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Flashcards in Hematology Deck (97)
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31

Buzzwords for lead poisoning

BASOPHILIC STIPPLING
Led lines on gingivae (Burton lines)
Sideroblastic anemia
Wrist/foot drop
Increased protoporphyrin

32

Treatment of lead poisoning

Dimercaprol and EDTA are 1st line
Succimer in kids

33

Sideroblastic Anemia Mutation
- Microcytic anemia

X-linked defect in S-ALA synthase gene (rate-limiting step in protoporphyrin synthesis)
- Requires B6 as cofactor

34

Causes of sideroblastic anemia?

- Genetic
- Acquired (myelodysplastic syndrome)
- Reversible (alcohol, lead, B6 deficiency, copper deficiency, Isoniazid)

35

Sideroblastic anemia labs?

- Increased iron
- Normal/decreased TIBC
- Increased ferritin
- RINGED SIDEROBLAST (iron-laden Prussian blue-staned mt)
- basophilic stippling
- Decreased protoporphyrin

36

Treatment of sideroblastic anemia?

B6 (pyridoxine)

37

Which tapeworm can cause B12 deiciency?

Diphyllobothrium latum

38

Which spinal pathways are involved in subacute combined degeneration (B12 deficiency)?

Spinocerebellar (ataxia)
Lateral corticospinal tract (voluntary movement)
Dorsal column (P, vibration, fine touch, proprioception)

39

Orotic Aciduria Defect
- Macrocytic anemia

UMP Synthase defect --> can't convert orotic acid to UMP (de novo pyrimidine synthesis pathway)
- AR

40

Treatment of orotic aciduria?

Uridine monophosphate

41

Presentation of orotic aciduria?

- Kids
- FTT, developmental delay, megaloblastic anemia refractory to folate or B12
- NO hyperammonemia

42

Diamond-Blackfan Anemia

Rapid-onset anemia within 1st year of life due to intrinsic defect in erythroid progenitor cells

43

Intravascular Hemolysis Findings
- Mechanical hemolysis
- Paroxysmal nocturnal hemoglobinuria
- Microangiopathic hemolytic anemias

- Decreased haptoglobin (binds Hb, Hb is released into circulation)
- Increased LDH
- Schistocytes and reticulocyts on smear

44

Extravascular Hemolysis Findings

- Macs in spleen clear RBCs
- Spherocytes
- Increased LDH
- Increased unconjugated bilirubin --> jaundice

45

Anemia of Chronic Disease MOA

- Increased hepcidin (IL-6)
- Decrease release of iron from Macs, decreased iron absorption from gut

46

Anemia of Chronic DZ Findings

- Decreased iron
- Decreased TIBC
- Increased ferritin

47

Aplastic Anemia Findings

- Decreased reticulocyte count
- Increased EPO
- Pancytopenia

48

What does bone marrow look like in aplastic anemia?

- Hypocellular bone marrow with fatty infiltration

49

What is Fanconi anemia?

- DNA repair defect --> bone marrow failure
- Short stature, increased incidence of tumors/leukemia, cafe-au-lait spots, thumb/radial defects

50

What cells does parvovirus B19 infect?

Progenitor RBCs --> temporarily halts erythropoiesis
- Significant anemia in setting of preexisting marrow stress

51

Hereditary spherocytosis defect?

- RBC membrane skeleton and plasma membrane defect (ankyrin, spectrin)

52

Hereditary spherocytosis findings?

- Spherocytes
- Increased MCHC
- Splenomegaly
- Aplastic crisis (parvovirus B19)

53

What is the test for hereditary spherocytosis?

Osmotic fragility test + (glycerol)

54

G6PD deficiency defect?

- X-linked recessive defect in G6PD --> decreased NADPH --> decreased glutathione --> increased RBC susceptibility to oxidant stress (sulfa drugs, antimalrials, infections, fava beans)

55

G6PD Deficiency Findings?

- Back pain, hemoglobinuria
- Heinz bodies, bite cells

56

Pyruvate Kinase deficiency defect?

AR
Defect in pyruvate kinase --> decreased ATP --> rigid RBCs --> extravascular hemolysis

57

HbC Disease mutation

- Glutamic acid --> lysine in Beta-globulin
- Extravascular hemolysis

58

HbC blood smear?

Hemoglobin crystals inside RBCs
Target cells

59

Paroxysmal nocturnal hemoglobinuria defect?

Impaired synthesis of GPI anchor for decay-accelerating-factor that protects RBC membrane from complement --> increased complement-mediated intravascular RBC lysis
- Acquired in hematopoietic stem cell

60

PNHb triad?

- Coombs negative hemolytic anemia
- Pancytopenia
- Venous thrombosis