Rituximab plus Ifosfamide, Carboplatin and Etoposide for T-Cell/Histiocyte-Rich B-Cell Lymphoma Arising in Nodular Lymphocyte-Predominant Hodgkin's Lymphoma

Abstract

A small subset of patients with nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHLs) develop a non-Hodgkin lymphoma either concurrently or subsequently, usually T-cell/histiocyte-rich B-cell lymphomas (T/HRBCL), which are subtypes of diffuse large B-cell lymphomas (DLBCL). The standard treatment of DLBCL patients is rituximab-based chemotherapy with cyclophosphamide, adriamycin, vincristine and prednisolone. However, the administration of this chemotherapy regimen to patients with DLBCL arising in NLPHL brings concern about the cardiac toxicity of anthracycline because the majority of these patients had already received anthracycline-based chemotherapy with doxorubicin, bleomycin, vinblastine and dacarbazine at the time of NLPHL. Herein, we report 2 patients with sequential transformation of NLPHL to T/HRBCL. They initially presented with limited-stage NLPHL and subsequently developed T/HRBCL after 16 and 8 months, respectively. At the time of T/HRBCL, they were treated with rituximab, ifosfamide, carboplatin and etoposide, and complete responses were obtained.

Keywords: Chemotherapy; Nodular lymphocyte-predominant Hodgkin's lymphoma; T-cell/histiocyte-rich B-cell lymphoma; Transformation.

Fig. 1

Case 1: NLPHL. HE staining showed vague nodules with large atypical lymphocytic and histiocytic cells in a background of reactive small lymphocytes (a). Immunohistochemical staining showed networks of follicular CD21+ dendritic cells in the reactive cells (b) and rosettes of CD57+ T lymphocytes (c).

Fig. 2

Case 1: T/HRBCL. HE staining showed a diffuse pattern with a polymorphous background rich in lymphocytes and histiocytes with intermingled isolated blast cells (a). Immunohistochemical analysis showed that blastic cells were CD3 negative (b) and CD20 positive (c), which was consistent with a B-lymphocyte phenotype. Most of the small non-neoplastic lymphocytes were CD3 positive and CD20 negative, consistent with T lymphocytes. Networks of follicular CD21 dendritic cells were absent (d).

Fig. 3

Case 2: NLPHL. HE staining showed vague nodules with large atypical lymphocytic and histiocytic cells on a background of reactive small lymphocytes (a). Immunohistochemical staining showed networks of follicular CD21+ dendritic cells in the reactive cells (b) and rosettes of CD57+ T lymphocytes (c).

Fig. 4

Case 2: T/HRBCL. HE staining showed a diffuse pattern with a polymorphous background rich in lymphocytes and histiocytes with intermingled isolated blast cells (a). The immunohistochemical analysis showed that blastic cells were CD3 negative (b) and CD20 positive (c), which was consistent with a B-lymphocyte phenotype. Most of the small non-neoplastic lymphocytes were CD3 positive and CD 20 negative, consistent with T lymphocytes. Networks of CD21 follicular dendritic cells were weak (d).