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Autoimmune Lymphoproliferative Syndrome with Red Cell Aplasia

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Abstract

Autoimmune Lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of abnormal lymphocyte apoptosis, leading to chronic lymphoproliferation. It presents as lymphadenopathy, hepatosplenomegaly and autoimmune phenomena. Pure red cell aplasia is characterized by normochromic normocytic anemia, reticulocytopenia, and absence of erythroblasts from a normal bone marrow. Only few lymphoproliferative disorders have been associated with erythroid aplasia. The authors are reporting a case of ALPS associated with red cell aplasia in a 7-y-old girl.

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References

  1. Madkaikar M, Mishra A, Ghosh K. Diagnostic approach to primary immunodeficiency disorders. Indian Pediatr. 2013;50:579–86.

    Article  CAS  PubMed  Google Scholar 

  2. Rao VK, Oliveira JB. How I treat autoimmune lymphoproliferative syndrome. Blood. 2011;118:5741–51.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  3. Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol. 2008;142:505–14.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  4. Drappa J, Vaishnaw AK, Sullivan KE, Chu JL, Elkon KB. Fas gene mutations in the Canale-smith syndrome, an inherited lymphoproliferative disorder associated with autoimmunity. N Engl J Med. 1996;335:1643–9.

    Article  CAS  PubMed  Google Scholar 

  5. Oliveira JB, Bleesing JJ, Dianzani U, et al. Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop. Blood. 2010;116:e35–40.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  6. John MJ, Rajasekhar R, Mathews V. Autoimmune lymphoproliferative syndrome (ALPS): a rare cause of immune cytopenia. Indian Pediatr. 2008;45:148–50.

    PubMed  Google Scholar 

  7. Vlachaki E, Diamantidis MD, Klonizakis P, Haralambidou-Vranitsa S, Ioannidou-Papagiannaki E, Klonizakis I. Pure red cell aplasia and lymphoproliferative disorders: an infrequent association. Sci World J. 2012;2012:475313.

    Article  Google Scholar 

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Contributions

KRM was involved in investigation support and development of the draft. SB was responsible for the care of the patient and draft preparation. KCT was responsible for finalization of the draft and will act as guarantor of the paper.

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Authors and Affiliations

  1. Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, 110029, India

    K. R. Meena, Supriya Bisht & K. C. Tamaria

Authors
  1. K. R. Meena
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  2. Supriya Bisht
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  3. K. C. Tamaria
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Correspondence to Supriya Bisht.

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Meena, K.R., Bisht, S. & Tamaria, K.C. Autoimmune Lymphoproliferative Syndrome with Red Cell Aplasia. Indian J Pediatr 82, 1172–1174 (2015). https://doi.org/10.1007/s12098-015-1779-2

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  • DOI: https://doi.org/10.1007/s12098-015-1779-2

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