Mu Heavy Chain Disease Treatment & Management

Updated: Feb 10, 2020
  • Author: Jessica Katz, MD, PhD, FACP; Chief Editor: Emmanuel C Besa, MD  more...
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Treatment

Approach Considerations

Because of the paucity of cases, no standard treatment has been established for mu heavy chain disease (mu-HCD). Currently, the identification of a mu-HCD protein in the serum of an apparently healthy patient should be considered a monoclonal gammopathy of undetermined significance, and the patient should be monitored closely for the development of a symptomatic lymphoproliferative disorder.

Once a lymphoproliferative disorder develops, chemotherapeutic agents are used as appropriate for the patient's disorder, stage, and clinical situation (eg, combinations of proteasome inhibitors, steroids, and immunomodulatory agents for multiple myeloma; purine analogs, alkylating agents, and monoclonal antibodies for chronic lymphocytic leukemia). Successful treatment with fludarabine has been reported in two cases. [15] The details of some of these therapies can be found in Chronic Lymphocytic Leukemia and Multiple Myeloma.

If no overt lymphoproliferative disorder is found, the patient should be evaluated every few months to assess the abnormal protein. Reasonable follow-up intervals are every 3 months in the first year following diagnosis, every 4 months for the next year, and every 6 months thereafter. The patient should be evaluated earlier if symptoms occur. If the patient is symptomatic in any way, then radiologic assessment and other diagnostic procedures for lymphoproliferative disorders are essential.

Surgical care usually is not required, although special circumstances may require surgery (eg, surgery needed to fix a pathologic fracture). Occasionally, consultation with a radiation oncologist may be indicated to reduce risk of pathologic fracture, to treat a pathologic fracture site after surgical correction, or to treat a site of symptomatic bony involvement.