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Adalimumab for treatment of hemophagocytic syndrome following unrelated bone marrow transplantation in a boy with Behcet’s disease and secondary myelodysplastic syndrome

Bone Marrow Transplantation volume 53, pages 1214–1217 (2018)Cite this article

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Hemophagocytic syndrome (HPS), also called as hemophagocytic lymphohistiocytosis (HLH), is a rare but fatal complication after stem cell transplantation (SCT). Appropriate and prompt intervention is necessary because of a significant risk of graft failure [1]. Most of the patients who developed HPS following SCT described in previous case reports were treated with corticosteroids (CS), high-dose intravenous immunoglobulin (IVIG), low-dose etoposide (VP-16), and/or second salvage SCT [1,2,3]. Some cases may not tolerate the salvage SCT because of coexistent complications. To date, prognosis of patients with HPS following SCT described in several previous reports seems considerably poor [1, 4], and optimal treatment strategy remains to be established.

Tumor necrosis factor (TNF)-α inhibitors such as infliximab, etanercept, and adalimumab are commonly used to treat patients with autoimmune disease including rheumatoid arthritis and inflammatory bowel disease. In addition, several recent reports demonstrated the feasibility of anti-TNF therapy for patients with autoimmune disease-associated HLH/macrophage activation syndrome (MAS) [5,6,7], a term used to describe HLH that occurs in patients with systemic juvenile idiopathic arthritis or adult-onset Still’s disease. Here we report a patient with Behcet’s disease who underwent SCT for secondary myelodysplastic syndrome (MDS). He developed HPS early after SCT followed by secondary graft failure, and was treated with adalimumab. To our knowledge, this is the first report describing the use of anti-TNF-α inhibitor for treatment of HPS occurring after SCT.

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Authors and Affiliations

  1. Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan

    Maiko Noguchi & Jiro Inagaki

  2. Division of Pediatrics, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

    Hiroshi Moritake & Sachiyo Kamimura

  3. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan

    Motoshi Sonoda & Masataka Ishimura

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  1. Maiko Noguchi
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Correspondence to Jiro Inagaki.

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Noguchi, M., Moritake, H., Kamimura, S. et al. Adalimumab for treatment of hemophagocytic syndrome following unrelated bone marrow transplantation in a boy with Behcet’s disease and secondary myelodysplastic syndrome. Bone Marrow Transplant 53, 1214–1217 (2018). https://doi.org/10.1038/s41409-018-0180-y

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