Pure intravascular recurrence of CD5-positive diffuse large B-cell lymphoma primarily arising from the nasal cavities
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CASE REPORT
Open Access
Pure intravascular recurrence of CD5positive diffuse large B-cell lymphoma primarily arising from the nasal cavities Rieko Kano1* , Hiroaki Masaie2, Akihisa Hino2, Hironao Yasuoka3, Shigenori Nagata1, Jun Ishikawa2 and Shin-ichi Nakatsuka1
Abstract Background: CD5-positive diffuse large B-cell lymphoma (DLBCL) and intravascular large B-cell lymphoma (IVL) are recognized as rare subsets of large B-cell lymphoma with poor prognosis. These two categories have similar clinicopathological features suggesting that they might overlap. Case presentation: We present a case of a 72-year-old man with submental tumors. Positron emission tomography/computed tomography (PET/CT) showed tumors in the nasal and paranasal region and multiple submental and jugular swollen lymph nodes with abnormal uptake of 18F-fluorodeoxyglucose (FDG). Histology of biopsy from nasal tumors showed diffuse infiltration of large lymphoid cells, which showed positive expressions for CD20, CD79a, CD5 and negative for CD3 on immunohistochemistry. Thus, a CD5-positive DLBCL was diagnosed. After administration of 8 cycles of R-THPCOP (rituximab, pirarubicin, cyclophosphamide, vincristine and prednisolone), complete remission was achieved. Eight months after the first chemotherapy dose, local recurrence occurred. After salvage chemotherapy, nasal and paranasal tumors and lymph node swelling disappeared on PET/ CT images, although the patient suffered from respiratory disturbance. A random skin biopsy revealed IVL, which was consistent with intravascular recurrence of CD5-positive DLBCL. Bone marrow smears showed hemophagocytosis. Conclusion: We present a rare case of primary CD5-positive DLBCL that relapsed as pure IVL after chemotherapy. Our case suggests that CD5-positive DLBCL is closely related to IVL. Keywords: CD5-positive diffuse large B-cell lymphoma, Intravascular large B-cell lymphoma, Asian variant, Random skin biopsy
Background Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of B-cell lymphoma with heterogeneous biological and clinical features. In a small subset of DLBCL cases, CD5, a pan-T-cell surface marker, is expressed. CD5 is also expressed in most cases of chronic lymphocytic leukemia (CLL) and mantle cell lymphoma. CD5-positive DLBCL is divided into de novo and secondary groups [1]. De novo CD5-positive DLBCL is associated with high-risk clinical features, performance status > 1, bone marrow * Correspondence: [email protected] 1 Department of Diagnostic Pathology and Cytology, Osaka International Cancer Institute Hospital, 3-1-69 Otemae, Chuo-ku, Osaka 541-8567, Japan Full list of author information is available at the end of the article
involvement, and central nervous system (CNS) recurrence [2–5]. The majority of CD5-positive DLBCL cases show an activated B-cell subtype phenotype on genetic profiling [2, 6] and non-germinal center B-cell phenotype on immunohistochemistry [4, 7]. De novo CD5-positive DLBCL frequently shows partial intravascular or intrasinusoidal infiltration [5], wh
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