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:
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Antibiotics for infections
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Complication-specific management of autoimmune disorders, hemolytic anemia, and other conditions
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Splenectomy or surgical debulking of massive, symptomatic lymphadenopathy (rarely necessary)
Consultations
Possible consultations may include oncologists, hematologists, and/or oncologic surgeons.
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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.
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Lateral radiograph of the skull. This image demonstrates numerous lytic lesions, which are typical for the appearance of widespread myeloma.
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Bone marrow biopsy specimen.
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Bone marrow biopsy specimen in fixative solution.
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Bone marrow aspiration and biopsy slides before staining.
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Histology of eosinophilic granuloma.
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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.
