Clinical manifestations, pathologic features, and diagnosis of small lymphocytic lymphoma
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jon C Aster, MD
Jon C Aster, MD
- Professor of Pathology
- Harvard Medical School
Small lymphocytic lymphoma (SLL) is a mature (peripheral) B cell neoplasm characterized by a progressive accumulation of functionally incompetent lymphocytes, which are monoclonal in origin. It is considered to be synonymous (ie, one disease at different stages) to chronic lymphocytic leukemia (CLL) [1,2]. The malignant cells seen in SLL and CLL have identical pathologic and immunophenotypic features. A distinction between these two diagnoses is made based upon the patient's clinical presentation. (See "Clinical presentation, pathologic features, diagnosis, and differential diagnosis of chronic lymphocytic leukemia" and 'Diagnosis' below.)
The pathologic features, diagnosis, and differential diagnosis of SLL will be reviewed here. The pathophysiology, molecular biology, cytogenetic abnormalities, and treatment of SLL are discussed elsewhere. (See "Overview of the treatment of chronic lymphocytic leukemia" and "Pathophysiology and genetic features of chronic lymphocytic leukemia".)
CLL/SLL comprises 90 percent of chronic lymphocytic leukemias in the United States and Europe. Less than 10 percent of patients present with a non-leukemic picture having only nodal involvement (ie, SLL); this presentation accounts for less than 5 percent of all non-Hodgkin lymphomas. However, most patients with SLL at presentation ultimately develop bone marrow and blood infiltration (CLL).
The median age at diagnosis is 65 years . Approximately 80 percent have stage IV disease and 70 to 90 percent have bone marrow involvement at diagnosis (table 1 and table 2). (See "Evaluation, staging, and response assessment of non-Hodgkin lymphoma", section on 'Staging'.)
Most patients with SLL present with painless generalized lymphadenopathy, which has frequently been present for several years. B symptoms occur in a minority of patients . Both hepatosplenomegaly and extranodal infiltrates may be seen.
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