Isolated neurocutaneous peripheral T cell lymphoma, NOS

Talal Hilal

doi:10.5348/ijcri-2014149-cr-10460

Abstract

Peripheral T cell lymphomas (PTCL) are a heterogeneous group of diseases that are a relatively uncommon subtype of non-Hodgkins lymphoma (NHL) with an overall poor prognosis. They are difficult to classify and targeted therapy does not exist. Patients are usually treated with B cell specific drugs that have not shown to be effective in most subtypes. A 52-year-old male presented with a one-week history of confusion. He was found to have diffuse subcutaneous lumps on his extremities, back, and thorax, and a large swelling over his left face. Imaging revealed a right frontal lobe mass. Biopsy of the mass confirmed PTCL. Further histopathological analysis and imaging for staging diagnosed the subtype as not otherwise specified (NOS). The patient received radiation therapy to the brain followed by systemic chemotherapy with cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP). He responded by the end of the first cycle, but his long-term clinical course remains to be seen. Conclusion: Treatment for PTCL is a realm requiring further research with efforts focusing on the development of T cell specific drugs.

Highlights

Peripheral T cell lymphomas (PTCL) are a heterogeneous group of diseases that are a relatively uncommon subtype of non-Hodgkin’s lymphoma (NHL) with an overall poor prognosis
Patients are usually treated with B cell specific drugs that have not shown to be effective in most subtypes
PTCLs are a group of lymphomas that fall under the category of mature T cell lymphomas, and are an uncommon subtype of non-Hodgkin’s lymphoma (NHL)

Summary

INTRODUCTION

Peripheral T cell lymphomas (PTCLs) derive from post-thymic T cells and generally arise in lymphoid tissue “peripheral” to the thymus such lymph nodes, spleen, gastrointestinal tract and skin They have a mature T cell phenotype and are grouped, along with mature natural killer lymphomas, according to clinical presentation as leukemic, nodal or extranodal [1]. Inspection of his oral cavity revealed a necrotic growth protruding from his soft palate He had multiple diffuse violaceous lesions on his skin, some of which were ulcerated with central necrosis (Figure 1). Initial assessment focused on histologic diagnosis and a biopsy of the soft palate growth and one of the skin lesions, both of which revealed features consistent with PTCL (Figure 3). The patient responded within a few days and all his nodules disappeared by the end of the first of four cycles of therapy

Findings

DISCUSSION

CONCLUSION