Heme - ITP & TTP/HUS

Question 0

Causes of impaired platelet production?

Concomitant finding?

Answer 0

1. Myelofibrosis
2. Bone marrow suppression due to drugs, radiation
3. Malignancy

Also will see a decrease in RBCs and WBCs

Question 1

Physical findings suggestive of thrombocytopenia/abnormal platelet function versus coagulation abnormality?

Answer 1

Mucosal bleeding, epistaxis/gums, petechia, purpura

versus

hemarthrosis, hematomas, Retroperitoneal bleeding

Question 2

ITP affects what age groups? Difference in course of disease?

Answer 2

Most common children following up URI. Results spontaneously within 3 to 6 months.

Also affects women ages 20 to 40. Persist for months to years with uncommon spontaneous remission

Question 3

Test for ITP?

Answer 3

Direct Coombs test

Question 4

Treatment of ITP and adults?

Answer 4

1 to 2 mg/kilograms of prednisone

Add IVIg if platelet counts are <10,000

If patients do not respond to steroids, can perform splenectomy

Question 5

Causes of drug induced thrombocytopenia?

Answer 5

H2-blockers, quinine, sulfonamides

Question 6

Types of heparin-induced thrombocytopenia?

Answer 6

HIT-1: Not immune mediated, onset <48 hours, caused Platelet clumping

HIT-2: caused by platelet activating antibodies, occurs after three days (Unless patient has been sensitized prior to this)

Question 7

Consequence of HIT? Diagnosed by? Treatment? No not treat with?

Answer 7

Thrombosis (not bleeding)

ELISA for PF4 OR 5HT release assay

Direct thrombin inhibitor – argatroban, lepirudin THEN warfarin

Platelet transfusion

Question 8

PT/PTT and Coombs’ test results in HUS versus TTP?

Answer 8

All normal

Question 9

Treatment for severe HUS/TTP? If delay in getting patient to plasmapheresis, use?

Do not give these patients?

Answer 9

Plasmapheresis

Infuse FFP

Platelets

Question 10

Patient presents with isolated thrombocytopenia and normal sized spleen – most likely diagnosis?

Answer 10

ITP

Question 11

Patient presents with episodes of bleeding and a platelet count of 17,000. PE normal - treatment??

Answer 11

ITP

Prednisone

Question 12

Treatment protocol for ITP?

Answer 12

#No bleeding and platelets over 30,000 – no treatment
#Mild bleeding platelets under 30,000 – glucocorticoids
#Severe bleeding platelets under 10,000 – IV IG, anti-Rho
#Recurrent bleeding episodes, and steroid dependence – splenectomy
#Splenectomy not effective – Romiplostim (synthetic thrombopoetin) or steroid alternates

Question 13

Before splenectomy, give all patients?

Answer 13

Meningococcus, pneumococcus, H. flu

Question 14

Patient presents with platelet bleeding (epistaxes, gingival, gums) with a normal platelet count – suspected diagnosis? Will likely worsen if patient takes? This lab may be elevated?

Answer 14

Von Willebrand disease

Aspirin

PTT

Question 15

Patient with suspected von Willebrand disease – best initial therapy? If no response?

Answer 15

DDAVP (Releases said endothelial stores von Willebrand factor)

If no response, use factor VIII replacement

Question 16

Diagnostic tests for Von Willebrand disease?

Answer 16

#Ristocetin cofactor assay/von Willebrand factor activity test
#Von Willebrand factor level
#Factor 8 activity

Question 17

Coagulation studies in hemophilia? Most accurate test? Treatment?

Answer 17

Elevated PTT, corrects with mixing study

Specific assay for factor 8 or 9

#Mild cases – DDAVP
#Severe bleeding – factor replacement

Question 18

factor 11 deficiency - labs? Presentation? Treatment?

Answer 18

Elevated PTT that is normalized after mixing study

Increased bleeding after trauma/surgery

Fresh frozen plasma

Question 19

Patient with elevated PTT but no increased risk of bleeding?

Answer 19

Factor 12 deficiency

Question 20

Lab findings in DIC?

Answer 20

#Elevation in PT and PTT
#Low platelet count
#Elevated D dimer and fibrin split products
#Decreased fibrinogen

Replace platelets and clotting factors (FFP)

Question 21

Best initial test for APLS?

Answer 21

Mixing study – PTT will remain elevated