Pure intravascular recurrence of CD5-positive diffuse large B-cell lymphoma primarily arising from the nasal cavities

• Published in: Diagnostic pathology 2018-07, Vol.13 (1), p.46-46, Article 46
• Main Authors: Kano, Rieko, Masaie, Hiroaki, Hino, Akihisa, Yasuoka, Hironao, Nagata, Shigenori, Ishikawa, Jun, Nakatsuka, Shin-Ichi
• Format: Article
• Language: English
• Subjects: Aged; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Asian variant; B-cell lymphoma; Biomarkers, Tumor - analysis; Biopsy; Blood; Blood diseases; Bone marrow; Case Report; CD20 antigen; CD3 antigen; CD5 antigen; CD5 Antigens - analysis; CD5-positive diffuse large B-cell lymphoma; Chemotherapy; Computed tomography; Cyclophosphamide; Fluorodeoxyglucose F18 - administration & dosage; Histology; Humans; Immunohistochemistry; Infiltration; Intravascular large B-cell lymphoma; Laboratories; Leukemia; Lymph nodes; Lymphatic system; Lymphocytes B; Lymphoid cells; Lymphoma; Lymphoma, Large B-Cell, Diffuse - diagnostic imaging; Lymphoma, Large B-Cell, Diffuse - drug therapy; Lymphoma, Large B-Cell, Diffuse - immunology; Lymphoma, Large B-Cell, Diffuse - pathology; Male; Medical prognosis; Metastases; Monoclonal antibodies; Nasal Cavity - diagnostic imaging; Nasal Cavity - immunology; Nasal Cavity - pathology; Nose; Nose Neoplasms - diagnostic imaging; Nose Neoplasms - drug therapy; Nose Neoplasms - immunology; Nose Neoplasms - pathology; Positron emission; Positron emission tomography; Positron Emission Tomography Computed Tomography; Prednisolone; Radiopharmaceuticals - administration & dosage; Random skin biopsy; Recurrence; Remission; Rituximab; Salvage; Sinuses; Skin; Targeted cancer therapy; Tomography; Treatment Outcome; Tumors; Vascular Neoplasms - immunology; Vascular Neoplasms - pathology; Vincristine
• ISSN: 1746-1596; 1746-1596
• DOI: 10.1186/s13000-018-0724-x

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. 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 F-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. 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.