PNH Clinical Case Review

Ilene C. Wetiz, MD, professor of clinical medicine at the Keck School of Medicine at the University of Southern California, discusses the baseline characteristics of the case:

So, case three is [paroxysmal nocturnal hemoglobinuria (PNH)] with aplastic anemia — a 54 year old woman who presented in 2007 with pancytopenia. Her bone marrow was consistent with aplastic anemia. She was treated with immunosuppressive therapy and achieved a complete remission. And as frequently happens at our county hospital, she was lost to follow-up.

And then in April of 2012, she complained of increasing fatigue that had been going on for 6 months. Her hemoglobin at the time she presented was 2.9 [mg/dL]. Her MCV was 107 [fl]. Her white count was 1.9 [x 10⁹/L] with an [absolute neutrophil count (ANC)] of 900, and her platelet count was 107,000[/µL]. Her reticulocyte count was actually on the low side — 26,000[/µL] — and her LDH was 1,500 [IU/L]. Bilirubin was 1.8 [mg/dL] total, and the direct was 0.3 [mg/dL]. A PNH flow was sent. They did not do a bone marrow because they were worried that if she had any bleeding, she didn't have any room to have a bleed with a hemoglobin that low. Her granulocytes on the PNH flow were 85%, were GPI deficient, and the red cells — 78% were type III cells, consistent with PNH.