Disorders of hemostasis

Question 1

Immune thrombocytopenic purpura- Eti

Answer 1

Idiopathic. Immune mediated destruction of platelets. can be associated with autoimmune disorders- SLE, HIV, Hep C.

Question 2

Immune thrombocytopenic purpura- Sx

Answer 2

Very low platelet count with normal blood cells & smear. Mucocutaneous bleeding- eccymosis, petechiae

Question 3

Immune thrombocytopenic purpura- Dx

Answer 3

Isolated thrombocytopenia- platelet <100,00. Exclude HIV, Hep B &C

Question 4

Immune thrombocytopenic purpura- Tx

Answer 4

Corticosteroids. Platelet transfusion if active bleeding present

Question 5

Thrombotic thrombocytopenic purpura- Eti

Answer 5

Deficiency or antibodies to protease that cleaves VWF- causes platelet adhesion and aggregation. Women, HIV & pregnancy

Question 6

Thrombotic thrombocytopenic purpura- Sx

Answer 6

Thrombocytopenia due to platelets into thrombi in microvasculature, -Microcangiopathic hemolytic anemia
hemolysis, microvascular thrombosis. Pentad: + fever, renal failure & neurologic abn (HA, coma, seizure, delerium).

Question 7

Thrombotic thrombocytopenic purpura- Dx

Answer 7

Anemia, reticulocytosis, normal coagulation studies, elevated creatanine

Question 8

Thrombotic thrombocytopenic purpura- Tx

Answer 8

Plasma exchange to restore platelet count.

Question 9

Hemolytic-uremic syndrome- Eti

Answer 9

Following diarrheal illness. Common in carcinoma- breast, gastric, lung- onset 4-8 weeks after chemo.

Question 10

Hemolytic-uremic syndrome- Sx

Answer 10

Microangiopathic hemolytic anemia, thrombocytopenia plus oliguria, proteinuria and uremia. May be accompanied by renal failure, hypertension & pulmonary edema.

Question 11

Hemolytic-uremic syndrome- Dx

Answer 11

Anemia, elevated serum creatanine, hematuria, proteinuria

Question 12

Hemolytic-uremic syndrome- Tx

Answer 12

Urinary sx- fluid & lytes
Renal involvement- dialysis
Plasma exchange

Question 13

Henoch-Schonlein purpura- Eti

Answer 13

Most common systemic vasculitis in children. IgA mediated disorder that causes bleeding in small vessels.

Question 14

Henoch-Schonlein purpura- Sx

Answer 14

Purpuric skin lesion on lower extremities, arms, trunk & butt,
joint symptoms- ankles & knees
Abd pain

Question 15

Henoch-Schonlein purpura- Dx

Answer 15

Skin biopsy- leukocytoclastic vasculitis with IgA

Kidney biopsy- segemental glomeulonephritis

Question 16

Henoch-Schonlein purpura- Tx

Answer 16

Prednisone- often resolves without treatment

Question 17

Heparin-induced thrombocytopenia- Eti

Answer 17

Thrombocytopenia within 5-10 days of heparin exposure. Decrease in platelet >50%. Approx 3% heparin pts

Question 18

Heparin-induced thrombocytopenia- Sx

Answer 18

Thrombosis, throbocytopenia and prothrombic state. often asymptomatic, bleeding rare.

Question 19

Heparin-induced thrombocytopenia- Dx

Answer 19

> 50% decrease in platelets, new onset of throbocytopenia following heparin exposure. ELISA

Question 20

Heparin-induced thrombocytopenia- Tx

Answer 20

Discontinue heparin, RO DVT, platelet inhibitor-Argatroban