Primary CNS post-transplant lymphoproliferative discover following haplo-identical HSCT using post-transplant high-dose cyclophosphamide

Authors: Masako Toyosaki, Makoto Onizuka, Jun Amaki, Sawako Shiraiwa, Yasuyuki Aoyama, Shinichiro Machida, Hidetsugu Kawai, Hiromichi Murayama, Daisuke Ogiya, Keiko Matsui, Shouhei Kawakami, Kaito Harada, Yusuke Kondo, Kenichi Hirabayashi, Naoya Nakamura, Yoshiaki Ogawa, Hiroshi Kawada, Kiyoshi Ando

first published: October 26, 2018

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Vol.2 Issue 1 No.1 2019 / BCT-2018-004


Neurological complications after hematopoietic stem cell transplantation (HSCT) are frequently life-threatening, and their clinical management can be highly challenging. In the case of central nervous system lesions post-HSCT, a definitive diagnosis is often difficult to reach because many different causative and contributing conditions may be present, including bacterial, fungal, or viral infections; original disease relapse; and post-transplant lymphoproliferative disorder (PTLD). Here, we report a case of a 32-year-old male patient with Philadelphia chromosome-positive acute lymphoid leukemia who underwent three HSCTs and was then diagnosed with primary central nervous system (PCNS) PTLD by brain biopsy. The third HSCT was a haplo-identical peripheral blood stem cell transplantation from his mother, with post-transplant high-dose cyclophosphamide and tacrolimus used as graft-versus-host disease prophylaxis. Four months after the HSCT, multiple small ring lesions were detected in the para-basal ganglia of the patient’s brain during magnetic resonance imaging. A lesion biopsy indicated Epstein-Barr virus (EBV)-positive, diffuse large B-cell lymphoma. Because the patient had no evidence of systemic lymphadenopathy, we diagnosed him with PCNS-PTLD. There was no EBV DNA in this patient’s cerebrospinal fluid. The diagnosis of PCNS-PTLD by EBV DNA polymerase chain reaction is difficult and highlights the importance of a brain biopsy to diagnose PCNS-PTLD, especially in cases showing no EBV DNA in the cerebrospinal fluid. Although a rare condition, it is essential to locate and analyze cases of PCNS-PTLD after HSCT to establish the optimal strategy for treatment or prophylaxis.

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Masako Toyosaki1, Makoto Onizuka1, Jun Amaki1, Sawako Shiraiwa1, Yasuyuki Aoyama1, Shinichiro Machida1, Hidetsugu Kawai1, Hiromichi Murayama1, Daisuke Ogiya1, Keiko Matsui1, Shouhei Kawakami1 Kaito Harada1, Yusuke Kondo2, Kenichi Hirabayashi2, Naoya Nakamura2, Yoshiaki Ogawa1, Hiroshi Kawada1, Kiyoshi Ando1

1: Division of Hematology and Oncology, Department of Medicine, Tokai University school of Medicine, Kanagawa, Japan

2: Department of Pathology, Tokai University school of Medicine, Kanagawa, Japan


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