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Board Review - C - Heme/Oncology > Heme/Onco II > Flashcards

Heme/Onco II Flashcards

1
Q

thalassemia

A

microcytic anemia

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2
Q

a-thalassemia

A

defect a-globin gene defect

cis deletion - asian
trans deletion - AA

severity - based on number of deletions

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3
Q

4 allele deletion a-thalassemia

A

see Hb barts - gamma 4

hydrops fetalis

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4
Q

3 allele deletion a-thalassemia

A

HbH disease

little a-globin

excess B-globin - HbH

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5
Q

B-thalassemia

A

point mutation splice site - promoter sequences
-decreased B-globin synthesis

mediterranean populations

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6
Q

B-thal minor

A

heterozygote

low B-chain

increased HbA2

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7
Q

B-thal major

A

homoygote

B-chain absent

severe anemia - need transfusions

risk of parvo B19 infection

increased HbF - a2 g2 - protective in infant - symptomatic at 6 months

marrow expansion - crew cut - like with sickle cell

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8
Q

lead poisoning

A

lead inhibit ferrochelatase and ALA dehydratase

decrease heme synthesis
increased RBC protoporphyrin

see basiphilic stippling

buron lines - gingiva

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9
Q

tx of lead poison

A

chelation
dimercaprol and EDTA

succimer in kids

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10
Q

sideroblastic anemia

A

defective heme synthesis
X-linked
defect d-ALA synthase

get ringed sideroblasts with iron mito

tx - pyridoxine

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11
Q

pyridoxine

A

vit B6

cofactor for d-ALA synthase

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12
Q

B12 vs. folate deficiency

A

B12 - brain - neuro sx

folate - no neuro sx

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13
Q

diphyllobothrium latum

A

fish tapeworm

lead to B12 deficient

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14
Q

orotic aciduria

A

cannot convert orotic acid to UMP
-de novo pyrimidine synthesis

defect UMP synthase

auto rec

tx - uridine monophosphate - bypass mutated euzyme

no hyperammoniemia (vs. OTC deficiency)

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15
Q

normocytic anemias

A

hemolysis - intra and extravascular

anemia of chronic disease

aplastic anemia

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16
Q

LDH

A

marker for hemolysis - elevated

17
Q

anemia of chronic disease

A

inflammation - increased hepcidin - inhibit iron transport

decreased iron
decreased TIBC
increased ferritin

tx - EPO - if chronic kidney disease

18
Q

aplastic anemia

A

failure of myeloid stemo cells

  • radiation and drugs
  • viral
  • fanconi - DNA repair defect

get pancytopenia
-dry bone marrow tap

tx - RBC/platelet transfusion or GM-CSF

19
Q

aplastic anemia drugs

A

benzene
chloramphenicol
alkylating agent
antimetabolite

20
Q

hereditary spherocytosis

A

defect ankyrin, band 3, protein 4.2, spectrin

small round RBC

elevated MCHC and RDW

tx - splenectomy

21
Q

osmotic fragility test

A

diagnosis heredtiary spherocytosis

22
Q

G6PD deficiency

A

X-linked recessive

heinz body and bite cells

23
Q

pyruvate kinase deficiency

A

auto rec
decreased ATP - rigid RBC

hemolytic anemia in newborn

24
Q

HbC defect

A

glutamic acid to lysine mutation - B-globin

25
Q

paroxysmal nocturnal hemoglobinuria

A

complement mediated RBC lysis

impaired GPI anchor that protect from complement

CD55/59 negative

26
Q

eculizumab

A

terminal complement inhibitor

tx for paroxysmal nocturnal hemoglobinuria

27
Q

sickle cell anemia

A

point mutation
-glutamic acid to valine - B-chain

deox - HbS sickles

newborn - asymptomatic - bc high HbF

crew cut on X-ray - marrow expansion

28
Q

heterozygote for HbS

A

resistant to malaria

29
Q

autosplenectomy

A

with sickle cell disease

infection with encapsulated organisms

30
Q

osteomyelitis in sickle cells

A

salmonella

31
Q

tx sickle cells

A

hydroxyurea - increases HbF

and hydration

32
Q

warm agglutinin

A

IgG

chronic anemia with SLE and CLL

33
Q

cold aggultinin

A

IgM
-anemia with cold

CLL, mycoplasma pneumona, nononucleosis

34
Q

coombs test

A

determine autoimmune hemolytic anemia

determine anti-Ig Abs on RBCs

direct and indirect

35
Q

microangiopathic anemia

A

RBC damaged passing narrow lumen

DIC, TTP/HUS, SLE, malignant HTN

see schistocytes

also with prosthetic heart valve

36
Q

destruction of RBC infections

A

malaria and babesia

37
Q

anemia lab values

A

TIBC - indirect measure of transferrin
transferrin - transport
ferritin - iron storage protein

38
Q

circulating iron

A

needed by pathogens to survive

-so body developed way to store iron

39
Q

anemia of chronic disease

A

low serum iron
low transferrin
high ferritin

Board Review - C - Heme/Oncology (4 decks)

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