Isolated central nervous system relapse in type II enteropathy-associated T cell lymphoma

Hiroko Nishida, Kiyoshi Endo, Mami Hatano, Nobuo Tatekawa, Eri Mashima, Risa Hashida, Masao Hori, Katsuyuki Obara

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)692-698
Number of pages7
Journal[Rinshō ketsueki] The Japanese journal of clinical hematology
Volume56
Issue number6
DOIs
Publication statusPublished - 2015 Jun 1

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Enteropathy-Associated T-Cell Lymphoma
Descending Colon
Granzymes
Central Nervous System
CD8-Positive T-Lymphocytes
Biopsy
Recurrence
Drug Therapy
Investigational Therapies
Gadolinium
Brain
Jejunum
Rare Diseases
Methotrexate
Hypertrophy
Disease Progression
Dilatation
Lymphoma
Lymphocytes
Neoplasms

ASJC Scopus subject areas

  • Medicine(all)

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Isolated central nervous system relapse in type II enteropathy-associated T cell lymphoma. / Nishida, Hiroko; Endo, Kiyoshi; Hatano, Mami; Tatekawa, Nobuo; Mashima, Eri; Hashida, Risa; Hori, Masao; Obara, Katsuyuki.

In: [Rinshō ketsueki] The Japanese journal of clinical hematology, Vol. 56, No. 6, 01.06.2015, p. 692-698.

Research output: Contribution to journalArticle

Nishida, H, Endo, K, Hatano, M, Tatekawa, N, Mashima, E, Hashida, R, Hori, M & Obara, K 2015, 'Isolated central nervous system relapse in type II enteropathy-associated T cell lymphoma', [Rinshō ketsueki] The Japanese journal of clinical hematology, vol. 56, no. 6, pp. 692-698. https://doi.org/10.11406/rinketsu.56.692
Nishida, Hiroko ; Endo, Kiyoshi ; Hatano, Mami ; Tatekawa, Nobuo ; Mashima, Eri ; Hashida, Risa ; Hori, Masao ; Obara, Katsuyuki. / Isolated central nervous system relapse in type II enteropathy-associated T cell lymphoma. In: [Rinshō ketsueki] The Japanese journal of clinical hematology. 2015 ; Vol. 56, No. 6. pp. 692-698.
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