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01.06.2014 | original article | Ausgabe 11-12/2014

Wiener klinische Wochenschrift 11-12/2014

Cold antibody autoimmune hemolytic anemia and lymphoproliferative disorders: a retrospective study of 20 patients including clinical, hematological, and molecular findings

Zeitschrift:
Wiener klinische Wochenschrift > Ausgabe 11-12/2014
Autoren:
Cathrin Arthold, MD Cathrin Skrabs, MD Gerlinde Mitterbauer-Hohendanner, MD Renate Thalhammer, MD Ingrid Simonitsch-Klupp, MD Simon Panzer, Peter Valent, MD Klaus Lechner, MD Ulrich Jäger, MD Christian Sillaber

Summary

A total of 20 patients with cold antibody hemolytic anemia were evaluated in a retrospective study of them, 15 had a monoclonal gammopathy of unknown significance (MGUS): 14 with MGUS of immunoglobulin M (IgM) subtype and 1 with immunoglobulin G subtype. One patient had smoldering Waldenström’s macroglobulinemia, but four patients had no monoclonal protein and no evidence of lymphoma. However, in three of these patients, we were able to demonstrate a (mono-)clonal rearrangement of their immunoglobulin heavy and/or light chains. Of the 20 patients, 5 had IgHV34 nucleotide sequence indicating that the antibody was directed against the “I” antigen. Two patients exhibited a progressive increase of IgM over time, however without increasing hemolytic activity. Moreover, in two patients with long-term follow-up, we were able to correlate recurrent hemolytic activity with low environmental temperatures. Among four patients treated with rituximab, all four responded to treatment. However, treatment effect was only transient in all of them.

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