title: [Isolated central nervous system relapse in type II enteropathy-associated T cell lymphoma].
abstract: A 75-year-old male presented with progressive lower abdominal discomfort. CT scan demonstrated hypertrophy of the intestinal wall, small bowel dilatation, and masses in the descending colon. Biopsy specimens of the jejunum and descending colon revealed widespread distribution of medium-sized atypical lymphocytes with an immunophenotype, positivity for CD3, CD8, CD56, TAI-1, granzyme B and TCRβ, but negativity for CD4, CD5, CD20, CD30 and EBER-ISH. Type II enteropathy-associated T cell lymphoma (EATL; Lugano, stage IIE) was diagnosed. Subsequently, he received 6 cycles of chemotherapy with 2/3 dose CHOP and obtained complete remission. However, 18 months after the initial presentation, he presented with rapidly progressive mental deterioration. Gadolinium enhanced T1-weighted brain MR images showed multiple masses with mild heterogeneous enhancement. Brain biopsy revealed necrotic tumors composed of medium-sized atypical lymphocytes, positive for CD3, CD8, CD56, TIA-1, granzyme B and TCRβ, but negative for CD4, CD20, and EBER-ISH. CT scan disclosed no evidence of systemic lymphoma relapse, indicating central nervous system relapse of EATL. Despite immediate high-dose chemotherapy with methotrexate, he died of disease progression. EATL is a rare disease with a very poor outcome, for which a validated standard treatment is still lacking. Further studies are needed to identify innovative therapies for treating EATL.
authors.affiliation: Department of Pathology, Keio University, School of Medicine.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Nishida
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Endo
authors.forename: Mami
authors.initials: M
authors.lastname: Hatano
authors.forename: Nobuo
authors.initials: N
authors.lastname: Tatekawa
authors.forename: Eri
authors.initials: E
authors.lastname: Mashima
authors.forename: Risa
authors.initials: R
authors.lastname: Hashida
authors.forename: Masao
authors.initials: M
authors.lastname: Hori
authors.forename: Katsuyuki
authors.initials: K
authors.lastname: Obara
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000188:drug therapy / Q000556:secondary*; D003131:Combined Modality Therapy; D017809:Fatal Outcome; D006801:Humans; D007410:Intestinal Diseases / Q000209:etiology* / Q000601:surgery; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000628:therapy; D008297:Male; D012008:Recurrence; D014057:Tomography, X-Ray Computed
chemical_list: D000970:Antineoplastic Agents