Hematology

Question 1

ITP

Answer 1

Chronic in adults
Self limited in kids (post infection or immunization)

Production and destruction problem

Steroids, IVIG, platelets (critical only, will be destroyed), splenectomy

Question 2

TTP

Answer 2

Fever, Anemia, Thrombocytopenia, Renal Failure, Neuro

***Do not give platelets!

Increased unconjugated bili, increased LDH, normal fibrin/fibrinogen

Platelet only clots

Send ADAMTS-13 levels (low), vWF gel electrophoresis confirms

Tx = FFP, plasma exchange, steroids, splenectomy

Question 3

HUS

Answer 3

Childhood disease

MAHA, Thrombocytopenia, ARF

Post E.Coli 0157:H7 (shiga-like toxin)

Platelet-fibrin clots

Dx = stool/urine for shiga toxin
Increased UC bili and LDH, normal fibrin/fibrinogen

Tx = supportive
Abx makes it worse
Don’t give platelets!

Question 4

Hemophilia

Answer 4

Factor 8 - Type A
Factor 9 - Type B
Intrinsic Pathway (PTT elevated)

X linked recessive

For factor, assume pt’s function is 0.
8 = 1 unit replaces 2%.
9 = 1 unit replaces 1%.

FFP when 8 not available, Cryo has a little more, can use DDVAP which works on vWF (carries factor 8)

Question 5

Von Willebrand’s

Answer 5

vWF attached to factor 8

Quantitative or qualitative defect

**If complete lack of vWF, DDAVP won’t work

Prolonged bleeding time

Tx = DDAVP, cryo, non-recombinant (not synthetic) factor 8