Primary Cutaneous T-Cell-Rich B-Cell Lymphoma and Hodgkin’s Disease in a Patient with Gardner’s Syndrome

Abstract

A 50-year-old patient, suffering from familial polyposis (Gardner’s syndrome), initially presented with several nodules on his left arm. Histological examination revealed primary cutaneous T-cell-rich B-cell lymphoma (TCRBCL). Staging procedures failed to detect any systemic involvement. Three years after total excision of the tumours, the patient presented with a non-specific dermatitis, enlarged axillary lymph nodes and splenomegaly. Histological and immunohistochemical examination of lymph node and spleen biopsy specimens resulted in the diagnosis of Hodgkin’s disease (HD) of the nodular sclerosis type. Sequence analysis of single cells micromanipulated from skin and from lymph node lesions indicated that both lymphoma infiltrates were derived from the same precursor germinal centre B-cell clone. This is a case showing a clonal relationship between TCRBCL and HD, providing support to the B-cell origin of Hodgkin and Reed-Sternberg cells.

Cutaneous T-Cell/Histiocyte-Rich B-Cell Lymphoma: A Case Report and Review of the Literature

“…Another possible explanation is hypothesized on the basis of tumor progression from lymphocyte-predominant Hodgkin’s disease, sharing common morphological features and supporting the recent evidence of hematopoietic plasticity in a single case of an acute B-lymphoblastic leukemia and Langerhans’ cell sarcoma [2,23]. Usually, lymphocyte-predominant Hodgkin’s lymphoma can also be differentiated by the lack of nodularity of the infiltrate, the absence of typical lymphohistiocytic cells, the large number of small B lymphocytes composing the nodules, the absence of CD57+ cells in the reactive infiltrate and the coexpression of CD15 and EMA in the blasts [2,11,17]. Differences between THRLBCL and lymphomatoid granulomatosis [24], an angiocentric variant of T-cell and/or histiocyte-rich B-cell monoclonal proliferation, are summarized in table 2.…”

WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects

“…14), CD22, and CD79a, with light‐chain restriction, but are negative for CD15 and CD30, which excludes Hodgkin lymphoma. The reactive small‐cell population represents T‐helper cells but small reactive B cells can also be found 190 . Due to the lower number of tumor cells, detection of IgH gene rearrangements may be difficult to assess in some cases.…”