eMedicine Specialties > Hematology > Stem Cells and Disorders

Lymphoproliferative Syndrome, X-linked: Follow-up

Author: Karen Seiter, MD, Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College
Coauthor(s): Doris Ponce, MD, Fellow, Department of Hematology/Oncology, New York Medical College; 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
Contributor Information and Disclosures

Updated: Sep 15, 2008

Follow-up

Complications

Prognosis

  • Except for reported cases of optimal outcome after monoclonal anti-CD20, and remission due to stem cell transplantation, few children with X-linked lymphoproliferative syndrome (XLP) survive to adulthood. Therefore, most patients with available matched donors apparently should be referred for transplantation. However, more data are needed to substantiate the efficacy of this form of therapy for X-linked lymphoproliferative disease (XLP).

Miscellaneous

Medicolegal Pitfalls

  • X-linked lymphoproliferative syndrome (XLP) should be suspected in the case of an exceptionally severe lymphadenopathic reaction in a patient with mononucleosis. Otherwise, the rarity of X-linked lymphoproliferative syndrome (XLP) suggests little likelihood of legal risk.
 


More on Lymphoproliferative Syndrome, X-linked

Overview: Lymphoproliferative Syndrome, X-linked
Differential Diagnoses & Workup: Lymphoproliferative Syndrome, X-linked
Treatment & Medication: Lymphoproliferative Syndrome, X-linked
Follow-up: Lymphoproliferative Syndrome, X-linked
References

References

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

  3. Lankester AC, Visser LF, Hartwig NG, et al. Allogeneic stem cell transplantation in X-linked lymphoproliferative disease: two cases in one family and review of the literature. Bone Marrow Transplant. Jul 2005;36(2):99-105. [Medline].

  4. Milone MC, Tsai DE, Hodinka RL, et al. Treatment of primary Epstein-Barr virus infection in patients with X-linked lymphoproliferative disease using B-cell-directed therapy. Blood. Feb 1 2005;105(3):994-6. [Medline][Full Text].

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  6. Brandau O, Schuster V, Weiss M, et al. Epstein-Barr virus-negative boys with non-Hodgkin lymphoma are mutated in the SH2D1A gene, as are patients with X-linked lymphoproliferative disease (XLP). Hum Mol Genet. Dec 1999;8(13):2407-13. [Medline][Full Text].

  7. Coffey AJ, Brooksbank RA, Brandau O, et al. Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene. Nat Genet. Oct 1998;20(2):129-35. [Medline].

  8. Nichols KE, Harkin DP, Levitz S, et al. Inactivating mutations in an SH2 domain-encoding gene in X-linked lymphoproliferative syndrome. Proc Natl Acad Sci U S A. Nov 10 1998;95(23):13765-70. [Medline][Full Text].

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  12. Tangye SG, Lazetic S, Woollatt E, et al. Cutting edge: human 2B4, an activating NK cell receptor, recruits the protein tyrosine phosphatase SHP-2 and the adaptor signaling protein SAP. J Immunol. Jun 15 1999;162(12):6981-5. [Medline][Full Text].

  13. Sayos J, Wu C, Morra M, et al. The X-linked lymphoproliferative-disease gene product SAP regulates signals induced through the co-receptor SLAM. Nature. Oct 1 1998;395(6701):462-9. [Medline].

  14. Gross TG, Filipovich AH, Conley ME, et al. Cure of X-linked lymphoproliferative disease (XLP) with allogeneic hematopoietic stem cell transplantation (HSCT): report from the XLP registry. Bone Marrow Transplant. May 1996;17(5):741-4. [Medline].

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Further Reading

Keywords

X-linked lymphoproliferative syndrome, X-linked lymphoproliferative disorder, lymphoproliferative disorders, lymphoproliferative diseases, XLP syndrome, Duncan syndrome, Duncan's syndrome, X-linked recessive progressive combined variable immunodeficiency syndrome, familial fatal EBV infection, Purtilo syndrome, Epstein-Barr virus, EBV, infectious mononucleosis, hypogammaglobulinemia, lymphoma

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

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.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME Editor

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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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