Lymphoproliferative Disorders Treatment & Management

  • Author: Stuart S Winter, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Nov 24, 2009
 

Medical Care

  • 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.
Next

Surgical Care

  • 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.
Previous
Next

Consultations

  • 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.
Previous
Next

Diet

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

Activity

  • Activity does not appear to play a role in the treatment or pathogenesis of lymphoproliferative disorders.
Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Stuart S Winter, MD  Associate Professor, Department of Pediatrics, University of New Mexico Health Sciences Center

Stuart S Winter, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, New Mexico Pediatric Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology Institute, UCLA

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, University of Cincinnati College of Medicine; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, Cincinnati Children's Hospital Medical Center

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

References

  1. Spector BD, Perry GS III, Kersey JH. Genetically determined immunodeficiency diseases (GDID) and malignancy: report from the immunodeficiency--cancer registry. Clin Immunol Immunopathol. Sep 1978;11(1):12-29. [Medline].

  2. Engel P, Eck MJ, Terhorst C. The SAP and SLAM families in immune responses and X-linked lymphoproliferative disease. Nat Rev Immunol. Oct 2003;3(10):813-21. [Medline].

  3. Latour S. Natural killer T cells and X-linked lymphoproliferative syndrome. Curr Opin Allergy Clin Immunol. Dec 2007;7(6):510-4. [Medline].

  4. Worth A, Thrasher AJ, Gaspar HB. Autoimmune lymphoproliferative syndrome: molecular basis of disease and clinical phenotype. Br J Haematol. Apr 2006;133(2):124-40. [Medline].

  5. Kaplan J, De Domenico I, Ward DM. Chediak-Higashi syndrome. Curr Opin Hematol. Jan 2008;15(1):22-9. [Medline].

  6. Notarangelo LD, Miao CH, Ochs HD. Wiskott-Aldrich syndrome. Curr Opin Hematol. Jan 2008;15(1):30-6. [Medline].

  7. Ohta H, Fukushima N, Ozono K. Pediatric post-transplant lymphoproliferative disorder after cardiac transplantation. Int J Hematol. Sep 2009;90(2):127-36. [Medline].

  8. Baehner RL. Lymphocytes. In: Miller DR, Baehner RL, eds. Blood Diseases in Infancy and Childhood. St. Louis, MO: CV Mosby; 1990:578-603.

  9. Bird AG, Britton S. The relationship between Epstein-Barr virus and lymphoma. Semin Hematol. Oct 1982;19(4):285-300. [Medline].

  10. Brusamolino E, Pagnucco G, Bernasconi C. Secondary lymphomas: a review on lymphoproliferative diseases arising in immunocompromised hosts: prevalence, clinical features and pathogenetic mechanisms. Haematologica. Nov-Dec 1989;74(6):605-22. [Medline].

  11. Certain S, Barrat F, Pastural E, et al. Protein truncation test of LYST reveals heterogenous mutations in patients with Chediak-Higashi syndrome. Blood. Feb 1 2000;95(3):979-83. [Medline].

  12. Craig FE, Gulley ML, Banks PM. Posttransplantation lymphoproliferative disorders. Am J Clin Pathol. Mar 1993;99(3):265-76. [Medline].

  13. Fiorilli M, Carbonari M, Crescenzi M, et al. T-cell receptor genes and ataxia telangiectasia. Nature. Jan 17-23 1985;313(5999):186. [Medline].

  14. Kempf W. CD30+ lymphoproliferative disorders: histopathology, differential diagnosis, new variants, and simulators. J Cutan Pathol. Feb 2006;33 Suppl 1:58-70. [Medline].

  15. Locker J, Nalesnik M. Molecular genetic analysis of lymphoid tumors arising after organ transplantation. Am J Pathol. Dec 1989;135(6):977-87. [Medline].

  16. Loughrey M, Trivett M, Lade S, et al. Diagnostic application of Epstein-Barr virus-encoded RNA in situ hybridisation. Pathology. Aug 2004;36(4):301-8. [Medline].

  17. Lundell R, Elenitoba-Johnson KS, Lim MS. T-cell posttransplant lymphoproliferative disorder occurring in a pediatric solid-organ transplant patient. Am J Surg Pathol. Jul 2004;28(7):967-73. [Medline].

  18. Marti GE, Rawstron AC, Ghia P, et al. Diagnostic criteria for monoclonal B-cell lymphocytosis. Br J Haematol. Aug 2005;130(3):325-32. [Medline].

  19. Nichols KE. X-linked lymphoproliferative disease: genetics and biochemistry. Rev Immunogenet. 2000;2(2):256-66. [Medline].

  20. Rigaud S, Fondaneche MC, Lambert N, et al. XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome. Nature. Nov 2 2006;444(7115):110-4. [Medline].

  21. Sanal O, Wei S, Foroud T, et al. Further mapping of an ataxia-telangiectasia locus to the chromosome 11q23 region. Am J Hum Genet. Nov 1990;47(5):860-6. [Medline].

  22. Sander CA, Medeiros LJ, Weiss LM, et al. Lymphoproliferative lesions in patients with common variable immunodeficiency syndrome. Am J Surg Pathol. Dec 1992;16(12):1170-82. [Medline].

  23. Snyder MJ, Stenzel TT, Buckley PJ, et al. Posttransplant lymphoproliferative disorder following nonmyeloablative allogeneic stem cell transplantation. Am J Surg Pathol. Jun 2004;28(6):794-800. [Medline].

  24. Straus SE, Sneller M, Lenardo MJ, et al. An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. Ann Intern Med. Apr 6 1999;130(7):591-601. [Medline].

  25. Su HC, Lenardo MJ. Genetic defects of apoptosis and primary immunodeficiency. Immunol Allergy Clin North Am. May 2008;28(2):329-51, ix. [Medline].

  26. Uzel G. The range of defects associated with nuclear factor kappaB essential modulator. Curr Opin Allergy Clin Immunol. Dec 2005;5(6):513-8. [Medline].

  27. Vetrie D, Vorechovsky I, Sideras P, et al. The gene involved in X-linked agammaglobulinaemia is a member of the src family of protein-tyrosine kinases. Nature. Jan 21 1993;361(6409):226-33. [Medline].

 
Previous
Next
 
Parenchymal nodularities.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.