2017
DOI: 10.1016/j.bjhh.2016.09.014
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Splenic marginal zone lymphoma: a literature review of diagnostic and therapeutic challenges

Abstract: Splenic marginal zone lymphoma (SMZL) is a low-grade B-cell non-Hodgkin's lymphoma characterized by massive splenomegaly, moderate lymphocytosis with or without villous lymphocytes, rare involvement of peripheral lymph nodes and indolent clinical course. As a rare disease, with no randomized prospective trials, there is no standard of care for SMZL so far. Splenectomy has been done for many years as an attempt to control disease, but nowadays it has not been encouraged as first line because of new advances in … Show more

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Cited by 33 publications
(35 citation statements)
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“…Splenic marginal zone lymphoma (SMZL) is a non-Hodgkin lymphoma malignancy of mature B-cells that originally involves the spleen, but it can also affect peripheral organs. SMZL is a rare malignancy that accounts for less than 2% of all lymphomas and accounts for less than 1% of non-Hodgkin's lymphomas [26]. Di Lisio et al evaluated a set of miRNAs in B cell lymphomas, including SMZL identifying expressed miRNAs in varying types of B-cell lymphomas.…”
Section: Mir-144 In Splenic Marginal Zone Lymphomamentioning
confidence: 99%
“…Splenic marginal zone lymphoma (SMZL) is a non-Hodgkin lymphoma malignancy of mature B-cells that originally involves the spleen, but it can also affect peripheral organs. SMZL is a rare malignancy that accounts for less than 2% of all lymphomas and accounts for less than 1% of non-Hodgkin's lymphomas [26]. Di Lisio et al evaluated a set of miRNAs in B cell lymphomas, including SMZL identifying expressed miRNAs in varying types of B-cell lymphomas.…”
Section: Mir-144 In Splenic Marginal Zone Lymphomamentioning
confidence: 99%
“…There are no defining diagnostic molecular features of SMZL, but characteristic findings in a minority of cases include del(7q), trisomy 3, NOTCH2 mutations, and KLF2 mutations (39,40). There are no defining diagnostic molecular features of SMZL, but characteristic findings in a minority of cases include del(7q), trisomy 3, NOTCH2 mutations, and KLF2 mutations (39,40).…”
Section: Cd5-negative Cd10-negative Small B-cell Neoplasmsmentioning
confidence: 99%
“…SMZL lacks most HCL markers, but may weakly express CD103. There are no defining diagnostic molecular features of SMZL, but characteristic findings in a minority of cases include del(7q), trisomy 3, NOTCH2 mutations, and KLF2 mutations (39,40). Recently, expression of CD1d in conjunction with CD200 was reported to be of diagnostic utility in sub-classification of CD5-negative/CD10-negative B-cell neoplasms (41,42).…”
Section: Cd5-negative Cd10-negative Small B-cell Neoplasmsmentioning
confidence: 99%
“…Both were positive for the B-cell markers (Figure 6M-N'). These features are reminiscent of human SMZL (Splenic Marginal Zone B-cell -Lymphoma), a clonal B-cell neoplasm that manifests itself in elderly patients and consists of small lymphocytes that infiltrate the lymph nodes and other organs(Arcaini et al, 2016;Piris et al, 2017;Santos et al, 2017). As would be expected, the enlarged lymph nodes and spleens were highly proliferative as indicated by Ki67 and phospho-H3 staining (Supplemental…”
mentioning
confidence: 66%
“…For example, Notch1 promotes T-cell development (Pui et al, 1999;Radtke et al, 2010;Radtke et al, 1999), while Notch2 is indispensable for Marginal Zone B-cell (MZB) development (Gibb et al, 2010;Hozumi et al, 2004;Liu et al, 2015;Maillard et al, 2004;Moran et al, 2007;Saito et al, 2003;Tanigaki et al, 2002;Witt et al, 2003). Accordingly, elevated Notch1 signaling is oncogenic in T-cells driving Acute Lymphoblastic Leukemia (T-ALL) (Ellisen et al, 1991;Weng et al, 2004) while increased Notch2 signaling is associated with splenic marginal zone B-cell transformation (Arcaini et al, 2016;Piris et al, 2017;Santos et al, 2017). Inversely, where Notch signals promote differentiation, the pathway can have a tumor suppressor function with diminished Notch signaling being associated with cancer (Demehri et al, 2009;Koch and Radtke, 2007).…”
Section: Introductionmentioning
confidence: 99%