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AOHE: manuscript AOHE-D-16-00564 paroxysmal nocturnal hemoglobinuria with autoimmune hemolytic anemia following eculizumab therapy—with large granular lymphocytic leukemia

2016; Springer Science+Business Media; Volume: 95; Issue: 10 Linguagem: Inglês

10.1007/s00277-016-2752-5

1432-0584

Nathan Visweshwar, Michael Jaglal, Cassie Booth, Patrick T. Griffin, Damian A. Laber,

Renal Diseases and Glomerulopathies

A 49-year-old Turkish male presented with intermittent passage of dark-colored urine since 2007.Flow cytometry demonstrated absence of CD55 and CD59 antigens, consistent with a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH).He initially responded to eculizumab, with improvement in anemia and resolution of hemoglobinuria.In 2009, the patient developed Coombs positive autoimmune hemolytic anemia (AIHA), which responded to steroids.Since he continued to require high doses of prednisone, splen e c t o m y w a s p e r f o r m e d i n N o v e m b e r 2 0 0 9 .Unfortunately, his AIHA persisted and he was treated with four weekly doses of rituximab 375 mg/m 2 in June 2010.At that time, fluorescently labeled aerolysin (FLAER) labeling on leukocytes failed to demonstrate