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3 Patho > RBC and Bleeding > Flashcards

RBC and Bleeding Flashcards

1
Q

Main hematopoietic organ at 3rd week

A

yolk sac

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2
Q

Main hematopoietic organ at 3rd month

A

liver

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3
Q

Main hematopoietic organ at 4th month

A

BM

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4
Q

Hematopoietic organs at birth:

A

Mainly BM.

Liver is minimal

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5
Q

Normal Hb for male? female?

A

13-16 g/dl

12-14

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6
Q

Normal Hct for male? female?

A

39-48%

33-42%

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7
Q

What are the target molecules in chronic ITP?

A

glycoprotein IIb-IIa
or
glycoprotein Ib-IX

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8
Q

Normal MCV:

A

80 <, Normal < 94

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9
Q

Normal MCHC

A

Normal >= 32

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10
Q

This is a thrombo-hemorrhagic disorder resulting from a complication of a variety of diseases. It is characterized by excessive activation of coagulation, leading to the formation of thrombi in the microvasculature of the body.

A

Disseminated intravascular coagulopathy

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11
Q

Autoantibody blocks B12-IF complex to ileal receptor describes what anemia?

A

Type II anti-IF pernicious anemia

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12
Q

Main symptoms of hereditary spherocytosis

A

anemia; splenomegaly & jaundice

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13
Q

This viral infection can cause aplastic crisis in SC anemia and spherocytosis.

A

Parvovirus B19

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14
Q

IgG-coated RBC binds to monocytes and splenic macrophages. Non-complement. What type of IHA?

A

Warm agglutinin IHA (Warm is “G”reat weather)

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15
Q

Two major variants in G6PD deficiency we studied:

A 
G6PD A- (only mature lack)
G6PD Mediterranean (all RBC lack)
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16
Q

Hemolysis in G6PD def is intra- or extravascular?

A

both

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17
Q

Hemolytic anemia resulting from trauma to RBCs from narrowing or obstruction of microvasculature

A

microangiopathic hemolytic anemia

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18
Q

RBCs lysed in the circulation releasing hemoglobin into the plasma describes what kind of hemolysis?

A

intravascular

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19
Q

Massive destruction of RBC passing through spleen causing SUDDEN splenomegaly leading to hypovolemic shock

A

Sequestration crisis

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20
Q

Complement-mediated hemolytic anemia characterized by increased urine hemosiderin and thrombosis.

A

Paroxysmal nocturnal hemoglobinuria.

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21
Q

Cooley’s anemia is associated with what (specific) anemia?

A

Beta-thalassemia major

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22
Q

Presence of abnormal Hb due to substitution of valine for glutamine at 6th position of Beta chain which leads to low O2 or dehydration precipitates describes which disease’s pathogenesis:

A

sickle cell anemia

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23
Q

Crew cut appearance on xray

A

sickle cell anemia

Beta thalassemia major

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24
Q

Coomb’s test is a major diagnostic criterion for which group of anemias?

A

immunohemolytic anemias (tests antiglobulin)

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25
Q

Impaired DNA synthesis –> maturation of nucleus delayed relative to cytoplasm –> ineffective erythropoiesis resulting in pancytopenia. This describes the pathogenesis of:

A

Megaloblastic anemia

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26
Q

What causes the crew cut appearance of skull x-ray?

A

marrow expansion from increased erythropoeisis

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27
Q

Back pain, hemoglobinuria a few days later.

PBS: bite cells and Heinz bodies

A

G6PD deficiency

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28
Q

Multiple simultaneous painful thrombosis in different organs (dactylitis and acute chest syndrome seen) is known as what? This is a complication of which disease?

A

vaso-occlusive crisis

sickle cell anemia

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29
Q

In G6PD deficiency, what can cause oxidant stress leading to hemolytic anemia:

A

sulfa drugs
infections
fava beans

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30
Q

Which membrane proteins are deficient because of a defective PIGA gene?

A

CD59 (lysis inhibitor)
CD55 (DAF)
and C8

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31
Q

Hemoglobin electrophoresis revealed No HbA, but marked elevation in HbF/HbA2. What anemia does this person have?

A

Beta-thalassemia major

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32
Q

Hemoglobin electrophoresis revealed 4 beta chains. What anemia does this person have?

A

alpha thalassemia (HbH)

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33
Q

IgM-binds to RBC. Complement activated.

A

Cold agglutinin IHA (RBC binding at 0-4 degrees C)

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34
Q

Hemoglobin electrophoresis revealed decreased HbA and slight elevation in HbA2 (>3.5%)? What anemia is this?

A

beta thalassemia minor

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35
Q

This anemia results from an abnormal PIGA gene.

A

Paroxysmal nocturnal hemoglobinuria

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36
Q

Which microcytic, hypochromic anemia is prevalent in Asian and African populations:

A

alpha-thalassemia

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37
Q

Sudden failure of bone marrow to compensate with RBC production despite anemia

A

aplastic crisis

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38
Q

Chronic anemia seen in SLE, CLL, or with certain drugs:

A

Warm agglutinin IHA

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39
Q

Defective proteins in spherocytosis:

A

ankyrin, spectrin, band 3, protein 4.2

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40
Q

Hemoglobin electrophoresis revealed 4 gamma chains. What anemia?

A

alpha thalassemia (Barts)

41
Q

Most common causes: Mycoplasma pneumoniae infections or infectious Mononucleosis

A

Cold agglutinin IHA

COLD icecream = MMM ….. IgM, mycoplasma, mono

42
Q

IgG binds to RBC in cold temp. Hemolysis occurs when blood returns to warmer areas.

A

Paroxysmal cold hemoglobinuria ( Donath-Landsteiner syndrome)
or Cold hemolysin IHA

43
Q

Common causes of Donath-Landsteiner syndrome:

A

(AKA Paroxysmal cold hemoglobinuria or Cold hemolysin IHA)
Mycoplasma pneumoniae,
measles
mumps, other flu-like viruses

44
Q

DIC, TTP, SLE, malignant hypertension can manifest this type of anemia.

A

microangiopathic anemia.

45
Q

RBCs phagocytized by macrophages in the spleen and liver describes what type of hemolysis?

A

extravascular

46
Q

Vitamin B12 and/or folate deficiency can cause what type of anemia?

A

Megaloblastic anemia:

47
Q

Major s/sx of SCA:

A

1) infarction
2) splenomegaly
3) predisposition to infection of encapsulated organ
4) chronic hemolysis

48
Q

How does defective DNA synthesis as seen in megaloblastic anemias cause macrocytosis?

A

There is cell growth (G2) without cell division (M).

49
Q

Explain how pernicious anemia can cause megaloblastic anemia.

A

Autoantibody to Intrinsic Factor or gastric parietal cells –> impaired or deficient IF –> B12 deficiency –> folate d

50
Q

Autoantibody blocks attachment of B12 to IF. What kind of anemia?

A

Type I anti-IF pernicious anemia

51
Q

PBS of hereditary spherocytosis reveals:

A

Spheroid RBC, loss of central pallor

52
Q 
Lab findings include:
Hypersegmented neutrophils
glossitis
increased homocysteine
normal methylmalonate
A

Folate deficient megaloblastic anemia

53
Q 
Lab findings include:
Hypersegmented neutrophils
glossitis
increased homocysteine
increased methylmalonate
A

B12 deficient megaloblastic anemia

54
Q

Diphyllobothrium latum can cause what anemia?

A

B12 deficient megaloblastic anemia

55
Q

What drugs can cause megaloblastic anemia?

A

methotrexate, trimethoprim (anti-folate drugs)

56
Q

Lab findings:
Achlorhydria
(+) serum antibodies
atrophic glossitis

Most likely diagnosis:

A

pernicious anemia

57
Q

Lab findings:
Macrocytic, hyperchromic RBC
Leukopenia with hypersegmented neutrophils
Thrombocytopenia

A

megaloblastic anemia

58
Q

Lab findings of IDA?

A

Lab findings:
PBS = hypochromic microcytic
BM = decreased sideroblasts
Decreased iron and ferritin, increased TIBC

59
Q

Triad of esophageal webs + atrophic glossitis + IDA = ?

A

Plummer Vinson Syndrome

60
Q

Decreased serum iron
Increased serum ferritin
Decreased TIBC

A

Anemia of chronic disease

61
Q

Decreased serum iron
Decreased serum ferritin
Increased TIBC

A

IDA

62
Q

Increased serum iron
Increased serum ferritin
Decreased TIBC

A

Thalassemia

63
Q

Failure or destruction of myeloid stem cells results in this type of anemia:

A

aplastic anemia

64
Q

Hereditary aplastic anemia caused by DNA repair defect:

A

Fanconi’s anemia

65
Q

A primary stem cell defect causing aplastic anemia can be described as:

A

idiopathic

66
Q

Drugs which my cause failure or destruction of myeloid stem cells:

A

chloramphenicol, carbamazepine, phenytoin can cause aplastic anemia

67
Q

Viral agents that can cause failure or destruction of myeloid stem cells:

A

CMV, parvovirus B19, EBV

68
Q

Space occupying lesion that causes displacement and destruction of BM.
BM reveals SOL
PBS reveals pancytopenia and teardrop-shaped RBC

A

Myelophthisic anemia

69
Q

Disease caused by deficiency of ADAMTS 13.

A

Thrombotic thrombocytopenic purpura.

In TTP, deficiency of ADAMTS 13 enzyme results in a decreased degradation of vWF multimers –> excessive activation/aggregation of platelets (thus thrombocytopenia) –> thrombosis

70
Q

Secondary absolute polycythemia is caused by

A

increased EPO secretion

71
Q

This syndrome is a stress polycythemia usually associated with hypertension, obesity, anxiety, smokers

A

Gaisböck syndrome

72
Q

Mutation of this gene causes polycythemia vera

A

tyrosine kinase JAK2

73
Q

Anemia presenting with cyanosis, pruritis, peptic ulcers

A

Polycythemia vera

Cyanosis - unoxygenated RBCs stay in low pressure circulation
Pruritis - increased basophils –> histamine
Peptic ulcer - increased basophil/histamine –> increased HCl secretion

74
Q

This is a complication of polycythemia vera in which bone marrow no longer responds to elevated blood cells, which may develop into leukemia

A

myelofibrosis

75
Q

Infections that may cause vessel wall abnormalities:

A

Meningococcemia, measles, rickettsia, endocarditis

76
Q

Impaired formation of collagen which results in abnormal vessel walls may be caused by

A

scurvy

Ehlers Danlos syndrome

77
Q

Drugs which can cause wall vessel abnormalities:

A

sulfas, penicillin (hypersensitivity)

78
Q

Systemic hypersensitivity disease characterized by arthritis, purpura, and coliky abdominal pain.

A

Henoch–Schönlein purpura

79
Q

Anemia with gait instability with decreased proprioception in lower extremities.

A

B12 deficient anemia

80
Q

Autosomal dominant disorder characterized by dilated, tortuous BVs with thin walls that bleed readily:

A

hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)

81
Q

Self-limited anti-platelet disorder in children following a VIRAL infection

A

acute idiopathic thrombocytopenic purpura

82
Q

Anti-platelet autoimmune disease seen in adults (usually IgG class), especially reproductive females.

A

chronic idiopathic thrombocytopenic purpura

83
Q 
This disease is characterized by the pentad of:
Microangiopathic hemolytic anemia
FEVER
transient NEURO deficits
RENAL failure
thrombocytopenia
A

Thrombotic thrombocytopenic purpura usually ocurs in the fourth decade of life

84
Q

Primary absolute polycythemia is known as and is ____ independent

A

polycythemia vera
erythropoietin

(polycythemia vera is a myeloproliferative disorder with mutations that lead to growth of red cell progenitors independent of EPO)

85
Q

Spontaneous bleeding in mucous membranes and excessive bleeding from wounds. Normal PC, elevated BT and possibly APTT.

A

von Willebrand’s disease

normal PC because platelets are unaffected
elevated PT because platelet plug formation is impaired (decreased vWF)
elevated or nomal APTT because Factor VIII is part of intrinsic pathway (vWF affects FVIII stabiliy)

86
Q

Factor VII abnormality shows this test result

A

Prolonged PT (normal PC, BT, CT)

87
Q

FVIII, IX, XI, XII abnormality shows this test result

A

Prolonged APTT (normal PC, BT, CT)

88
Q

Deficiency of which clotting factor does not cause a bleeding disorder

A

F XII (Hageman)

89
Q

Hemophilia A is caused by

A

F VIII deficiency

90
Q

Hemophilia B is caused by

A

F IX deficiency

91
Q

Bleeding disorder with prolonged PTT and normal PT. It is an X-linked recessive disorder.

A

Hemophilia (A or B)

92
Q

Vit K disease results in deficiency of what factors?

A

II, VII, IX, X

Also seen in liver disease

93
Q

Its pathology is based on hemolysis in the splenic capillaries due to the RBC’s abnormally sphere shape:

A

Hereditary spherocytosis

94
Q

Widespread thrombosis causing fibrinolysis or consumption of clotting factors and platelets which ultimately results in bleeding is seen in what disorder?

A

disseminated intravascular coagulopathy

widespread thrombi then bleeding

95
Q

This clinical syndrome associated with bleeding produces adrenal hemorrhaging because of fibrin thrombi within the microcirculation of the ADRENAL CORTEX

A

Waterhouse-Friderichsen syndrome

96
Q

This clinical syndrome associated with bleeding causes pituitary necrosis after complicated delivery.

A

Sheehan’s postpartum necrosis.

97
Q

Defective platelet adhesion - no GP Ib

A

Bernard Soulier

98
Q

Defective platelet aggregation - no GP IIb/IIIa

A

Glanzmann Thrombasthenia

3 Patho (8 decks)

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