Hematology 2 Flashcards

1
Q

Mutation in hereditary spherocytosis

A

spectrin, ankyrin, Band 3, protein 4.2

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2
Q

Band 3 mutation

A

hereditary spherocytosis

SE asian ovalocytosis

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3
Q

CBC abnormality in hereditary spherocytosis

A

increased MCHC

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4
Q

DDX of spherocytes

A

HS, immune mediated hemolysis

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5
Q

Mutation in hereditary elliptocytosis

A

Spectrin

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6
Q

Spectrin mutation

A

hereditary elliptocytosis

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7
Q

DDX if elliptocytes <25%

A

iron deficiency
megaloblastic anemia
MDS
Myelophthesis

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8
Q

Hereditary pyropoikilocytosis

A

variant of HE
hemolysis at 45C
transient in neonates

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9
Q

SE Asian ovalocytosis

A

Band 3 mutation

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10
Q

SE Asian ovalocytosis protects against

A

P. vivax

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11
Q

smear findings in Rh null phenotype

A

stomatocytes

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12
Q

Dz with thrombosis after splenectomy

A

hereditary stomatocytosis

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13
Q

name of precipitated Hgb seen in RBCs

A

Heinz bodies

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14
Q

Peripheral smear in G6PD def

A

Heinz bodies, poikilocytosis, spherocytes, bite/blister cells

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15
Q

Smear in pyruvate kinase def

A

ecchinocytes (burr cells) mostly after splenectomy

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16
Q

Autohemolysis test

A
Hereditary Spherocytosis (corrects with glucose)
Pyruvate kinase def (does not correct)
17
Q

Fluorescent spot test used for

A

pyruvate kinase def

18
Q

Congenital dyserythropoietic anemia II

A

HEMPA, + acidified serum test (heterologous only)

19
Q

Disorder with HAM + only with heterologous serum

A

Congenital Dyserythropoietic Anemia II (HEPAS)

20
Q

Disorder with HAM+ with both heterologous and autologous serum

A

Paroxysmal Nocturnal Hemolobinuria

21
Q

Increased density of i-antigen seen in

A

CDA II

Blackfan-Diamond

22
Q

Location of PIG-A gene

A

X-chromosome

23
Q

Decreased in PNH

A

CD55, CD59, AchE, CD16, CD48

24
Q

Sucrose hemolysis test + in

A

PNH

25
Q

LAP score in PNH

A

decreased

26
Q

Labs in sideroblastic anemia (haptoglobin, transferrin saturation, ferritin, Iron, LDH)

A

increased iron, transferrin saturation, ferritin, LDH

decreased haptoglobin

27
Q

Causes of acquired sideroblastic anemia

A

MDS, meds, radiation, EtOH, Pearson syndrome, Copper deficiency

28
Q

Pearson syndrome

A

sideroblastic anemia with pancreatic insuffiency

29
Q

congenital pure red cell aplasia

A

Blackfan-Diamond syndrome

30
Q

treatment of Blackfan-Diamond

A

corticosteroids

31
Q

Type of thymoma assd with red cell aplasia

A

spindle cell/meduallary/typeA