Abstract
Hemophagocytic syndrome is a rare disorder mainly affecting children. Symptoms include prolonged fever, hepatosplenomegaly and cytopenias. Allogeneic stem cell transplant appears to provide the best overall cure rate in this disease. The authors report a young boy, the second child of consanguineous parents, diagnosed with familial hemophagocytic lymphohistiocytosis (HLH) who underwent allogeneic stem cell transplant form HLA matched father.
References
Henter JI, Horne A, Aricó M, et al. HLH 2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124–31.
Allen CE, Yu X, Kozinetz CA, McClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008;50:1227–35.
Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr. 2007;166:95–109.
Janka G, Henter JI, Imashuku S. Clinical aspects and therapy of hemophagocytic lymphohistiocytosis. In: Carroll WL, Finlay JL ,eds. Histiocytic Disorders of Children and Adults. Los Angeles & New York:Cambridge University Press; 2005.pp.353-79.
Karras A, Hermine O. Hemophagocytic syndrome. Rev Med Interne. 2002;23:768–78.
Aricò M, Janka G, Fischer A, et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL study group of the histiocyte society. Leukemia. 1996;10:197–203.
Ohadi M, Lalloz M, Sham P, et al. Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q21.3-22 by homozygosity mapping. Am J Hum Genet. 1999;64:165–71.
Horne A, Janka G, Maarten Egeler R, Gadner H, et al. Haematopoietic stem cell transplantation in haemophagocytic lymphohistiocytosis. Br J Haematol. 2005;129:622–30.
Cesaro S, Locatelli F, Lanino E, et al. Hematopoietic stem cell transplantation for hemophagocytic lymphohistiocytosis: a retrospective analysis of data from the Italian Association of Pediatric Hematology Oncology (AIEOP). Haematologica. 2008;93:1694–701.
Hakim A. Comments on consanguineous marriages in Pakistan. Pak Dev Rev. 1994;33:675–6.
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Ali, N., Fadoo, Z., Masood, N. et al. Successful Engraftment and Survival Following Allogeneic Hematopoietic Stem Cell Transplant in a Child with Familial Hemophagocytic Lymphohistiocytosis. Indian J Pediatr 80, 65–66 (2013). https://doi.org/10.1007/s12098-011-0658-8
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DOI: https://doi.org/10.1007/s12098-011-0658-8