B-chronic lymphocytic leukemia, small lymphocytic lymphoma, and lymphoplasmacytic lymphoma, including Waldenström's macroglobulinemia: a clinical, morphologic, and biologic spectrum of similar disorders
- PMID: 10319379
B-chronic lymphocytic leukemia, small lymphocytic lymphoma, and lymphoplasmacytic lymphoma, including Waldenström's macroglobulinemia: a clinical, morphologic, and biologic spectrum of similar disorders
Abstract
Among small lymphocyte cell disorders, B-chronic lymphocytic leukemia (B-CLL), small lymphocytic lymphoma (SLL), and lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia (LPL/MW) are included. B-CLL patients always have blood and bone marrow (BM) involvement by a CD5+ B lymphocyte. They frequently present with lymphadenopathy and/or hepatosplenomegaly, although in a considerable number of patients, no abnormal physical findings are found. They are prone to develop hypogammaglobulinemia, autoimmune hemolysis, or autoimmune thrombocytopenia. The typical immunophenotype of the malignant cell is CD5+, surface immunoglobulin (slg)+ (weak), CD23+, CD79b-, and FMC7-. Trisomy 12 and 13q deletions are frequent chromosomal abnormalities. The bcl-2 protein is usually overexpressed. SLL patients present with lymphadenopathy, usually generalized. Lymphocytosis is by definition absent and BM involvement, usually nodular, is found in 25% to 50% of patients. The lymph node lymphocytes are CD5+ and have a similar immunophenotype with CLL, but frequently express the LFA-1 adhesion molecule. Patients are at low risk to develop hypogammaglobulinemia, autoimmune hemolysis, or autoimmune thrombocytopenia. LPL/MW patients may present either with an accidental discovery of IgM gammopathy, symptoms related to paraproteinemia, or lymphadenopathy and/or splenomegaly. The BM is frequently involved and a leukemic picture may be found. A monoclonal gammopathy of IgM class is by definition present in MW and is frequently accompanied by hypogammaglobulinemia. Immunophenotypic studies usually reveal a CD5-, slg+ (moderate), cytoplasmic immunoglobulin (clg)+, FMC7+, and CD38+ cell. A significant proportion of cases carry the translocation t(9;14)(p13;q32) involving the PAX-5 gene. All of these disorders may potentially undergo transformation to large-cell lymphoma or Richter's syndrome. Prognostic factors have been extensively studied in B-CLL, but more studies are needed for SLL and LPL/MW. These entities should be differentiated from other B-chronic small lymphocyte cell disorders, particularly when the latter are leukemic.
Similar articles
-
Immunophenotypic analysis of Waldenstrom's macroglobulinemia.Semin Oncol. 2003 Apr;30(2):187-95. doi: 10.1053/sonc.2003.50074. Semin Oncol. 2003. PMID: 12720134
-
Immunophenotypic profile of lymphoplasmacytic lymphoma/Waldenström macroglobulinemia.Am J Clin Pathol. 2005 Sep;124(3):414-20. doi: 10.1309/3G1X-DX0D-VHBN-VKB4. Am J Clin Pathol. 2005. PMID: 16191510
-
Differential diagnosis of Waldenstrom's macroglobulinemia from other low-grade B-cell lymphoproliferative disorders.Semin Oncol. 2003 Apr;30(2):201-5. doi: 10.1053/sonc.2003.50046. Semin Oncol. 2003. PMID: 12720136
-
Lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia.Crit Rev Oncol Hematol. 2008 Aug;67(2):172-85. doi: 10.1016/j.critrevonc.2008.03.008. Epub 2008 May 21. Crit Rev Oncol Hematol. 2008. PMID: 18499469 Review.
-
Lymphoplasmacytic lymphoma/immunocytoma: towards a disease-targeted treatment?J Exp Clin Cancer Res. 2001 Sep;20(3):351-8. J Exp Clin Cancer Res. 2001. PMID: 11718214 Review.
Cited by
-
Immunogenicity of influenza vaccination in patients with non-Hodgkin lymphoma.J Clin Immunol. 2007 May;27(3):339-46. doi: 10.1007/s10875-007-9073-3. Epub 2007 Mar 8. J Clin Immunol. 2007. PMID: 17345151
-
VR09 cell line: an EBV-positive lymphoblastoid cell line with in vivo characteristics of diffuse large B cell lymphoma of activated B-cell type.PLoS One. 2012;7(12):e52811. doi: 10.1371/journal.pone.0052811. Epub 2012 Dec 21. PLoS One. 2012. PMID: 23285191 Free PMC article.
-
CD5 maintains tolerance in anergic B cells.J Exp Med. 2000 Mar 6;191(5):883-90. doi: 10.1084/jem.191.5.883. J Exp Med. 2000. PMID: 10704468 Free PMC article.
-
Campath-1H (anti-CD52) monoclonal antibody therapy in lymphoproliferative disorders.Med Oncol. 2001;18(2):99-107. doi: 10.1385/mo:18:2:99. Med Oncol. 2001. PMID: 11778765 Review.
-
Babesia microti-induced fulminant sepsis in an immunocompromised host: A case report and the case-specific literature review.Open Life Sci. 2022 Sep 14;17(1):1200-1207. doi: 10.1515/biol-2022-0448. eCollection 2022. Open Life Sci. 2022. PMID: 36185407 Free PMC article.
Publication types
MeSH terms
Substances
LinkOut - more resources
Research Materials