Enteropathy associated T cell lymphoma presenting as an isolated CNS lymphoma three years after diagnosis of coeliac disease: T cell receptor polymerase chain reaction studies failed to show the original enteropathy to be a clonal disorder.

Abstract

A case of enteropathy associated T cell lymphoma is reported in a 45 year old woman, presenting with isolated disease in the CNS, three years after diagnosis of coeliac disease. Initial staging showed no evidence of gastrointestinal tract lymphoma. A presumptive diagnosis of T cell primary cerebral lymphoma was made and the patient was treated with combination chemotherapy and craniospinal radiotherapy. The patient relapsed, seven months after treatment, with small bowel lymphoma. The inmunophenotype and T cell receptor polymerase chain reaction analysis confirmed the same tumour as in the CNS. Retrospective polymerase chain reaction analysis of intraepithelial lymphocytes in the duodenal biopsy sample, taken at the time of diagnosis of coeliac disease, failed to show evidence of a clonal T cell proliferation. (Gut 1997; 40: 801-803) Enteropathy associated T cell lymphoma usually presents as extranodal intestinal disease. Nodal involvement with associated lesions in the lung, thyroid, skin, and Waldeyer's ring have been described. Isolated lymphoma has also been reported in the lung, thyroid, skin, and Waldeyer's ring alone.5'6We report a case of coeliac associated T cell lymphoma, presenting as isolated CNS disease mimicking primary CNS lymphoma, and the presumed course of the lymphoma based on gene rearrangement studies.Case report A 45 year old woman presented in 1992 with intermittent diarrhoea, abdominal discomfort, skin rash, and a haematological picture consistent with malabsorption. A distal duodenal biopsy specimen showed subvillous atrophy and intraepithelial lymphocytosis consistent with coeliac disease. A gluten free diet was started with rapid resolution of the symptoms. Duodenal biopsy was not repeated. She remained on a gluten free diet.In 1995 she developed lethargy, confusion, short term memory impairment, and bitemporal headache. Brain computed tomography (CT) scan showed a low attenuation mass adjacent to and compressing the anterior horn of the right lateral ventricle, with considerable surrounding oedema and enhancement with intravenous contrast (Figure). The appearances were suggestive of primary cerebral lymphoma. A stereotactic biopsy specimen showed a necrotic tumour composed of pleomorphic lymphoid cells with immunophenotype CD45+ CD45RO+ CD3+ CD30+ CD20-EMA-. Polymerase chain reaction analysis for T cell receptor and immunoglobulin heavy chains was performed using three primer pairs.7 This demonstrated a clonal rearrangement of T cell receptor ,B and y chain genes of 80 bp and 70 bp respectively. The histological diagnosis was of a T cell variant of large cell anaplastic lymphoma.Staging investigations, which included full blood count, erythrocyte sedimentation rate, lactate dehydrogenase, bone marrow aspirate, and

Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation

“…We added intermediate-dose MTX to the protocol as prophylaxis against previously described relapse/progression of disease in the central nervous system. 24,25 Treatment starts with 1 cycle of CHOP to establish initial disease control; to minimize the risk of side effects such as perforation, anastomotic breakdown, or wound dehiscence (in the postsurgery patient); and to allow a limited recovery period before the more intensive part of the regimen begins.…”

Enteropathy-Associated T-Cell Lymphoma: Epidemiology, Clinical Features, and Current Treatment Strategies

“…The SNLG reported 6% of patients with bone marrow infiltration [25••]. One particular extranodal site of involvement in EATL worth special mention is the central nervous system, which is rarely involved at presentation [28] but may be the only site of relapse, particularly late relapse [29]. Treatment outcomes in this situation are very poor.…”

Supratentorial metastatic enteropathy-associated T-cell lymphoma: A case report and literature review

“…[378] Recent research findings based on gene analysis studies have suggested that there may be two distinct subtypes of EATL. Type I accounts for 80 to 90 percent of cases, and is strongly associated with celiac disease.…”

“…The neoplastic lymphocytes stain positively for CD45 (leukocyte common antigen), CD3, CD30, and TIA-1 (cytotoxic granule marker) and Ki-67. [2468] It is important to highlight that CD30 positivity may vary, and it is more frequently expressed in large T-cell lymphomas, rather than small-to-medium-sized lesions. [5] Serum anti-gliadin antibodies are normally positive.…”

“…[2–46–8] However, physicians must be aware of the possibility that neurological symptoms may be masqueraded or delayed by the concomitant use of steroids, which are frequently used for the management of celiac and inflammatory bowel disease.…”