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

25
LAP score in PNH
decreased
26
Labs in sideroblastic anemia (haptoglobin, transferrin saturation, ferritin, Iron, LDH)
increased iron, transferrin saturation, ferritin, LDH | decreased haptoglobin
27
Causes of acquired sideroblastic anemia
MDS, meds, radiation, EtOH, Pearson syndrome, Copper deficiency
28
Pearson syndrome
sideroblastic anemia with pancreatic insuffiency
29
congenital pure red cell aplasia
Blackfan-Diamond syndrome
30
treatment of Blackfan-Diamond
corticosteroids
31
Type of thymoma assd with red cell aplasia
spindle cell/meduallary/typeA