Pulmonary lymphocyte-rich classical Hodgkin lymphoma with early response to ABVD therapy

Abstract

Dear Editor, Pulmonary Hodgkin lymphoma is a rare subgroup of Hodgkin lymphoma which primarily affects the lung with minimal extrapulmonary lesions [1]. The disease manifests as multiple nodules or, less frequently, as a solitary mass in the pulmonary parenchyma [2]. The histological spectrum of pulmonary Hodgkin lymphoma resembles that of nodular Hodgkin lymphoma; nodular sclerosis and mixed cellularity account for common subtypes, while lymphocyte-rich classical Hodgkin lymphoma (LRCHL) is much less frequent. A meta-analysis of 61 cases with pulmonary Hodgkin lymphoma identified only one case (2%) of LRCHL [1]. Therefore, LRCHL with primary pulmonary lesions is extremely rare. We herein describe another case of pulmonary LRCHL and discuss the optimal treatment strategy which has not yet been determined. A 35-year-old female presented with a 2-month history of progressive nonproductive cough and intermittent fever. Physical examination detected mild enlargement of a left supraclavicular node. Laboratory test results included hemoglobin level of 110 g/L and leukocyte count of 10.4× 10 /L. Chest radiograph was remarkable for multiple parenchymal nodules in both lungs (Fig. 1a). Transbronchial biopsy of a nodule did not reach a diagnosis although microscopic examination showed dominant infiltration of lymphocytes. Open biopsy of a supraclavicular node was also performed, and histopathology was notable for a vague nodular lesion with scattered binuclear large cells in the background of smallto medium-sized lymphocytes without neutrophils or eosinophils (Fig. 1b). Immunophenotype of the large cells was CD30+, CD15+ (partial), and CD20−, consistent with Reed–Sternberg (RS) cells. She was then referred to us for further scrutiny and treatment. F-18 fluorodeoxyglucose positron emission tomography (FDGPET) combined with computed tomography (CT) scan revealed uptakes in multiple pulmonary nodules, mediastinum, supraclavicular lymph nodes, hepatic hilar lymph nodes, and splenic nodules (Fig. 1c, d). There was no evidence of granulomatosis, sarcoidosis, carcinoma, or infection. Taken together, the patient was diagnosed as pulmonary LRCHL with nodal and splenic involvement. Treatment was initiated with six cycles of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) regimen. Interim F-18 FDG-PET/CT scan after two cycles disclosed salient regression of parenchymal nodules and complete resolution of the extrapulmonary lesions (Fig. 1e, f). The patient has so far been relapse-free for 14 months. In the pre-ABVD era, pulmonary Hodgkin lymphoma was associated with relatively dismal outcomes. In one study, mechlorethamine, vincristine, procarbazine, and prednisolone (MOPP) therapy resulted in a relapse rate of 56% (five of nine cases) [2]. This series included one case of relapsed pulmonary LRCHL. These results are partly explained by the inability to use radiotherapy for pulmonary parenchymal lesions. Our case was featured by early response of pulmonary lesions to ABVD therapy. This regimen was superior to MOPP therapy as first-line treatment A. Honda : F. Nakamura :Y. Nannya :M. Ichikawa : M. Kurokawa (*) Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan e-mail: kurokawa-tky@umin.ac.jp