Therapy for Chronic Cold Agglutinin Disease

• Status: Completed
• Trial end date: 2010-05-01
• Target enrollment: 0
• Participant gender: All

Summary

Chronic cold agglutinin disease (CAD) is a type of autoimmune hemolytic anemia (anemia due to destruction of red blood cells by abnormal antibodies). Almost all patients also suffer from cold-induced disturbances of blood circulation. The purpose of this study is to assess the efficacy and safety of combination therapy with rituximab (an antibody against B lymphocytes) and fludarabine (a cytotoxic drug) for CAD. Another aim is to try to assess whether these agents in combination are better than single agent therapy with rituximab.

• Phase: Phase 2
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: University of Bergen
• Treatments: Agglutinins; Cold agglutinins; Fludarabine; Fludarabine phosphate; Rituximab; Vidarabine

Criteria

Inclusion Criteria:

1. CAD diagnosis defined by the combination of:

• Chronic haemolysis

• Cold agglutinin titre > 64

• Positive direct antiglobulin test when performed with polyspecific antiserum, negative (or only weakly positive) with anti-IgG, and strongly positive with anti-C3d

2. The presence of a clonal B-cell lymphoproliferative disorder defined by:

• Monoclonal IgMκ band by serum electrophoresis and immunofixation, and

• Lymphocyte phenotype with κ/λ-ratio > 3.5 and CD20+,κ+ co-expression, using flowcytometric immunophenotyping of bone marrow aspirates

3. Clinical symptoms requiring treatment, such as anaemia or Raynaud-like symptoms

4. Informed consent

Exclusion Criteria:

1. An aggressive lymphoma

2. Blood lymphocyte count > 50 . 109/L

3. Non-lymphatic malignant disease other than basal cell carcinoma

4. Contra-indications to rituximab or fludarabine therapy

5. Inability to cooperate