Heme/onc: random memorization

Question 1

Life-span of platelets?

Answer 1

8-10 days

Question 2

What fraction of platelet pool is stored in spleen?

Answer 2

1/3

Question 3

What do platelet dense granules contain?

Answer 3

ADP

Calcium

Question 4

What do platelet alpha granules contain?

Answer 4

vWF

Fibrinogen

Question 5

What’s the vWF receptor?

Answer 5

GpIb

Question 6

What’s the fibrinogen receptor?

Answer 6

GpIIb/IIIa

Question 7

What do pmn specific granules contain?

Answer 7

ALP
Collagenase
Lysozyme
Lactoferrin

Question 8

What do pmn azurophilic granules (lysosomes) contain?

Answer 8

Proteinases
Acid phosphatase
Myeloperoxidase
Beta-glucuronidase

Question 9

CD14 is a cell surface marker for what cell?

Answer 9

Macrophages

Question 10

What kind of nucleus do eosinophils have?

Answer 10

Bilobed

Question 11

Eosinophils make 2 major substances that help limit rxns after mast cell degranulation. What are they?

Answer 11

Histaminase

Arylsulfatase

Question 12

What do basophils’ granules contain?

Answer 12

Hepatin
Histamine
Leukotrienes

Question 13

Isolated basophilia can be a sign of what scary disease?

Answer 13

CML

Question 14

What 3 substances are released upon mast cell degranulation?

Answer 14

Histamine
Heparin
Eosinophilic chemotactic factor

Question 15

Eosin-5-maleimide binding test is used to screen for what hematologic disease?

Answer 15

Hereditary spherocytosis

Question 16

Why can patients with G6PD deficiency have increased bac/fungal infections?

Answer 16

Decreased glutathione interferes w/ normal fxns of MPO system, needed for clearance of bac/fungi.

Question 17

People with HbC defect have what very characteristic finding in their rbcs?

Answer 17

HbC crystals

Question 18

What is glutamic acid mutated to in HbC?

Answer 18

Lys

Question 19

Rx of PNH?

Answer 19

Eculizumab

Question 20

PNH has an increased risk of what kind of cancer?

Answer 20

Acute leukemias

Question 21

Sickle cell trait: where’s the only site of sickling (under normal conditions)?

Answer 21

Renal medulla

Question 22

MCC of death in sickle cell dz: adults?

Answer 22

Acute chest syndrome

Question 23

MCC of death in sickle cell dz: kids?

Answer 23

Infection w/ encapsulated organisms

Question 24

AutoAbs in ITP?

Answer 24

Anti-GpIIb/IIIa

Question 25

Prothrombin gene mutation causes a hypercoagulable state. Describe the actual mutation.

Answer 25

Mutation in 3' UTR--> incr production of prothrombin

Question 26

What inherited disease would cause a diminished increase in PTT after heparin admin?

Answer 26

Antithrombin 3 deficiency

Question 27

Which step of protoporphyrin synth requires B6? Name reactants, products, and the enz.

Answer 27

Gly + succinyl CoA--> delta-ALA. | Enz: Delta-ALA synthase

Question 28

What's the rate-limiting enz of protoporphyrin synth?

Answer 28

Delta-ALA synthase

Question 29

Deficiency of ALA-synthase causes what disease?

Answer 29

X-linked sideroblastic anemia

Question 30

Deficiency of porphobilinogen deaminase causes what disease?

Answer 30

Acute intermittent porphyria

Question 31

Deficiency of uroporphyrinogen deaminase causes what disease?

Answer 31

Porphyria cutanea tarda

Question 32

Why is DDAVP helpful in vWD?

Answer 32

Releases stored vWF from the endothelium

Question 33

What could cause acquired antithrombin deficiency?

Answer 33

Renal failure or nephrotic syndrome

Question 34

How does leukocyte ALP differ in CML and leukemoid rxn?

Answer 34

Increased in leukemoid rxn; decreased in CML

Question 35

1 unit of packed RBCs should increase HCT by how much?

Answer 35

3%

Question 36

1 unit of platelets should increase platelet count by how much?

Answer 36

5000 platelets/ mm^3

Question 37

How can transfusion affect K levels?

Answer 37

Can cause hyperkalemia due to lysis of RBCs in old blood

Question 38

What translocation is seen in mantle cell lymphoma? What gene does this activate?

Answer 38

t(11;14), cyclin D1

Question 39

The mixed cellularity type of Hodgkin's lymphoma can characteristically release a certain IL, which attracts a specific set of wbcs. Name the IL and wbc.

Answer 39

IL-5, eosinophils