Cd5 negative diffuse mantle cell lymphoma with splenomegaly and bone marrow involvement

• Published in: Southern medical journal (Birmingham, Ala.) Vol. 91; no. 6; pp. 584 - 587
• Main Authors: BELL, N. D, KING, J. C, KUSYK, C, NELSON, B. P, SENDELBACH, K. M
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 01-06-1998
• Subjects: Aged; Biological and medical sciences; Biopsy; Bone Marrow - pathology; Bone Marrow Neoplasms - genetics; Bone Marrow Neoplasms - pathology; CD5 Antigens - analysis; Chromosomes, Human, Pair 11; Chromosomes, Human, Pair 14; Hematologic and hematopoietic diseases; Humans; Immunophenotyping; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymph Nodes - pathology; Lymphoma, Non-Hodgkin - genetics; Lymphoma, Non-Hodgkin - pathology; Male; Medical sciences; Polymerase Chain Reaction; Spleen - pathology; Splenic Neoplasms - genetics; Splenic Neoplasms - pathology; Splenomegaly - pathology; Translocation, Genetic - genetics; Human; Splenomegaly; Splenic disease; Instrumentation therapy; Malignant hemopathy; B-Lymphocyte; Replacement; Non Hodgkin lymphoma; Case study; Association; Biopsy; Lymphoproliferative syndrome; Mantle cell lymphoma; Bone marrow; Digestive diseases; Diagnosis; Elderly
• ISSN: 0038-4348; 1541-8243
• DOI: 10.1097/00007611-199806000-00016

We report the case of a 78-year-old man in whom routine physical examination revealed cervical adenopathy and splenomegaly. Peripheral blood showed a normal white blood cell count with an absolute lymphocytosis, which included a population with slightly indented nuclei. Lymph node biopsy showed morphology compatible with mantle cell lymphoma. Bone marrow biopsy showed replacement by a lymphoid proliferation composed of lymphocytes with features similar to those found in the peripheral blood. Immunophenotypic analysis of both peripheral blood and lymph node showed positivity for CD19, CD20 and CD22, with lambda light chain restriction. Tests for CD5 and CD10 were negative. Cytogenetic analysis and polymerase chain reaction studies confirmed the presence of t(11,14) supporting a diagnosis of mantle cell lymphoma. This unusual case of CD5-negative mantle cell lymphoma exemplifies the importance of combined molecular, cytogenetic, and morphologic evaluation when confronted with a lymphoma having an atypical phenotype.