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Remission of severe cold agglutinin disease Rituximab therapy

Leukemia volume 15, pages 187–188 (2001)Cite this article

Rituximab (Mabthera; Roche, Brussels, Belgium) has demonstrated activity in the treatment of non-Hodgkin's lymphoma (NHL) of B cell origin.1 This antibody, which binds specifically to the CD20 antigen, a pan B cell marker, can deplete B cells through complement- and antibody-dependent cellular cytotoxicity. Rituximab is presumably active in Waldenström's macroglobulinemia (WM) because of the elimination of either CD20+ clonotypic precursor B cells, or CD20+ plasma cells, which have both been detected in most WM patients.2 The same rationale could apply to other immunoglobulin-mediated diseases of B lymphocytes, such as cold agglutinin disease (CAD) or cryoglobulinemia, which could also theoretically benefit from CD20 targeted therapy. We hereby describe a patient with refractory CAD who went into remission after therapy with Rituximab.

A 67-year-old patient presented in April 1997 for acrocyanosis, weakness and dyspnoea. Her medical history included the diagnosis of an indolent NHL of the bone marrow (BM) in 1993, with secondary CAD. She had received chlorambucil (CLB) daily (5 mg/d) from January 1993 until April 1996 with resolution of signs of hemolysis. There were no enlarged lymph nodes. There was no hepatosplenomegaly. Laboratory studies showed a hemoglobin of 7.6 g/dl, a hematocrit of 23%, and a reticulocyte count of 66 000/μl. The white blood cell count was 1770/μl with 51% neutrophils, 32% lymphocytes, and no abnormal forms. The platelet count was 37 000/μl. LDH were at 610 UI/l and total bilirubin was 1.7 mg/dl. Haptoglobin level was <12 mg/dl. The direct antiglobulin test was positive with IgM and complement. At elution, a monoclonal IgM with anti-I cold-agglutinin activity was found. BM analysis showed a diffuse and nodular lympho-plasmocytoid infiltrate (52% of total cellularity) with focal reticulinic fibrosis, together with erythroid hyperplasia and discrete dyserythropoiesis. Megakaryocytes were at the lower normal limit. Clonal rearrangement of the immunoglobulin heavy chain gene was found. The karyotype was normal on 10 mitoses. Whole-body CT scan was normal.

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  1. Service d'Hématologie et de Biologie Hématologique, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium

    N Layios, E Van Den Neste, E Jost, V Deneys, JM Scheiff & A Ferrant

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  1. N Layios
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  2. E Van Den Neste
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Layios, N., Van Den Neste, E., Jost, E. et al. Remission of severe cold agglutinin disease Rituximab therapy. Leukemia 15, 187–188 (2001). https://doi.org/10.1038/sj.leu.2401976

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