Heme- Anemia, Coag, Malignancy

Question 1

X-linked recessive heme disorders

Answer 1

Hemophilias

G6PDD

Question 2

Thalassemia is a(n) ______ inherited disorder

Answer 2

autosomal recessive

Question 3

Sickle Cell Anemia is a(n) ______ inherited disorder

Answer 3

autosomal recessive

Question 4

Hereditary Spherocytosis is a(n) ______ inherited disorder

Answer 4

autosomal dominant

Question 5

Von Willebrand Disease is a(n) ______ inherited disorder

Answer 5

autosomal dominant

Question 6

Hemophilia A is a(n) ______ inherited disorder

Answer 6

x-linked recessive

Question 7

MCC of Anemia worldwide

Answer 7

Iron Deficiency Anemia

Question 8

MCC of iron defic anemia

Answer 8

Blood loss

-GI, menstrual, blood donation

Question 9

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

Answer 9

Iron Deficiency Anemia

Question 10

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

Answer 10

Vit B6 defic anemia

dx via plasma pyridoxal phosphate

Question 11

plasma pyridoxal phosphate used to dx what defic?

Answer 11

Vit B6

Question 12

how to dx folate def anemia

Answer 12

RBC folate level

Question 13

patho of aplastic anemia

Answer 13

bone marrow failure from suppression/injury to hematopoietic stem cells

Question 14

pancytopenia and bone marrow bx that demonstrates hypocellularism

Answer 14

aplastic anemia

Question 15

micryocytic hypochromic anemia

micryocytosis that is out of proportion to the anemia

Answer 15

Thalassemia

Question 16

Alpha thalassemia is d/t gene ______

Answer 16

deletions

more alpha globin chains not transcribed means worse sxs

Question 17

Heinz bodies

Answer 17

G6PDD

Question 18

fava beans, antimalarials, sulfa, ASA

Answer 18

G6PDD

Question 19

hyperchromic microcytosis with spheroctyes and increased retic on peripheral smear
splenomegaly

Answer 19

heredity spherocytoss

Question 20

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

Answer 20

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

Question 21

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

Answer 21

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

Question 22

Christmas disease

Answer 22

hemophilia B, factor 9 def

Question 23

“Hemophlia C”

Answer 23

factor XI def

Question 24

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

Answer 24

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

Question 25

MC inherited bleeding disorder

Answer 25

von Willebrand Dis

Question 26

von Willebrand had what kind of bleeding?

Answer 26

platelet type: | petechial, gingival bleeding, epistaxis bruising

Question 27

tx vWF dis

Answer 27

DDAVP | ~vwf Concentrate

Question 28

TMA pentad

Answer 28

``` microagniopathic hemolytic anemia thrombocytopenia fever renal insuff neruo abnl ```

Question 29

adamst13

Answer 29

TTP

Question 30

tx TTP

Answer 30

plasma exchange immed | NO platelets

Question 31

monitor warfarin with

Answer 31

PT/INR

Question 32

reversal of heparin

Answer 32

protamine sulfate

Question 33

monitor heparin with

Answer 33

aPTT

Question 34

MC inheritable cause of hypercoag

Answer 34

Factor V Leiden

Question 35

how to dx sickle cell

Answer 35

electrophroesis

Question 36

tx PV

Answer 36

phlebotomy hydroxyurea ASA

Question 37

JAK2 mutation

Answer 37

PV

Question 38

pruritus following warm shower

Answer 38

PV

Question 39

Philadelphia chromosome

Answer 39

CML

Question 40

isolated lymphocytosis | smudge cells

Answer 40

CLL

Question 41

auer rods

Answer 41

AML

Question 42

bone marrow hypercellular with blass | (+) sudan black esterase strain

Answer 42

AML

Question 43

terminal deoxynuceltidyl transferase

Answer 43

ALL

Question 44

MC leukemia of children

Answer 44

ALL

Question 45

Reed Sternberg cells

Answer 45

Hodgkin lymphoma

Question 46

bence jones proteins in urine

Answer 46

MM

Question 47

xray with punched out lytic lesions

Answer 47

MM