X-linked Lymphoproliferative Syndrome Medication

  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Mar 9, 2011
 

Medication Summary

No clearly effective medications exist for X-linked lymphoproliferative disease (XLP), although cytotoxic chemotherapy agents may be useful. Further study is needed.

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Antineoplastic Agents

Class Summary

Antineoplastic agents inhibit cell growth and proliferation.

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Etoposide (VePesid, VP-16)

 

Topoisomerase II inhibitor that leads to single-strand DNA breaks and cell cycle arrest. Has activity in a number of tumors, including small cell lung cancer, germ cell tumors, and lymphoma.

One reported case used 200 mg/m2/d IV for 3 d during acute EBV infection in a boy aged 6 years. Led to dramatic, although temporary, improvement. Little data support etoposide therapy in this syndrome.

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Monoclonal Antibody

Class Summary

Monoclonal antibodies are genetically engineered antibodies directed against specific antigens found in targeted cells.

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Rituximab (Rituxan)

 

Genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of B lymphocytes.

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Blood Products

Class Summary

Blood products are use for improvement of immunodeficiency.

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Immune globulin, intravenous (Gamimune, Gammar-P, Sandoglobulin, Gammagard)

 

Limited literature suggests that the use of intravenous immunoglobulin may help speed resolution of the acute IM syndrome and prevent some secondary infections due to humoral immunodeficiency. No controlled studies exist, and its use is still speculative.

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Contributor Information and Disclosures
Author

Karen Seiter, MD  Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology

Disclosure: Novartis Honoraria Speaking and teaching; Schering Honoraria Speaking and teaching; Cephalon Honoraria Speaking and teaching; Celgene Honoraria Speaking and teaching

Coauthor(s)

Doris Ponce, MD  Fellow, Department of Hematology/Oncology, New York Medical College

Doris Ponce, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Clinical Oncology, and American Society of Hematology

Disclosure: Nothing to disclose.

M Wayne Saville, MD  Associate Professor of Clinical Medicine, University of California at San Diego; Director, Hematology and Oncology, Global Medical Affairs, Biogen Idec, Inc

M Wayne Saville, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Koyamangalath Krishnan, MD, FRCP, FACP  Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, Program Director, Hematology-Oncology Fellowship, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Troy H Guthrie, Jr, MD  Director of Cancer Institute, Baptist Medical Center

Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University

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

Disclosure: Nothing to disclose.

References

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