Cold antibody autoimmune hemolytic anemia and lymphoproliferative disorders: a retrospective study of 20 patients including clinical, hematological, and molecular findings.
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.
Division of Hematology and Hemostaseology, Depatment of Internal Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, 1097, Vienna, Austria.
Cathrin
C
Arthold
Cathrin
C
Skrabs
Gerlinde
G
Mitterbauer-Hohendanner
Renate
R
Thalhammer
Ingrid
I
Simonitsch-Klupp
Simon
S
Panzer
Peter
P
Valent
Klaus
K
Lechner
Ulrich
U
Jäger
Christian
C
Sillaber
D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001323:Autoantibodies / Q000276:immunology*; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology*; D008297:Male; D012189:Retrospective Studies; D000069283:Rituximab; D016896:Treatment Outcome
D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007155:Immunologic Factors; D000069283:Rituximab