Hematology

Question 1

megaloblastic anemia and neurologic deficits

Answer 1

Vitamin B12 deficiency

can be caused by a strict vegan diet

megaloblastic anemia without neurological signs can be a sign of a B6 deficiency

Question 2

High ferritin, low iron, low TIBC with anemia

Answer 2

Anemia of Chronic Disease

associated with conditions such as RA, SLE, neoplastic disorders and kidney disease.

The inflammation leads to increased hepcidin (released by liver). Eventually, iron is not released from the macrophages and there is a decrease in iron absorption from the gut

Question 3

What drugs cause anemia in patients with G6PD deficiencies?

Answer 3

Sulfa

ASA

NSAIDS

Nitrofurnatoin

Quinidine

Quinine

pts with a G6PD deficiency can not maintain glutathione in its reduced state. They are more susceptible to damage by free radicals because they can not make NADPH

Question 4

What enzyme metabolized 6-mercaptopurine?

Answer 4

Xanthine Oxidase

use with caution in combination with Allopurinol

Question 5

Inherited

abnormal blood vessel formation in the skin and mucus membranes

Answer 5

Osler-Weber-Rendau Syndrome

Inherited abnormalities in blood vessel formation in the skin and mucus membranes.

Can present as: chronic nose bleeds, GI bleeding

Autosomal dominant

Question 6

Puritis

Hemoglobin >17 in men or >15 in women

Answer 6

Polycythemia vera

Question 7

Platelet Adhesion

Answer 7

Platelets binding with vWF and sticking to the endothelium

vWF binds to exposed collagen (after endothelial injury) .

Gp1b (found on platelets) binds to the vWF which attaches the platelets to the wall of the endothelium.

Question 8

Bernard-Soulier syndrome

Answer 8

deficiency in Gp1b

deficiency in adhesion of platelets to vWF

longer bleeding time

enlarged platelets

Question 9

GpIIb/IIIa

Answer 9

factors required for platelet aggregation

deficiency: Glanzmann thrombasthenia

can present as abnormal mucosal bleeding (recurrent epistaxis)

Question 10

P2Y₁₂ inhibitors

Answer 10

“-grel”

clopridigrel, prasugrel

ticagrelor (reversible)

ticlopidine

Question 11

HIT

Answer 11

Heparin induced Thrombocytopenia

Antibodies to the complex of platelet factor 4 and heparin leads to platelet activation and consumption

Question 12

warfarin skin necrosis

Answer 12

deficiency in protein C

Question 13

Ab responsible for (+) VRDL

Answer 13

anti-cardiolipin Ab

Question 14

Most common cause of inherited hypercoagulability in caucasians

Answer 14

Factor V Leiden mutations

point mutations resulting in a mutant factor V result in the factor being resistant to degredation by activated protein C.

This means that factor V has a longer half life resulting in the overactivity of the clotting cascade

Question 15

Heparin resistance

Answer 15

anti-thrombin III deficiency

UFH activates antithrombin III- if you administer heparin and have no change in the PT, it means that you have a deficiency of antithrombin III

Question 16

marfanoid habitus

increased risk of stroke

Answer 16

elevated homocystine dt a deficiency in cystathionine beta synthase

elevated homocystine levels predispose patients to thrombosis and atheroma

Question 17

Thrombotic Thrombocytopenic Purpura

Answer 17

inhibition or deficiency of AADAMTS13 resulting in a decrease in degredation of vWF multimers = increased clotting

S/S: fever, neurologic deficits, renal failure, petichae

schistocytes on peripheral smear

normal PT/PTT time

Question 18

Increased Bleeding time

Normal PT

normal or increased PTT

Answer 18

von Willebrand Disease

autosomal dominant

no platelet aggregation with ristocetin cofactor asasy

commonly presents with menorrhagia as first sign

tx: desmopressin or aminocaproic acid

Question 19

Howell-Jolly Bodies

Answer 19

Howell-Jolly bodies are nuclear reminants that are normally removed by the spleen. In patients with absent or decreased splelenic function, the Howell-Jolly bodies are not removed

Patients with sickle cell disease are at increased risk for development of autosplenectomy