RBC and Bleeding Flashcards
1
Q
Main hematopoietic organ at 3rd week
A
yolk sac
2
Q
Main hematopoietic organ at 3rd month
A
liver
3
Q
Main hematopoietic organ at 4th month
A
BM
4
Q
Hematopoietic organs at birth:
A
Mainly BM.
Liver is minimal
5
Q
Normal Hb for male? female?
A
13-16 g/dl
12-14
6
Q
Normal Hct for male? female?
A
39-48%
33-42%
7
Q
What are the target molecules in chronic ITP?
A
glycoprotein IIb-IIa
or
glycoprotein Ib-IX
8
Q
Normal MCV:
A
80 <, Normal < 94
9
Q
Normal MCHC
A
Normal >= 32
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
11
Q
Autoantibody blocks B12-IF complex to ileal receptor describes what anemia?
A
Type II anti-IF pernicious anemia
12
Q
Main symptoms of hereditary spherocytosis
A
anemia; splenomegaly & jaundice
13
Q
This viral infection can cause aplastic crisis in SC anemia and spherocytosis.
A
Parvovirus B19
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)
15
Q
Two major variants in G6PD deficiency we studied:
A
G6PD A- (only mature lack) G6PD Mediterranean (all RBC lack)
16
Q
Hemolysis in G6PD def is intra- or extravascular?
A
both
17
Q
Hemolytic anemia resulting from trauma to RBCs from narrowing or obstruction of microvasculature
A
microangiopathic hemolytic anemia
18
Q
RBCs lysed in the circulation releasing hemoglobin into the plasma describes what kind of hemolysis?
A
intravascular
19
Q
Massive destruction of RBC passing through spleen causing SUDDEN splenomegaly leading to hypovolemic shock
A
Sequestration crisis
20
Q
Complement-mediated hemolytic anemia characterized by increased urine hemosiderin and thrombosis.
A
Paroxysmal nocturnal hemoglobinuria.
21
Q
Cooley’s anemia is associated with what (specific) anemia?
A
Beta-thalassemia major
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
23
Q
Crew cut appearance on xray
A
sickle cell anemia
Beta thalassemia major
24
Q
Coomb’s test is a major diagnostic criterion for which group of anemias?
A
immunohemolytic anemias (tests antiglobulin)
25
Impaired DNA synthesis --> maturation of nucleus delayed relative to cytoplasm --> ineffective erythropoiesis resulting in pancytopenia. This describes the pathogenesis of:
Megaloblastic anemia
26
What causes the crew cut appearance of skull x-ray?
marrow expansion from increased erythropoeisis
27
Back pain, hemoglobinuria a few days later.
| PBS: bite cells and Heinz bodies
G6PD deficiency
28
Multiple simultaneous painful thrombosis in different organs (dactylitis and acute chest syndrome seen) is known as what? This is a complication of which disease?
vaso-occlusive crisis
| sickle cell anemia
29
In G6PD deficiency, what can cause oxidant stress leading to hemolytic anemia:
sulfa drugs
infections
fava beans
30
Which membrane proteins are deficient because of a defective PIGA gene?
CD59 (lysis inhibitor)
CD55 (DAF)
and C8
31
Hemoglobin electrophoresis revealed No HbA, but marked elevation in HbF/HbA2. What anemia does this person have?
Beta-thalassemia major
32
Hemoglobin electrophoresis revealed 4 beta chains. What anemia does this person have?
alpha thalassemia (HbH)
33
IgM-binds to RBC. Complement activated.
Cold agglutinin IHA (RBC binding at 0-4 degrees C)
34
Hemoglobin electrophoresis revealed decreased HbA and slight elevation in HbA2 (>3.5%)? What anemia is this?
beta thalassemia minor
35
This anemia results from an abnormal PIGA gene.
Paroxysmal nocturnal hemoglobinuria
36
Which microcytic, hypochromic anemia is prevalent in Asian and African populations:
alpha-thalassemia
37
Sudden failure of bone marrow to compensate with RBC production despite anemia
aplastic crisis
38
Chronic anemia seen in SLE, CLL, or with certain drugs:
Warm agglutinin IHA
39
Defective proteins in spherocytosis:
ankyrin, spectrin, band 3, protein 4.2
40
Hemoglobin electrophoresis revealed 4 gamma chains. What anemia?
alpha thalassemia (Barts)
41
Most common causes: Mycoplasma pneumoniae infections or infectious Mononucleosis
Cold agglutinin IHA
| COLD icecream = MMM ..... IgM, mycoplasma, mono
42
IgG binds to RBC in cold temp. Hemolysis occurs when blood returns to warmer areas.
Paroxysmal cold hemoglobinuria ( Donath-Landsteiner syndrome)
or Cold hemolysin IHA
43
Common causes of Donath-Landsteiner syndrome:
(AKA Paroxysmal cold hemoglobinuria or Cold hemolysin IHA)
Mycoplasma pneumoniae,
measles
mumps, other flu-like viruses
44
DIC, TTP, SLE, malignant hypertension can manifest this type of anemia.
microangiopathic anemia.
45
RBCs phagocytized by macrophages in the spleen and liver describes what type of hemolysis?
extravascular
46
Vitamin B12 and/or folate deficiency can cause what type of anemia?
Megaloblastic anemia:
47
Major s/sx of SCA:
1) infarction
2) splenomegaly
3) predisposition to infection of encapsulated organ
4) chronic hemolysis
48
How does defective DNA synthesis as seen in megaloblastic anemias cause macrocytosis?
There is cell growth (G2) without cell division (M).
49
Explain how pernicious anemia can cause megaloblastic anemia.
Autoantibody to Intrinsic Factor or gastric parietal cells --> impaired or deficient IF --> B12 deficiency --> folate d
50
Autoantibody blocks attachment of B12 to IF. What kind of anemia?
Type I anti-IF pernicious anemia
51
PBS of hereditary spherocytosis reveals:
Spheroid RBC, loss of central pallor
52
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Lab findings include:
Hypersegmented neutrophils
glossitis
increased homocysteine
normal methylmalonate
```
Folate deficient megaloblastic anemia
53
```
Lab findings include:
Hypersegmented neutrophils
glossitis
increased homocysteine
increased methylmalonate
```
B12 deficient megaloblastic anemia
54
Diphyllobothrium latum can cause what anemia?
B12 deficient megaloblastic anemia
55
What drugs can cause megaloblastic anemia?
methotrexate, trimethoprim (anti-folate drugs)
56
Lab findings:
Achlorhydria
(+) serum antibodies
atrophic glossitis
Most likely diagnosis:
pernicious anemia
57
Lab findings:
Macrocytic, hyperchromic RBC
Leukopenia with hypersegmented neutrophils
Thrombocytopenia
megaloblastic anemia
58
Lab findings of IDA?
Lab findings:
PBS = hypochromic microcytic
BM = decreased sideroblasts
Decreased iron and ferritin, increased TIBC
59
Triad of esophageal webs + atrophic glossitis + IDA = ?
Plummer Vinson Syndrome
60
Decreased serum iron
Increased serum ferritin
Decreased TIBC
Anemia of chronic disease
61
Decreased serum iron
Decreased serum ferritin
Increased TIBC
IDA
62
Increased serum iron
Increased serum ferritin
Decreased TIBC
Thalassemia
63
Failure or destruction of myeloid stem cells results in this type of anemia:
aplastic anemia
64
Hereditary aplastic anemia caused by DNA repair defect:
Fanconi's anemia
65
A primary stem cell defect causing aplastic anemia can be described as:
idiopathic
66
Drugs which my cause failure or destruction of myeloid stem cells:
chloramphenicol, carbamazepine, phenytoin can cause aplastic anemia
67
Viral agents that can cause failure or destruction of myeloid stem cells:
CMV, parvovirus B19, EBV
68
Space occupying lesion that causes displacement and destruction of BM.
BM reveals SOL
PBS reveals pancytopenia and teardrop-shaped RBC
Myelophthisic anemia
69
Disease caused by deficiency of ADAMTS 13.
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
Secondary absolute polycythemia is caused by
increased EPO secretion
71
This syndrome is a stress polycythemia usually associated with hypertension, obesity, anxiety, smokers
Gaisböck syndrome
72
Mutation of this gene causes polycythemia vera
tyrosine kinase JAK2
73
Anemia presenting with cyanosis, pruritis, peptic ulcers
Polycythemia vera
Cyanosis - unoxygenated RBCs stay in low pressure circulation
Pruritis - increased basophils --> histamine
Peptic ulcer - increased basophil/histamine --> increased HCl secretion
74
This is a complication of polycythemia vera in which bone marrow no longer responds to elevated blood cells, which may develop into leukemia
myelofibrosis
75
Infections that may cause vessel wall abnormalities:
Meningococcemia, measles, rickettsia, endocarditis
76
Impaired formation of collagen which results in abnormal vessel walls may be caused by
scurvy
| Ehlers Danlos syndrome
77
Drugs which can cause wall vessel abnormalities:
sulfas, penicillin (hypersensitivity)
78
Systemic hypersensitivity disease characterized by arthritis, purpura, and coliky abdominal pain.
Henoch–Schönlein purpura
79
Anemia with gait instability with decreased proprioception in lower extremities.
B12 deficient anemia
80
Autosomal dominant disorder characterized by dilated, tortuous BVs with thin walls that bleed readily:
hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
81
Self-limited anti-platelet disorder in children following a VIRAL infection
acute idiopathic thrombocytopenic purpura
82
Anti-platelet autoimmune disease seen in adults (usually IgG class), especially reproductive females.
chronic idiopathic thrombocytopenic purpura
83
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This disease is characterized by the pentad of:
Microangiopathic hemolytic anemia
FEVER
transient NEURO deficits
RENAL failure
thrombocytopenia
```
Thrombotic thrombocytopenic purpura usually ocurs in the fourth decade of life
84
Primary absolute polycythemia is known as and is ____ independent
polycythemia vera
erythropoietin
(polycythemia vera is a myeloproliferative disorder with mutations that lead to growth of red cell progenitors independent of EPO)
85
Spontaneous bleeding in mucous membranes and excessive bleeding from wounds. Normal PC, elevated BT and possibly APTT.
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
Factor VII abnormality shows this test result
Prolonged PT (normal PC, BT, CT)
87
FVIII, IX, XI, XII abnormality shows this test result
Prolonged APTT (normal PC, BT, CT)
88
Deficiency of which clotting factor does not cause a bleeding disorder
F XII (Hageman)
89
Hemophilia A is caused by
F VIII deficiency
90
Hemophilia B is caused by
F IX deficiency
91
Bleeding disorder with prolonged PTT and normal PT. It is an X-linked recessive disorder.
Hemophilia (A or B)
92
Vit K disease results in deficiency of what factors?
II, VII, IX, X
Also seen in liver disease
93
Its pathology is based on hemolysis in the splenic capillaries due to the RBC's abnormally sphere shape:
Hereditary spherocytosis
94
Widespread thrombosis causing fibrinolysis or consumption of clotting factors and platelets which ultimately results in bleeding is seen in what disorder?
disseminated intravascular coagulopathy
| widespread thrombi then bleeding
95
This clinical syndrome associated with bleeding produces adrenal hemorrhaging because of fibrin thrombi within the microcirculation of the ADRENAL CORTEX
Waterhouse-Friderichsen syndrome
96
This clinical syndrome associated with bleeding causes pituitary necrosis after complicated delivery.
Sheehan's postpartum necrosis.
97
Defective platelet adhesion - no GP Ib
Bernard Soulier
98
Defective platelet aggregation - no GP IIb/IIIa
Glanzmann Thrombasthenia
