Lymphoproliferative Disorders Treatment & Management

Updated: Dec 09, 2014
  • Author: Donna A Wall, MD; Chief Editor: Robert J Arceci, MD, PhD  more...
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Treatment

Medical Care

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  • Children with inherited immunodeficiency syndromes
    • Truly malignant neoplasms are sometimes difficult to differentiate from nonmalignant lymphoproliferative disorders (LPDs) with aggressive features. When the underlying immunodeficiency manifested to only a minor degree and when the histologic features are marked nuclear atypia and other features of a high-grade neoplasm, standard chemotherapeutic regimens are usually recommended. These regimens include cyclophosphamide, prednisone, vincristine, and doxorubicin.
    • In other cases, local control of the lymphoproliferative disorder by using surgical resection or irradiation with adjunctive interleukin-2 or monoclonal antibody therapy may prove beneficial.
    • Boys with X-linked immunodeficiency syndrome appear to benefit from immunoglobulin therapy.
    • If cytotoxic therapy is chosen in child with an underlying immunodeficiency syndrome, myelosuppressive therapy may worsen their immunocompromise beyond what is ordinarily expected. Therefore, care should be taken to begin support for febrile neutropenia and other infections in a timely fashion. As described above, bone marrow reconstitution with an immunocompetent donor appears to be the best method to prevent lymphoproliferative disorders in children with severe inherited immunodeficiency syndromes.
  • Patients with posttransplant lymphoproliferative disorder (PTLD)
    • PTLDs are varied and somewhat depend on the nature of the allograft and on the immunosuppressive agents used to prevent graft (or host) rejection. The histologic grades of lymphoproliferative disorders can vary widely in this setting and range from a benign oligoclonal expansion of lymphoid cells to a high-grade neoplastic process. Low-grade tumors usually respond favorably to a reduction in immunosuppression, whereas high-grade tumors may require chemotherapy, irradiation, and/or surgery.
    • Cyclosporin A and antithymocyte globulin are associated with the development of lymphoproliferative disorders within months of transplantation, often in the GI tract. In many instances, Epstein-Barr virus (EBV) DNA transcripts can be identified with Southern blotting, anti-EBV-encoded RNA (EBER) staining, or polymerase chain reaction (PCR), but results of serologic tests are frequently nonreactive. The lymphocytic infiltration into transplanted organs can often mimic organ rejection.
    • In contrast to the lymphoproliferative disorders observed in primary immunodeficiency syndromes, a sometimes successful treatment after transplantation is to decrease or discontinue immunosuppressive drug therapy.
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Surgical Care

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  • Surgical resection can sometimes play an important role in managing lymphoproliferative disorders.
  • Circumstances are limited to obtaining enough tissue to make a diagnosis and to debulking large tumors that compromise surrounding vital structures. However, in most cases, the primary means to control lymphoproliferative disorders is medical management.
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Consultations

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  • In children with a suspected lymphoproliferative disorders, consultation with a physician familiar with the underlying immunodeficiency syndrome is indicated, in addition to consultation with a pediatric oncologist.
  • Consider an infectious process with appropriate consultation with a pediatric infectious disease specialist.
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Diet

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  • In children, diet does not appear to play a role in the pathogenesis or treatment of lymphoproliferative disorders.
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Activity

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  • Activity does not appear to play a role in the treatment or pathogenesis of lymphoproliferative disorders.
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