Gamma Heavy Chain Disease Treatment & Management
- Author: Guy B Faguet, MD; Chief Editor: Emmanuel C Besa, MD more...
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.[12, 13, 14]
Intravenous Ig has been used in certain cases associated 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)
Possible consultations may include oncologists, hematologists, and/or oncologic surgeons.
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