Heme- Anemia, Coag, Malignancy Flashcards

1
Q

X-linked recessive heme disorders

A

Hemophilias

G6PDD

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2
Q

Thalassemia is a(n) ______ inherited disorder

A

autosomal recessive

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3
Q

Sickle Cell Anemia is a(n) ______ inherited disorder

A

autosomal recessive

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4
Q

Hereditary Spherocytosis is a(n) ______ inherited disorder

A

autosomal dominant

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5
Q

Von Willebrand Disease is a(n) ______ inherited disorder

A

autosomal dominant

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6
Q

Hemophilia A is a(n) ______ inherited disorder

A

x-linked recessive

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7
Q

MCC of Anemia worldwide

A

Iron Deficiency Anemia

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8
Q

MCC of iron defic anemia

A

Blood loss

-GI, menstrual, blood donation

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9
Q

hypochromic microcytic on smear
decreased serum Fe, serum ferritin
increased TIBC

A

Iron Deficiency Anemia

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10
Q
c/o weakness, irritability, decreased concentration
microcytic hypochromic anemia
exam with glossitis
w/o
dx
A

Vit B6 defic anemia

dx via plasma pyridoxal phosphate

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11
Q

plasma pyridoxal phosphate used to dx what defic?

A

Vit B6

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12
Q

how to dx folate def anemia

A

RBC folate level

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13
Q

patho of aplastic anemia

A

bone marrow failure from suppression/injury to hematopoietic stem cells

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14
Q

pancytopenia and bone marrow bx that demonstrates hypocellularism

A

aplastic anemia

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15
Q

micryocytic hypochromic anemia

micryocytosis that is out of proportion to the anemia

A

Thalassemia

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16
Q

Alpha thalassemia is d/t gene ______

A

deletions

more alpha globin chains not transcribed means worse sxs

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17
Q

Heinz bodies

A

G6PDD

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18
Q

fava beans, antimalarials, sulfa, ASA

A

G6PDD

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19
Q

hyperchromic microcytosis with spheroctyes and increased retic on peripheral smear
splenomegaly

A

heredity spherocytoss

20
Q

hemophilia A is deficiency of factor ______
inherited ______
prolonged_____
tx with ______

A
A=8
xlinked recessive
prolonged PTT
tx with factor VIII, DDAVP for minor bleeds
cox2 (Celebrex) for joint pain
21
Q

hemophilia B is deficiency of factor ______
inherited ______
prolonged_____
tx with ______

A

B=9
inherited linked recessive
prolonged PTT
tx with factor IX, DDAVP for minor bleeds

22
Q

Christmas disease

A

hemophilia B, factor 9 def

23
Q

“Hemophlia C”

A

factor XI def

24
Q

hemophilia “C” is deficiency of factor ______
inherited ______
prolonged_____
tx with ______

A
C= 11
AUTOSOMAL recessive, Ashkenazi Jews
prolonged PTT
tx with FFP
  aminocaproic acid for procedures
25
Q

MC inherited bleeding disorder

A

von Willebrand Dis

26
Q

von Willebrand had what kind of bleeding?

A

platelet type:

petechial, gingival bleeding, epistaxis bruising

27
Q

tx vWF dis

A

DDAVP

~vwf Concentrate

28
Q

TMA pentad

A
microagniopathic hemolytic anemia
thrombocytopenia
fever
renal insuff
neruo abnl
29
Q

adamst13

A

TTP

30
Q

tx TTP

A

plasma exchange immed

NO platelets

31
Q

monitor warfarin with

A

PT/INR

32
Q

reversal of heparin

A

protamine sulfate

33
Q

monitor heparin with

A

aPTT

34
Q

MC inheritable cause of hypercoag

A

Factor V Leiden

35
Q

how to dx sickle cell

A

electrophroesis

36
Q

tx PV

A

phlebotomy
hydroxyurea
ASA

37
Q

JAK2 mutation

A

PV

38
Q

pruritus following warm shower

A

PV

39
Q

Philadelphia chromosome

A

CML

40
Q

isolated lymphocytosis

smudge cells

A

CLL

41
Q

auer rods

A

AML

42
Q

bone marrow hypercellular with blass

(+) sudan black esterase strain

A

AML

43
Q

terminal deoxynuceltidyl transferase

A

ALL

44
Q

MC leukemia of children

A

ALL

45
Q

Reed Sternberg cells

A

Hodgkin lymphoma

46
Q

bence jones proteins in urine

A

MM

47
Q

xray with punched out lytic lesions

A

MM