Lymphoplasmacytic lymphoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Synonyms and keywords: Waldenstrom's macroglobulinemia; Waldenstrom's disease; Primary macroglobulinemia; Hyperviscosity syndrome; Lymphoplasmacytoid lymphoma
Overview
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][1] Waldenström macroglobulinemia is a clinicopathologic entity associated with an IgM monoclonal gammopathy in the blood that is virtually always a manifestation of LPL.Waldenström macroglobulinemia (WM) is a rare lymphoproliferative disorder characterized by the presence of a serum, IgM paraprotein, associated with infiltration of the bone marrow by lymphoplasmacytic lymphoma. Waldenström macroglobulinemia is a type of lymphoproliferative disease involving lymphocytes with IgM as the main attributing antibody and shares clinical characteristics with the indolent non-Hodgkin lymphomas. Waldenström's macroglobulinemia was first discovered by Jan G. Waldenström and represents 1% of all hematological cancers. Common causes of this disease include genetic, environmental, and autoimmune factors. While common risk factors include monoclonal gammopathy of undetermined significance, age >50 year old, white ethnicity, heredity, hepatitis C, and immune disorders. Genes involved in the pathogenesis of Waldenström macroglobulinemia include: MYD88-L265P, CXCR4 and chromosomes 6q, 13q, 3q, 6p and 18q. The hallmark of Waldenström's macroglobulinemia is hyper-viscosity syndrome. If left untreated, patients with asymptomatic Waldenström's macroglobulinemia may progress to develop a symptomatic disease. Common complications of Waldenström's macroglobulinemia include: hyperviscosity syndrome, cold haemagglutinin disease, cryoglobulinemia, peripheral neuropathy, venous thromboembolism, primary amyloidosis, mal-absorptive diarrhea, and bleeding manifestations. Prognosis varies depending on the multiple factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease. Signs and symptoms of patients with Waldenström's macroglobulinemias depend on the degree of tissue infiltration by malignant tumor cells, hyper-viscosity syndrome, and accumulation of paraprotein. The diagnosis of Waldenström macroglobulinemia is based on bone marrow biopsy and serum protein analysis. Risk stratification determines the protocol of management used for Waldenström macroglobulinemia patients. Watchful waiting is recommended for asymptomatic Waldenström's macroglobulinemia. Symptomatic Waldenström's macroglobulinemia is treated with Rituximab +/- Chemotherapy.
Diagnosis
- Not all the diagnostic tests mentioned are performed in a WM patient. A doctor takes into account the following factors before choosing diagnostic tests in a particular patient:
- Suspected type of cancer.
- Signs and symptoms.
- Age.
- Medical condition of the patient.
- Results of earlier medical tests.
References
- ↑ 1.0 1.1 Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R; et al. (2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.