X-linked Lymphoproliferative Syndrome Medication

Updated: Jan 27, 2019
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
  • Print
Medication

Medication Summary

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

Next:

Antineoplastic Agents

Class Summary

Antineoplastic agents inhibit cell growth and proliferation.

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.

Previous
Next:

Monoclonal Antibody

Class Summary

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

Rituximab (Rituxan)

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

Previous
Next:

Blood Products

Class Summary

Blood products are use for improvement of immunodeficiency.

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.

Previous