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DIT Cards > Heme Review > Flashcards

Heme Review Flashcards

1
Q

Which coagulation factor is deficient in hemophilia A? What’s the inheritance pattern?

A

factor VIII; X-linked recessive

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2
Q

Which coagulation factor is deficiency in hemophilia B? What is the inheritance pattern?

A

factor IX; X-linked recessive

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3
Q

What is the clinical consequence of a deficiency in either protein C or protein S?

A

increased coagulation

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4
Q

What factors are part of the intrinsic pathway?

A

8,9,11,12

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5
Q

What factors are part of the extrinsic pathway?

A

7

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6
Q

What factors are part of the common pathway?

A

1,2,5,10,13

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7
Q

What factors does the PT measure?

A

1,2,7,10 (extrinsic and common)

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8
Q

What factors does the PTT measure?

A

everything except 7 and 13 (intrinsic and common)

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9
Q

What lab test is used to monitor adequate anticoagulation in a patient taking heparin? Warfarin?

A

heparin - monitor PTT

warfarin - monitor PT and INR

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10
Q

What is the treatment for an overdose of heparin? Warfarin?

A

heparin - protamine sulfate

warfarin - Vit K, FFP, PCC (the last two only if active bleeding)

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11
Q

What is the treatment for heparin-induced thrombocytopenia?

A

stop heparin, switch to a direct thrombin inhibitor

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12
Q

What is the presentation of erythroblastosis fetalis?

A

Clinical features in neonate:

  • anemia due to hemolysis of RBCs by maternal antibodies
  • jaundice -> possible kernicterus
  • hydrops fetalis (generalized fetal edema)
  • intrauterine death
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13
Q

What allows RBCs to change shape as they pass through vessels?

A

biconcave shape (anucleate) d/t spectrin

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14
Q

Which pathologic form of RBC would you see with lead poisoning?

A

basophilic stippling

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15
Q

Which pathologic form of RBC would you see with G6PD deficiency?

A

Heinz bodies, bite cells

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16
Q

Which pathologic form of RBC would you see with DIC?

A

schistocytes

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17
Q

Which pathologic form of RBC would you see with abetalipoproteinemia?

A

acathocytes (spur cells)

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18
Q

Which pathologic form of RBC would you see with asplenia?

A

Howell-Jolly bodies, target cells

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19
Q

Where does fetal erythropoiesis take place? In which adult bones does erythropoiesis take place?

A

Fetal - yolk sac, liver, spleen, BM

Adult - axial skeleton (sternum, ribs, pelvis, vertebrae)

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20
Q

What are some of the different causes of polycythemia?

A

decreased plasma volume (dehydration, burns)
decreased O2 - pulmonary problems, heart Dz, high altitude
increased EPO - ectopic tumors (PRHH - pheochromocytoma, renal cell carcinoma, hemangioblastoma, hepatocellular carcinoma)
polycythemia vera

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21
Q

What are the hematologic and non-hematologic findings in a patient with lead poisoning?

A

hematologic - basophilic stippling, microcytic anemia
non-hematologic - encephalopathy, memory loss, HA, wrist/foot drop, lead lines on gingival, lead lines on bones (kids), abdominal colic, renal failure

22
Q

What is the rate-limiting step of heme synthesis? What is required for the reaction?

A

delta-ALA synthase, Vit B6

23
Q

What inhibits delta-ALA synthase?

A

glucose and heme

24
Q

What enzyme deficiency causes acute intermittent porphyria?

A

uroporphyrinogen-1-synthase

25
Q

What enzyme deficiency causes porphyria cutanea tardy?

A

uroporphyrinogen decarboxylase

26
Q

What enzyme deficiencies can cause lead poisoning?

A

delta-ALA-dehydratase and ferrochelatase

27
Q

What is the serum iron, TIBC, ferritin, and % transferring saturation (decreased or increased) for iron deficiency anemia?

A

serum iron - decreased
TIBC - increased
ferritin - decreased
% transferrin saturation - decreased (<12%)

28
Q

What is the serum iron, TIBC, ferritin, and % transferring saturation (decreased or increased) for anemia of chronic disease?

A

serum iron - decreased
TIBC - decreased
Ferritin - normal or increased
% transferrin saturation - normal (>18%)

29
Q

What is the serum iron, TIBC, ferritin, and % transferring saturation (decreased or increased) for hemochromatosis?

A

serum iron - increased
TIBC - decreased
ferritin - increased
% transferrin saturation - increased

30
Q

What is the serum iron, TIBC, ferritin, and % transferring saturation (decreased or increased) for sideroblastic anemia?

A

serum iron - increased
TIBC - decreased
ferritin - increased
% transferrin saturation - normal or increased

31
Q

What test can be used to diagnose beta thalassemia minor?

A

hemoglobin electrophoresis to look for greater than normal HgA2

32
Q

What should you rule out in a man over 50 with new onset iron deficiency anemia?

A

colon cancer

33
Q

Causes of hypo chromic, microcytic anemia

A 
iron deficiency anemia
alpha-thalassemia
beta-thalassemia
anemia of chronic Dz
lead poisoning
34
Q

Skull x-ray shows a “hair-on-end” appearance

A

marrow hyperplasia -> beta thalassemia or sickle cell Dz

35
Q

What are the causes of aplastic anemia?

A

radiation/drugs -> chloramphenicol and cancer drugs
viruses
fanconi syndrome
idiopathic

36
Q

A patient is diagnosed with a microcytic, megaloblastic anemia. What is the danger of giving folate alone?

A

will not treat a potential Vit B12 deficiency, but it will mask the anemia

37
Q

microcytic anemia and swallowing difficulty and glossitis

A

Plummer-Vinson syndrome

38
Q

Microcytic anemia with >3.5% HgA2

A

beta thalassemia minor

39
Q

macrocytic anemia and hypersegmented neutrophils

A

folate/B12 def -> megaloblastic anemia

40
Q

megaloblastic anemia not correctable by B12 or folate supplements

A

oratic aciduria

41
Q

microcytic anemia and basophilic stippling

A

lead toxicity

42
Q

microcytic anemia reversible with B6 supplements

A

sideroblastic anemia

43
Q

HIV-positive patient with macrocytic anemia

A

Zidovudine

44
Q

normocytic anemia and elevated creatinine

A

chronic kidney disease

45
Q

Acute intermittent porphyria

A

autosomal dominant
porphobilinogen deaminase deficiency (AIA uroporphyrinogen I synthase)
Sx - 5P’s - painful abdomen, port wine-colored urine, polyneuropathy, psychological disturbances, precipitated by drugs (barbiturates, seizure drugs, rifampin, metoclopramide)
Tx 0 glucose and heme, which inhibit ALA synthase

46
Q

Porphyria cutanea tarda

A

autosomal dominant
uroporphyrinogen decarboxylase deficiency
Sx - tea-colored urine (uroporphryin build up), blistering cutaneous photosensitivity and hyperpigmentation, hypertrichosis, increased liver function tests; exacerbated by alcohol consumption, and is associated with HepC

47
Q

sideroblastic anemia

A

causes: genetic, alcohol is most common reversible acquired cause, also Vit B6 deficiency, copper deficiency, and isoniazid
Lab findings: increased iron, increased ferritin, ringed sideroblasts (with iron-laden, Prussian blue-stained mitochondria) seen in BM
Tx - B6

48
Q

alpha-thalassemia

A

defect: alpha globin gene deletions -> decreased alpha-globin synthesis
prevalent in Asian and African populations
4 allele deletion: Hb Barts, incompatible with life, hydrops fetalis (gamma4 Hgb)
3 allele deletion: HbH disease; very little alpha-globin; beta4
2 allele deletion: less clinically severe anemia
1 allele deletion: no anemia

49
Q

beta-thalassemia

A

decreased beta globin synthesis; prevalent in mediterranean populations
beta-thalassemia minor - beta chain is underproduced; usually asymptomatic, diagnosis is confirmed by increased HbA2 on electrophoresis
beta-thalassemia major - beta chain absent -> severe microcytic, hypo chromic anemia with target cells and increased anisopoikilocytosis requiring blood transfusion (secondary hemochromatosis)
crew cut on skull x-ray, chipmunk facies; extra medullary hematopoiesis -> hepatosplenomegaly
increased risk of parvoB19-induced aplastic crisis
increased HbF

50
Q

How do you treat secondary hemochromatosis d/t transfusions in beta-thalassemia major?

A

deferoxamine (iron chelator)

51
Q

What drugs cause increased oxidative stress in patient with G6PD deficiency?

A 
Spleen Purges Nasty Inclusions From Damaged Cells
S - sulfonamides
P - primaquine
N - nitrofurantoin
I - isoniazid
F - fava beans
D - dapsone
C -  chloroquine

DIT Cards (16 decks)

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