Presentation: AA or mediterranean man with severe anemia and jaundice suddenly.
Best initial: Heinz bodies and bite cells. G6PD is NORMAL initially.
Most accurate: G6PD level 1-2 months after resolution.
HUS and TTP are similar in what ways? What are assoc with each one? Treatment?
ADAMTS13 def, intravascular hemolysis with shistocytes, thrombocytopenia, renal insufficiency.
HUS assoc with E. coli.
TTP assoc with ticlopidine, clopidogrel, cyclosporine, AIDS, and SLE
Treatment: severe- plasmapheresis or plasma exchange. NOT related to drugs/diarrhea- steroids. DO NOT TRANSFUSE platelets.
Myelofibrosis: presents in what age with what description? Smear shows? Treatment?
older people, pancytopenia, BM with fibrosis so blood production switches to the liver and spleen--> hepatosplenomegaly. Smear shows tear drop and nucleated RBCs. Tx with TNF inhibitors, thalidomide, to increase BM production.
Paroxysmal Nocturnal Hemoglobinuria: deficiency and mechanism of disease.
Clonal stem cell defect where RBCs are sensitive to complement in acidosis. Def in CD 55 and 59, AKA decay accelerating factor. Sleep causes hypoventilation, which induces acidosis and leads to hemolysis and thrombosis.
**DARK URINE with the FIRST urination of the day
*Pancytopenia and iron def anemia
PNH most accurate test, treatment, and most common cause of death
CD55 and CD59 flow cytometry.
Steroids, BM transplant, Eculizumab inactivates C5, and give folic acid.
- Best initial tx: DDAVP, releases subendothelial stores of vWF. No response, give factor VIII or vWF concentrate.
Hemophilia: presentation, lab studies, most accurate, treatment
delayed joint or muscle bleeding, male (XLR), aPTT prolonged, mixing studies will correct to normal because its just due to a deficiency of VIII or IX. Most accurate: assay for VIII or IX. Treatment: Mild- DDAVP, Severe- Factor replacement
Factor XII def
elevated aPTT, but NO bleeding. NO treatment needed
DIC occurs in what conditions? Lab results? Treatment