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Hemophagocytosis after bone marrow transplantation for JAK3‐deficient severe combined immunodeficiency
Author(s) -
Hashii Yoshiko,
Yoshida Hisao,
Kuroda Sato,
Kusuki Shigenori,
Sato Emiko,
Tokimasa Sadao,
Ohta Hideaki,
Matsubara Yasutaka,
Kinoshita Seiji,
Nakagawa Noriko,
Imai Kohsuke,
oyama Shigeaki,
Oshima Koichi,
Ohara Osamu,
Ozono Keiichi
Publication year - 2010
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2009.01217.x
Subject(s) - hemophagocytosis , medicine , severe combined immunodeficiency , immunology , tacrolimus , bone marrow , methotrexate , bone marrow transplantation , transplantation , biochemistry , pancytopenia , gene , chemistry
Hashii Y, Yoshida H, Kuroda S, Kusuki S, Sato E, Tokimasa S, Ohta H, Matsubara Y, Kinoshita S, Nakagawa N, Imai K, Nonoyama S, Oshima K, Ohara O, Ozono K. Hemophagocytosis after bone marrow transplantation for JAK3‐deficient severe combined immunodeficiency.
Pediatr Transplantation 2010: 14:E105–E109. © 2009 John Wiley & Sons A/S. Abstract: HSCT is the optimal treatment for patients with SCID. In particular, HSCT from a HLA‐identical donor gives rise to successful engraftment with long survival. We report a six‐month‐old girl with JAK3‐deficient SCID who developed hemophagocytosis after BMT without conditioning from her HLA‐identical father. She had suffered from pneumonia and hepatitis before BMT. Prophylaxis for GVHD was short‐term methotrexate and tacrolimus. On day 18 after BMT, the patient developed hemophagocytosis in bone marrow when donor lymphocytes were increasing in peripheral blood. Analysis of chimerism confirmed host origin of macrophages and donor origin of lymphocytes. Thus, host macrophage activation was presumably induced in response to donor lymphocytes through immunoreaction to infections and/or alloantigens. HSCT for SCID necessitates caution with respect to hemophagocytosis.