Failure of eculizumab to correct paroxysmal cold hemoglobinuria

June 29, 2017 | Autor: Stephen Opat | Categoria: Humans, Male, Monoclonal Antibodies, Middle Aged, Cyclophosphamide, Hemolysis
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Ann Hematol (2011) 90:989–990 DOI 10.1007/s00277-010-1123-x

LETTER TO THE EDITOR

Failure of eculizumab to correct paroxysmal cold hemoglobinuria Gareth Peter Gregory & Stephen Opat & Hang Quach & Jake Shortt & Huyen Tran

Received: 3 November 2010 / Accepted: 11 November 2010 / Published online: 26 November 2010 # Springer-Verlag 2010

Dear Editor, Paroxysmal cold hemoglobinuria (PCH) is a rare cause of intravascular hemolytic anemia in which an antibody against the P antigen develops and hemolysis ensues via a pathway involving thermally dependent complement binding. Previously an association of syphilis due to crossreactivity of antibody, PCH has since predominantly become a disease of childhood following varicella-zoster virus infection. The childhood form is typically acute, selflimiting, and steroid-responsive. Rare cases of PCH in adults have been described in association with chronic lymphocytic leukemia and lymphoma but never previously with myeloma [1]. We describe a case of steroid-refractory PCH in a patient with myeloma that was only partially corrected with eculizumab, a monoclonal antibody against C5 complement. A 51-year-old Nigerian man with Stage III ISS IgA kappa multiple myeloma in very good partial response following treatment with bortezomib, thalidomide, cyclophosphamide, and dexamethasone was admitted to our hospital with severe hemolytic anemia. At admission, G. P. Gregory (*) : S. Opat : H. Quach : J. Shortt : H. Tran Department of Clinical Hematology, Monash Medical Centre, Clayton, VIC, Australia e-mail: [email protected] S. Opat : H. Tran Australian Centre for Blood Diseases, Prahran, VIC, Australia

lactate dehydrogenase (LDH) was elevated at 543 U/L (100–200 U/L); bilirubin, 164 μmol/L (
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