Medication Summary
Chemotherapy is typically ineffective in γ-heavy chain disease (HCD). Agents that are effective in lymphoma and multiple myeloma have generally been used, but results have been disappointing. Treatment with fludarabine and/or rituximab has resulted in partial, short-lived responses.
Antineoplastic Agents
Class Summary
Antineoplastic agents inhibit cell growth and proliferation.
Fludarabine (Fludara, Oforta)
Fludarabine is a purine analogue that interferes with DNA synthesis by inhibiting ribonucleotide reductase. It is also incorporated into RNA, causing inhibition of RNA and protein synthesis; however, its primary effect may result from activation of apoptosis.
Rituximab (Rituxan)
Rituximab is a genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. The antibody is an immunoglobulin G1 (IgG1) kappa immunoglobulin containing murine light- and heavy-chain variable region sequences and human constant region sequences.
Vincristine (Oncovin, Vincasar PFS)
Vincristine is a vesicant agent that blocks mitosis by arresting cells in metaphase.
Doxorubicin (Adriamycin)
Doxorubicin is an anthracycline and antimicrobial compound produced by Streptomyces in culture, which causes topoisomerase-II–dependent DNA cleavage and intercalation with the DNA double helix.
Cyclophosphamide (Cytoxan)
Cyclophosphamide is a nitrogen-mustard alkylating compound that produces highly reactive carbonium ions, which react with electron-rich areas of susceptible molecules. It is used to treat a large number of malignancies.
Chlorambucil (Leukeran)
Chlorambucil is a nitrogen-mustard alkylating agent, generally used for the treatment of chronic lymphocytic leukemia, Waldenstrom macroglobulinemia, and as a second- or third-line therapy for patients with lymphomas. It alkylates cross-link the strands of DNA, inhibiting DNA replication and RNA transcription. It may be given as a single agent or as part of a multidrug treatment regimen; depending on protocol of choice, the dose and schedule of administration are decided.
Corticosteroids
Class Summary
Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. These agents modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Sterapred, Orasone)
Prednisone suppresses mRNA synthesis and causes lysis of lymphoid tumors. Cell death results from cell fragmentation.
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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.
