Post-transplant Hemophagocytic Lymphohistiocytosis in Benign Hematological Disorders: Experience of 4 Cases with Review
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ORIGINAL ARTICLE
Post-transplant Hemophagocytic Lymphohistiocytosis in Benign Hematological Disorders: Experience of 4 Cases with Review of Literature Akanksha Garg1 • Sandip Shah1 • Kinnari Patel1 • Kamlesh Shah1 Asha Anand1 • Harsha Panchal1 • Apurva Patel1 • Sonia Parikh1
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Received: 21 October 2019 / Accepted: 28 January 2020 Ó Indian Society of Hematology and Blood Transfusion 2020
Abstract Post transplant Hemophagocytic lymphohistiocytosis (HLH) is a form of secondary HLH, which can be either early onset or late onset and is associated with significant morbidity and mortality. With the increasing popularity of post transplant cyclophosphamide based haploidentical stem cell transplantation (SCT), post transplant HLH is becoming a significant complication especially in benign hematological disorders. Methods: We present 4 cases of post transplant HLH occurring in 2 cases of severe aplastic anemia (post haploidentical SCT) and 2 cases of thalassemia major (post matched sibling SCT). All 4 cases had early onset variety with dismal prognosis. Conclusion: Post-transplant HLH is an important entity in benign hematological disorders, which needs to be identified early and treated promptly with steroids, monoclonal agents or immunosuppressive therapy. Serum ferritin levels are an important biomarker and help in monitoring response. Keywords Post transplant HLH Allogenic stem cell transplant Haploidentical stem cell transplant Benign hematological disorders
Introduction Hemophagocytic lymphohistiocytosis (HLH) is a disorder characterized by fever, splenomegaly, cytopenias and hyperferritinemia and occurs due to uncontrolled activation & Akanksha Garg [email protected] 1
Department of Medical Oncology, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat 380016, India
of cytotoxic T cells causing excessive immune activation [1]. It can be of two forms-primary and secondary HLH. Post transplant HLH is a form of secondary HLH that can be either early-onset (\ 30 days post transplant) or lateonset (C 30 days post transplant) [2]. The incidence of post-transplant HLH in previous case series has been estimated from 0.3 to 16.8% [3]. It is seen commonly post transplant in both benign and malignant conditions. Here, we report a series of 4 cases of HLH occurring post allogenic stem cell transplantation (SCT) for benign disorders; 2 cases of thalassemia major (following matched sibling SCT) and two cases of aplastic anemia (following haploidentical SCT).
Case 1 A 32-year old male was diagnosed with severe aplastic anemia (SAA) in 2017. There were no matched sibling donors available; hence, he underwent antithymocyte globulin (ATG) therapy. He achieved partial response and was subsequently started on tacrolimus in view of cyclosporine toxicity. He was transfusion dependent, hence was planned for haploidentical SCT from sister. He received fludarabine-cyclophosphamide-ATG (Flu-Cy-ATG) post Cy conditioning regimen. Patient engrafted on Day ? 16, and was managed for febrile neutropenia according to unit protocol.
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