influenzae

Both are encapsulated

110
Q

What is Sickle cell crisis associated with?

A

Associated with infection, dehydration and acidosis (favor sickling)

Associated with severe pain

111
Q

What organs are involved in Vaso- occlusive episdoes?

A

bones, lungs, liver, brain spleen and penis

Priapism in male patients secondary to vascular engorgement of penis

112
Q

What is involved in aplastic crisis in relation to sickle cell?
and
what infection is usually associated?

A

temporary cessation of bone marrow cell production –> it stops working

often associated with PARVOVIRUS INFECTION

113
Q

what is Sequestration Crisis?
and
what clinical conditions are seen in CHILDREN still with spleen?

A

sequestration of most of blood volume in spleen

  • leads to SPLENOMEGALY, HYPOGLYCEMIA and SHOCK in severe cases only in children who still have spleen
114
Q

What is the definitive Dx for sickle cell

A 
HEMOGLOBIN ELECTROPHORESIS 
or 
fetal DNA analysis 
or 
metabisulfite screening test
115
Q

Sickel cell:

What does chronic hypoxia lead to?

A

impairment of growth and chronic organ damage to spleen, heart and lungs

116
Q

at what age do Homozygous Sickle cell disease usually live up to?

A

30s or 40s but many die in first three years of life secondary to bacterial infections

117
Q

What condition can develop in pt with sickle cell secondary to Salmonella infection?

A

Osteomyelitits

118
Q

What is Thalassemias?

A

ABNORMALLY DECREASED RATE OF PRODUCTION OF 1 or more GLOBIN CHAINS in a Hb tetramer

119
Q

What is a-Thalassemias?
and
Seen in what population?

A

decreased rate of production of a-globin chains allow excess production of beta, gamma and delta-globin chains

a-Thalassemias are DELETIONS of genes coding for a-globin chains

Usually seen in patients of Asian descent

120
Q

What is b-Thalassemias?
and
Seen in what population?

A

decreased rate of production of beta-globin chains allows for excess production in alpha-globin chain

Usually seen in patients of African/Mediterranean descent

121
Q

What is seen in a Hb structure?

A

Tetrameric protein:

2 alpha globin chains, 2 beta chains, heme for each chain, Fe2+ for each heme

122
Q

How many beta globin GENEs and alpha globin GENEs are there in a chain?

A

2 beta globin genes

4 alpha globin genes

123
Q

in patients with Sickle cell which globin chain is increased?

A

gamma

bc alpha and beta is abnormal

124
Q

What is the globin changes over time?

A

gestational age: alpha majority, gamma

fetal age: make more beta, alpha is consistent throughout

postnatal age: alpha and beta dominate

125
Q

Describe the mech. of disease of Thalassemia?

A

Excess a, b, g, or d chains precipitate out as tetrameric complexes (a4, b4, g4, or d4) –>
Lead to RBC membrane damage and early apoptotic necrosis

INEFFECTIVE ERYTHROPOIESIS within bone marrow and decreased RBC survival in peripheral blood

126
Q

What is b-Thalassemia Major?

A

beta-0: if homozygous (both beta genes are reduced/absent), essentially no beta-globin chain production

  • not making enough beta
127
Q

What is b-Thalassemia Minor?

A

beta-+: if homozygous or heterozygous (1 bete gene is missing or dsyfxnal) will still be some beta-globin chain production

  • you’re making enough just less than normal
128
Q

Which Thalassemia show up clinically?

A

b-Thalassemia Major

b-Thalassemia Minor is clinically silent
-decrease Hb, slight anemia

129
Q

what are some characteristics of b-Thalassemia Minor?

A
  • Usually low normal hemoglobin level or slight anemia
  • RBC count often elevated BUT low MCV (small RBCs)
  • Provides some protection against malaria
130
Q

what are some characteristics of b-Thalassemia Major?

A
  • Essentially no Hemoglobin A1 production
  • Increase in Hemoglobin F
  • Variable levels of Hemoglobin A2
131
Q

What is seen on skull xray for Thalassemia?

A

“crew cut” appearance in severe Thalassemia

-in young pts, bone marrow will go into spasms and skull bones will go though hematopoeisis

132
Q

What are the clinical findings of 1 deletion alpha chain gene ?

A

Silent Carrier, no physical abnormality

133
Q

2 deletion alpha chain gene?

A

a-Thalassemia Trait, like Beta-Thalassemia Minor

134
Q

3 deletion alpha chain gene?

A

Hemoglobin H (tetramer of beta chains) Disease, severe disease

135
Q

4 deletion alpha chain gene?

A

Hydrops Fetalis, incompatible with life—death in utero,

Presence of hemoglobin Barts (tetramer of gamma chains)

High oxygen affinity prevents oxygen delivery to tissues

136
Q

What is a target cell?

A

Aka: Codocytes - abnormal red blood cells with a bullseye appearance in hematology.

Usually seen in defective globin chain synthesis, (eg. hemoglobinopathies, thalassemia)

137
Q

Which defect is autoimmune hemolytic anemia (AIHA)?

A

Extrinsic defect

Warm (most common) vs Cold

138
Q

What do you find in the presence of Ig on teh surface of RBCs?

A
  • Autoantibodies

- Coomb’s positive (Direct anti-globulin test)

139
Q

why is direct coomb’s test done?

A

It finds antibodies attached to your red blood cells.

-The antibodies may be those your body made because of disease or those you get in a blood transfusion.

140
Q

What is a Direct Coomb’s Test?

A

Test done on a sample of red blood cells from the body. It detects antibodies that are already attached to red blood cells.

141
Q

What is an Indirect Coomb’s Test?

A

Test done on a sample of the liquid part of the blood (serum). It detects antibodies that are present in the bloodstream and could bind to certain red blood cells, leading to problems if blood mixing occurs.

  • used more in transfusion
142
Q

What is Coomb’s reagent?

A

antihuman antibodies

143
Q

How does Direct Coomb’s work?

A
  • blood sample taken from AIHA pt
  • after pts washed RBCs are incubated with antihuman Ab then RBC agglutinate
  • antihuman Ab form links btwn RBC by binding to the Ab already on the RBC
144
Q

How does Indirect Coomb’s work?

A
  • get recipient’s serum containing Ab (Ig’s)
  • Add donor’s blood sample to the serum
  • recipient’s Ig’s form ab-antigen complex with donor’s RBC
  • Coomb’s Ab (antihuman Ig) add to the solution
  • agglutination fo RBC bc human Ig’s are attached to RBC
145
Q

what is the primary cause of Warm AIHA?

A

60% of cases are idiopathic

40% are secondary to drugs or other disease

146
Q

Which auto-Abs dominate on a RBC in warm AIHA?

A

IgG (rarely IgA)

147
Q

what is the primary cause of Warm AIHA?

A

60% of cases are idiopathic

40% are secondary to drugs or other disease

148
Q

Which auto-Abs dominate on a RBC?

A

IgG (rarely IgA)

149
Q

In warm AHIA, hemolysis is … ?

A

extravascular (removal of opsonized RBC’s in spleen)

  • transforms normally biconcave RBCs into spherocytes
  • splenomegaly results over time
150
Q

Dx of AHIA?

A

osmotic fragility

151
Q

What are the 3 immunologic mechanisms for warm AIHA?

A

Hapten Model

Immune-Complex Model

Autoantibody Model

152
Q

what is Hapten Model?

A

Drug combines with RBC membrane to form a new Ag to which Ab binds
- related to LARGE DOSES of antibiotics

153
Q

What is Immune-Complex Model?

A

Drug binds to plasma protein and DRUG-PROTEIN COMPLEX serves as Ag for Ab binding
- Ag-Ab complex is non-specifically attached to RBC membrane

154
Q

what is Autoantibody Model?

A

Drug somehow causes the production of Abs against INTRINSIC RBC Ags

155
Q

Which defect is Cold Agglutinin Immune Hemolytic Anemia?

A

Extrinsic

IgM auto-antibodies bind to RBCs

  • IgM is pentameric and binds best at low temps
  • Exacerbated in winter months in northern climates
156
Q

How do the symptoms occur?

A
  • agglutination of RBCs with fixation of complement in distal parts of the body where temp < 30C
  • hemolysis occurs when RBCs return to 37C
  • IgM is released from RBCs BUT complement is still on RBC
  • which makes them more susceptible to phagocytosis in spleen and/or liver –> vas. obstruct, raynauds
157
Q

What are the clinical symptoms of cold AIHA?

A

vascular obstruction

Raynaud’s Phenomenon

158
Q

How do the symptoms occur?

A
  • agglutination of RBCs with fixation of complement in distal parts of the body where temp < 30C
  • hemolysis occurs
  • IgM is released from RBCs BUT complement is still on RBC
  • which makes them more susceptible to phagocytosis in spleen and/or liver –> vas. obstruct, raynauds
159
Q

Does Cold AIHA require specialized colleciton and analysis?

A

YES

160
Q

Trauma to RBCs is a … defect?
and
what does it lead to?

A

Extrinsic

Fragmentation of RBCs with trauma may lead to INTRAVASCULAR HEMOLYSIS

161
Q

Mechanism of autoantibody production in cold AIHA is …

A

…poorly understood

162
Q

Trauma to RBCs is a … defect?
and
what does it lead to?

A

Extrinsic

Fragmentation of RBCs with trauma may lead to INTRAVASCULAR HEMOLYSIS

163
Q

In PNH, what does the GPI protein do?

A

GPI proteins normally inactivate complement

Abnormal RBCs are sensitive to lysis from endogenous complement

Paroxysmal Noctural Hemoglobinuria (PNH):
Glycosyl phosphatidyl inositol (GPI)

164
Q

Differential Dx of Trauma to RBCs?

A

-Heart Valve Prostheses Defect
-Deposition of FIBRIN in Microvasculature
=TTP, HUS, DIC, malignancy, hypertension, renal cortical necrosis, SLE, metastatic adenocarcinoma

165
Q

In PNH, what does the GPI protein do?

A

GPI proteins normally inactivate complement

Paroxysmal Noctural Hemoglobinuria (PNH):
Glycosyl phosphatidyl inositol (GPI)

166
Q

Which sequellae of PNH has ~ 50% fatal rate?

A

Hypercoagulable State with repeated episodes of venous thrombosis involving hepatic, portal or cerebral veins

167
Q

What are the complications of PNH?

A

Hemosiderinuria leads to Iron Deficiency anemia –> lead to pancytopenia

pancytopenia: An abnormal reduction in the number of RBC, WBC, plt

168
Q

What is a possible outcome of PNH?

A

Hypercoagulable State with repeated episodes of venous thrombosis involving hepatic, portal or cerebral veins

Fatal in ~ 50% of cases

169
Q

What are other possible outcome of PNH?

A
  • Infections related to granulocyte (neutrophil) dysfunction or granulocytopenia may also occur
  • Clonal abnormality related to other stem cell disorders (aplastic anemia/acute leukemia)
  • Usually chronic disease with median survival of 10 years if untreated
170
Q

What other disease are associated with hemolysis?

A

HELLP – pregnant women
HUS – bacterial infections - E. coli O157:H7
TTP – thrombotic thrombocytopenic purpura
- Defect or auto-antibodies to ADAMTS13

HemOnc (8 decks)

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