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Gamma Heavy Chain Disease Treatment & Management

  • Author: Guy B Faguet, MD; Chief Editor: Emmanuel C Besa, MD  more...
Updated: May 19, 2014

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.[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.

Contributor Information and Disclosures

Guy B Faguet, MD Retired Professor, Department of Medicine, Section of Hematology and Oncology, Georgia Regents University

Guy B Faguet, MD is a member of the following medical societies: American Association of Immunologists, American Society of Hematology, International Society of Hematology, New York Academy of Sciences, Southern Medical Association, Southern Society for Clinical Investigation, American Federation for Clinical Research, Southeastern Cancer Research Association, Polycythemia Vera Study Group

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.


Wendy Brick, MD Consulting Staff, Department of Internal Medicine, Division of Hematology and Oncology, Mecklenburg Medical Group

Wendy Brick, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Medical Association, and American Society of Hematology

Disclosure: Nothing to disclose.

Russell Burgess, MD (Retired) Chief, Division of Hematology/Oncology, Eastern Carolina Internal Medicine, PA

Russell Burgess, MD is a member of the following medical societies: American College of Physicians and American Medical Assocation

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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The margins of this massive spleen were palpated easily preoperatively. Medially, the 3.18-kg (7-lb) spleen crosses the midline. Inferiorly, it extends into the pelvis.
Lateral radiograph of the skull. This image demonstrates numerous lytic lesions, which are typical for the appearance of widespread myeloma.
Bone marrow biopsy specimen.
Bone marrow biopsy specimen in fixative solution.
Bone marrow aspiration and biopsy slides before staining.
Histology of eosinophilic granuloma.
Radiograph of the right femur. This image demonstrates the typical appearance of a single myeloma lesion as a well-circumscribed lucency in the intertrochanteric region. Smaller lesions are seen at the greater trochanter.
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