Clinical manifestations, pathologic features, and diagnosis of lymphoplasmacytic 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
Lymphoplasmacytic lymphoma (LPL, previously termed lymphoplasmacytoid lymphoma) is an uncommon mature B cell lymphoma usually involving the bone marrow and, less commonly, the spleen and/or lymph nodes [1,2]. Waldenström macroglobulinemia is a clinicopathologic entity associated with an IgM monoclonal gammopathy in the blood that is virtually always a manifestation of LPL. (See "Classification of the hematopoietic neoplasms".)
The epidemiology, pathobiology, clinical presentation, pathologic features, diagnosis, and differential diagnosis of LPL will be reviewed here. The diagnosis, treatment, and prognosis of Waldenström macroglobulinemia are discussed separately. (See "Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia" and "Treatment and prognosis of Waldenström macroglobulinemia".)
LPL accounts for roughly 1 percent of hematologic malignancies in the United States and Western Europe with an incidence of approximately 8.3 cases per million persons per year [3-6]. The incidence is approximately 10-fold lower in Asia . The vast majority of patients are Caucasian with other ethnic groups accounting for approximately 5 percent of cases . The median age is 65 years and 50 to 60 percent of patients are male.
The majority of patients with LPL have a circulating monoclonal IgM that can lead to a hyperviscosity syndrome known as Waldenström macroglobulinemia (WM). Although LPL appears to be a sporadic disease in the majority of cases, a familial predisposition is present in some cases. The epidemiology of LPL and WM is discussed in more detail separately. (See "Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia", section on 'Epidemiology'.)
Occasional cases classified as LPL demonstrate a mixed (type II) cryoglobulinemia and concurrent hepatitis C virus (HCV) infection [9-11]. However, many such cases may in fact fall into the diagnostic category of splenic marginal zone lymphoma, which has also been associated with hepatitis C infection and may be accompanied by paraproteinemia. (See 'Marginal zone lymphoma' below.)
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- MYD88 mutations
- CLINICAL FEATURES
- LABORATORY FINDINGS
- - Bone marrow
- - Lymph node
- - Spleen
- - Peripheral blood
- Genetic features
- DIFFERENTIAL DIAGNOSIS
- Chronic lymphocytic leukemia
- Multiple myeloma
- Mantle cell lymphoma
- Marginal zone lymphoma
- Follicular lymphoma