Annals of Hematology | 2021
Indolent T cell lymphoma involving the central nervous system and successfully treated by bendamustine
Abstract
Peripheral T-cell lymphoma, not otherwise unspecified (PTCL-NOS), was most common among the peripheral T-cell lymphomas (PTCL) subtypes. The central nervous system (CNS) is rarely involved as the primary site, making it especially challenging for diagnosis and treatment. The present report describes a rare case of indolent PTCL involving the central nervous system, bone marrow, subcutaneous tissue, and muscles, which was successfully treated by the bifunctional alkylating agent bendamustine and allogeneic hematopoietic stem cell transplantation. In July 2020, a 20-year-old male presented to the Department of Hematology. He was originally diagnosed with hemophagocytic syndrome in 2013. Etoposide, dexamethasone, and cyclosporin A were hence given according to the HLH-2004 protocol, after which no fever, diminish splenomegaly, and partial recovery of blood count were observed. In September 2016, he developed ptosis of the left eyelid and bilateral temporal depression, which deteriorated further, with eyelid ptosis progression to bilateral and emergence of neurological anomaly, including distortions of commissure, masticatory atonia, involuntary shaking, numbness, and weakness of limbs since April 2020. On physical examination, he had diplopia, left eye movement limitation, and air leakage when the cheek bulges and was unable to show teeth. His left upper limb involuntary jittered and the posture of upper limbs and shoulders was strange during walking. He cannot complete both hands alternating movement test, heel-knee-tibia test, finger-nose test, and straight walking. Bone marrow smear revealed remarkably increased lymphoid cells up to 52.5%. Bone marrow (BM) biopsy discovered small patchy lymphocyte infiltrations (Fig. 1a) which were positive for markers CD2, CD3, and CD99, but negative for TdT, CD56, CD20, and PAX5, with a Ki-67 index of 3% (Fig. 1b). Flow cytometry showed 95% T lymphocytes expressing CD2, CD3, and TCR αβ, but negative for TCRγδ, CD57, CD56, and CD16 (Fig. 1c). TCR gene rearrangement proved that TCRβ was positive. PET/CT showed multiple abnormal nodular high intake of fluorodeoxyglucose in the subcutaneous, intramuscular, and retroperitoneal space (SUV 5.5–9.7) (Fig. 1d). Lumbar puncture was performed and cerebrospinal fluid (CSF) analysis was normal. Enhanced MRI in May 2020 observed multiple flaked anomalous reinforced signals in bilateral basal ganglia, thalamus, bilateral frontal and temporal lobes, right parietal occipital lobe, and cerebellum, while swollen and abnormal signals were presented in cranium muscles suggesting inflammations (Fig. 1e). A diagnosis of peripheral T cell lymphoma, not otherwise specified (PTCL-NOS), was made, which involved the bone marrow, central nervous system, subcutaneous tissue, and muscles. PTCL-NOS tends to have an aggressive course, and most patients present with advanced-stage disease and extremely poor prognosis [1]. But occult onset, 7-year disease course, and small T lymphocyte infiltrations in bone marrow with a Ki-67 index of 3% prompted an indolent T cell lymphoma/leukemia in this patient. He was then treated with four cycles of bendamustine (Le Weixin; Chia Tai Tianqing Pharmaceuticals; 90 mg/m2/day IV for two consecutive days on a 4-week schedule), after which a complete remission was observed in brain MRI (Fig. 1f). Bone marrow biopsy exhibited only slightly scattered lymphocytes without apparent aggregation or heteromorphism. There were no complications during chemotherapy. He received * Miao Chen [email protected]