Abstract
T-cell lineage lymphoma with an intense membranous and paranuclear CD30 expression in the absence of ALK1 raises a differential diagnosis of peripheral T-cell lymphoma (PTCL), NOS and anaplastic large cell lymphoma (ALCL), ALK negative. However, Epstein-Barr virus is consistently negative in ALCL and is not considered an implicating factor in its pathogenesis. We describe a case of T-cell lymphoma showing anaplastic large cell morphology with scattered hallmark cells and a uniform CD30 and Epstein-Barr virus encoded early RNA (EBER) expression that primarily involved the subcutaneous tissue at presentation. On incisional biopsy, the neoplastic cells were positive for CD3, CD2, and CD30 while negative for LCA, CD20, PAX5, CD56, ALK1, and cytotoxic granules. Molecular analysis identified a positive T-cell receptor (beta and gamma) gene rearrangement by PCR. Proliferation index approached 100% and the patient had a rapidly progressive course; the subcutaneous lesions more than doubled in size within couple of weeks with new evidence for widespread systemic involvement. This case emphasizes a rare EBV association with a CD30 positive T-cell lymphoma where the morphologic and immunophenotypic findings are otherwise nondiscriminatory between PTCL, NOS and ALCL, ALK negative.
Highlights
Mature T-cell lymphomas are diverse group of aggressive neoplasms with immunophenotype that varies greatly from case to case
World Health Organization (WHO) states that anaplastic large cell lymphoma (ALCL) is “consistently negative for Epstein-Barr virus” and studies have postulated that there is no role of EBV infection in ALCL [4]
The earlier belief, that ALCL, ALK negative does not seem to be distinctive at the immunophenotypic or molecular level and the prognosis is similar to that of patients with peripheral T-cell lymphoma (PTCL), NOS, has been challenged by clinical and gene expression profiling data [6]. These studies support their existence as two separate disease entities; the border between ALCL, ALK negative and PTCL, NOS is a matter of conjecture and imprecise
Summary
Mature T-cell lymphomas are diverse group of aggressive neoplasms with immunophenotype that varies greatly from case to case. A subset of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) displays large-cell morphology with substantial CD30 expression, rendering a precise distinction from ALCL, ALK negative problematic. The lymphoma had a proliferation index approaching 100% with rapid progression to systemic involvement and more than doubling in size of subcutaneous nodules within couple of weeks from diagnosis. This case emphasizes a peculiar CD30 positive immunophenotype with uniform Epstein-Barr encoded early RNA (EBER) expression in a subcutaneous T-cell lymphoma where the clinical presentation, morphology, and immunophenotype present a diagnostic dilemma between ALCL, ALK negative and PTCL, NOS
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