Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS)

V. Koneti Rao, Susan Price, Katie Perkins, Patricia Aldridge, Jean Tretler, Joie Davis, Janet K. Dale, Fred Gill, Kip R. Hartman, Linda Stork, David J. Gnarra, Lakshmanan Krishnamurti, Peter E. Newburger, Jennifer Puck, Thomas Fleisher

Research output: Contribution to journalArticle

53 Citations (Scopus)

Abstract

Background. ALPS is a disorder of apoptosis resulting in accumulation of autoreactive lymphocytes, leading to marked lymphadenopathy, hepatosplenomegaly, and multilineage cytopenias due to splenic sequestration and/or autoimmune destruction often presenting in childhood. We summarize our experience of rituximab use during the last 8 years in 12 patients, 9 children, and 3 adults, out of 259 individuals with ALPS, belonging to 166 families currently enrolled in studies at the National Institutes of Health. Methods. Refractory immune thrombocytopenia (platelet count

Original languageEnglish (US)
Pages (from-to)847-852
Number of pages6
JournalPediatric Blood and Cancer
Volume52
Issue number7
DOIs
StatePublished - Jul 1 2009

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Autoimmune Lymphoproliferative Syndrome
Idiopathic Thrombocytopenic Purpura
National Institutes of Health (U.S.)
Platelet Count
Lymphocytes
Apoptosis
Rituximab

Keywords

  • ALPS
  • Cytopenias
  • Lymphadenopathy
  • Rituximab
  • Splenomegaly

ASJC Scopus subject areas

  • Oncology
  • Pediatrics, Perinatology, and Child Health
  • Hematology

Cite this

Rao, V. K., Price, S., Perkins, K., Aldridge, P., Tretler, J., Davis, J., ... Fleisher, T. (2009). Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS). Pediatric Blood and Cancer, 52(7), 847-852. https://doi.org/10.1002/pbc.21965

Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS). / Rao, V. Koneti; Price, Susan; Perkins, Katie; Aldridge, Patricia; Tretler, Jean; Davis, Joie; Dale, Janet K.; Gill, Fred; Hartman, Kip R.; Stork, Linda; Gnarra, David J.; Krishnamurti, Lakshmanan; Newburger, Peter E.; Puck, Jennifer; Fleisher, Thomas.

In: Pediatric Blood and Cancer, Vol. 52, No. 7, 01.07.2009, p. 847-852.

Research output: Contribution to journalArticle

Rao, VK, Price, S, Perkins, K, Aldridge, P, Tretler, J, Davis, J, Dale, JK, Gill, F, Hartman, KR, Stork, L, Gnarra, DJ, Krishnamurti, L, Newburger, PE, Puck, J & Fleisher, T 2009, 'Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS)', Pediatric Blood and Cancer, vol. 52, no. 7, pp. 847-852. https://doi.org/10.1002/pbc.21965
Rao, V. Koneti ; Price, Susan ; Perkins, Katie ; Aldridge, Patricia ; Tretler, Jean ; Davis, Joie ; Dale, Janet K. ; Gill, Fred ; Hartman, Kip R. ; Stork, Linda ; Gnarra, David J. ; Krishnamurti, Lakshmanan ; Newburger, Peter E. ; Puck, Jennifer ; Fleisher, Thomas. / Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS). In: Pediatric Blood and Cancer. 2009 ; Vol. 52, No. 7. pp. 847-852.
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