Intradural extramedullary relapse of peripheral T-cell lymphomaNOS

摘要

A 73-year-old Japanese man was referred to our clinic with general malaise. He had ahistory of diabetes mellitus and hypertension of longer than 10 years. He presented withlymphadenopathy and liver dysfunction. Laboratory examination demonstrated a WBC count of23.6×10 /L with 10% abnormal lymphocytes having basophilic irregular nuclei,aspartate aminotransferase of 124 U/L (normal range: 5–40 U/L), alanine transaminase of 166U/L (5–35 U/L), total bilirubin of 4.1 mg/dL (1.0–0.3 mg/dL), creatinine of 1.46 mg/dL(0.6–1.1 mg/dL) and soluble interleukin-2 receptor (sIL-2R) of 15,889 U/mL (122–496 U/mL).Serological tests for HBV, HCV and HTLV-1 were all negative. The physical examinationrevealed bilateral cervical lymph node swelling, multiple abdominal skin pigmentation andperipheral edema. On computed tomography (CT) of the trunk, generalized multiplelymphadenopathy with mild splenomegaly was noted. Biopsy specimens of cervical lymph nodesand abdominal skin exhibited monotonous infiltration of medium to large-sized lymphocyteswith a phenotype of CD3+, CD5+, CD10−, CD20−, CD79a−, CD30−, CD56−, Bcl6−, granzyme B−,CD45RO−, CCR4+ and TdT-. ( ) The Ki-67labeling index was 80%. EBER hybridization was negative. Lymphomacells were also detected in the bone marrow. The prognostic index score for T-cell lymphomain this case was 4, considered to be high risk. The final diagnosis was PTCL, NOS, stage IVB. We treated the patientusing modified CHOP therapy. After one cycle of chemotherapy, the swelled lymph node shrunkand partial response was achieved. However, on day 13 after the modified CHOP therapy, hisgeneral condition deteriorated and the WBC increased to 9.6×10 /L with 36%lymphoma cells. The disease progressed and we decided to use the histone deacetylase (HDAC)inhibitor romidepsin (14 mg/m 1×/week for 3 weeks) as second-line therapy. Afterthe first administration of romidepsin, the patient recovered rapidly. His sIL-2R levelsdecreased to 1,428 U/mL. When the WBC count recovered to 7.6×10 /L 17 days later,8% lymphoma cells persisted in the peripheral blood and one cycle of the monoclonal antibodymogamulizumab (1 mg/kg for every 4 weeks) was added. He received a second cycle ofromidepsin, and the disappearance of lymphoma cells from the peripheral blood and all lymphnode swelling was confirmed. On day 7 after the second cycle of romidepsin, the patientsuddenly complained of severe lumbago with bilateral weakness of the lower limbs. InitialMRI of the entire spine detected no abnormalities. CT demonstrated complete remission of thelymphadenopathy. His sIL-2R value was stable at 1,423 U/mL. We consulted neurologistsregarding paraparesis. As they suspected drug-induced neuropathy, we decided to stop theromidepsin treatment. However, muscle weakness progressed and he became fully paralyzed onday 21. Repeated MRI of the head and cervical spine revealed no lesion. Lumbar punctureswere unsuccessful. On day 25, an intradural extramedullary mass was detected onthoracolumbar MRI, suggesting infiltrated lymphoma ( ). His performance status deteriorated due to neurological deficit and palliativespinal cord irradiation did not improve. The patient died due to PTCL at 3 months after theinitial diagnosis.