Skill Checkup: A 62-Year-Old Man With Lumps on His Neck and Under His Arm

Ann S. LaCasce, MD, MMSc


May 25, 2023

Based on the patient's symptoms, lab results, immunophenotype, and FISH results, he has MCL.

MCL is a typically aggressive subtype of B-cell non-Hodgkin lymphoma (NHL) that is not curable with standard immunochemotherapy. It is relatively rare, comprising approximately 5% of all NHLs. MCL is characterized by the expansion of the mantle zone that surrounds the lymph node germinal centers by small-to-medium atypical lymphocytes. B symptoms, such as fever, weight loss, and night sweats, occur in about 40% of patients. Fatigue may result from anemia or high-burden disease. The majority of patients with MCL present with generalized lymphadenopathy. Splenomegaly or, less commonly, hepatomegaly may also be present. Immunophenotyping indicates monoclonal B cells with CD5, CD20, and cyclin D1. It is distinguished from other B-cell lymphomas by diffuse positivity for cyclin D1 and SOX11 in the majority of cases. In addition, t(11;14), which results in overexpression of the cyclin D1 (CCND1) gene, is characteristic of the disease.

Immunophenotyping helps differentiate MCL from other small B-cell lymphomas. Immunophenotyping of CLL indicates the presence of circulating clonal B lymphocytes expressing CD5, CD19, CD20(dim), CD23, and an absence of FMC-7 staining. CLL is a monoclonal disorder characterized by progressive accumulation of functionally incompetent lymphocytes. Bloodwork for CLL will show absolute lymphocytosis, with > 5000 B lymphocytes/µL. Patients can present with a wide range of symptoms, including tiredness and fatigue, fever, recurring infections, night sweats, splenomegaly, hepatomegaly, petechiae, and pallor.

HCL is a chronic lymphoid leukemia, named after the hairlike cytoplasmic projections seen on the surface of the abnormal B cells. It is characterized by the accumulation of hairy cells in the bone marrow, liver, and spleen, with very little lymph node involvement. On flow cytometry, classic HCL is negative for CD5 and CD10, and positive for CD20 (bright), CD11c (bright), CD22 (bright), CD25, CD103, CD123, CD200, cyclin D1, and annexin A1. The most common symptoms and presenting complaints of HCL are weakness and fatigue owing to anemia, and the most common physical finding is splenomegaly, which is massive in > 80% of patients. Lymphadenopathy is found in 10% of patients with HCL, but peripheral lymphadenopathy is uncommon. Patients may have the B symptoms associated with other lymphoproliferative disorders. It can also cause bleeding from thrombopenia and fever and infections from neutropenia. HCL is associated with bacterial, fungal, and opportunistic infections.

FL is a type of NHL that most commonly presents as a painless, slowly progressive adenopathy. Involved lymph nodes typically are nontender, firm, and rubbery in consistency. Systemic B symptoms are infrequent at presentation but can be observed in later stages of the disease. Patients with FL may also have splenomegaly at presentation. The typical immunophenotype of FL is as follows: CD10+, BCL2+, CD23+/-, CD43-, CD5-, CD20+, and BCL6+.


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