Thursday, July 14, 2016

Paroxysmal Nocturnal Hemoglobinuria

Clinical Manifestations:

Hemolysis --> fatigue
Cytopenias
Venous thrombosis --> acute abdominal pain, esp. portal vein thrombosis

Workup:

CBC (find hypoplastic/aplastic anemia, thrombocytopenia, leukopenia)
Lactate dehydrogenase: elevated
Haptoglobin: Low (due to hemolysis)
Indirect bilirubin: elevated
UA: hemoglobinuria
Flow cytometry: absent CD55 & CD 59 (allow autoimmune MAC complexes)

Treatment: Iron, folate, Eculizumab (inhibs complement activation)


No comments:

Post a Comment