Gamma Heavy Chain Disease Treatment & Management

Updated: Feb 12, 2020
  • Author: Guy B Faguet, MD; Chief Editor: Emmanuel C Besa, MD  more...
  • Print
Treatment

Medical Care

Watchful observation is warranted in asymptomatic patients who have low serum IgG levels and no additional evidence of disease (monoclonal gammopathy of undetermined significance [MGUS]–like presentation).

Management of γ-heavy chain disease (HCD) should focus on palliative care, as disease treatment has been disappointing.

Chemotherapy, mostly using agents efficacious in lymphoma and multiple myeloma (eg, cyclophosphamide, prednisone, vincristine, chlorambucil, doxorubicin), has been disappointing. However, partial and short-lived responses have been reported using fludarabine and/or rituximab. [16, 17, 18]

Intravenous immunoglobulin (IVIg) has been used in select patients with severe hypogammaglobulinemia, although no clinical studies support the practice.

Management of complications is as follows:

  • Antibiotics for infections
  • Complication-specific management of autoimmune disorders, hemolytic anemia, and other conditions
  • Splenectomy or surgical debulking of massive, symptomatic lymphadenopathy (rarely necessary)
Next:

Consultations

Possible consultations may include oncologists, hematologists, and/or oncologic surgeons.

Previous