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Hematology


Answer 2
  1. Chlorambucil or another alkylating agent. This patient has developed symptomatic WM requiring therapy. Given the severity of this patient’s symptoms, observation alone would be inappropriate. With anemia and a positive Coombs’ test in this patient, cold agglutinin syndrome2 or warm antibody-mediated autoimmune hemolytic anemia (AIHA) should be considered. Although fludarabine or other purine analogues are used to treat WM, a patient with a positive baseline Coombs’ test may be at risk for a life-threatening exacerbation of AIHA following treatment with these agents, as has been described in patients with chronic lymphocytic leukemia.3 Chlorambucil would be a reasonable alternative, as up to 60% of patients with WM respond to this therapy.2 Compared with chlorambucil monotherapy, treatment with multidrug combinations does not improve outcomes and may increase toxicity. Prednisone 1 mg/kg/day would likely be helpful for treatment of AIHA but would not adequately address the patient’s other symptoms, such as splenomegaly.

    REFERENCES
    2.  Johnson SA, Birchall J, Luckie C, et al; Haemato-Oncology Task Force of the British Committee for Standards in Haematology. Guidelines on the management of Waldenström macroglobulinaemia. Br J Haematol 2006;132:683-97.

    3. Weiss RB, Freiman J, Kweder SL, et al. Hemolytic anemia after fludarabine therapy for chronic lymphocytic leukemia. J Clin Oncol 1998;16:1885-9.

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