Details of Disease

General Information of Disease (ID: DISLZ4AO)

• Disease Name: Marginal zone lymphoma
• Synonyms: marginal zone B cell lymphoma; MZL; marginal zone lymphoma; lymphoma of marginal zone B cell; MZBCL; marginal zone B-cell lymphoma
• Disease Class: 2A85: Mature B-cell lymphoma
• Definition: A usually indolent mature B-cell lymphoma, arising from the marginal zone of lymphoid tissues. It is characterized by the presence of small to medium sized atypical lymphocytes. It comprises three entities, according to the anatomic sites involved: extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, which affects extranodal sites (most often stomach, lung, skin, and ocular adnexa); nodal marginal zone B-cell lymphoma, which affects lymph nodes without evidence of extranodal disease; and splenic marginal zone B-cell lymphoma, which affects the spleen and splenic hilar lymph nodes, bone marrow, and often the peripheral blood.
• Disease Hierarchy:
  DISN6V4S: Lymphoma
  DISPW4EO: Indolent B-cell non-Hodgkin lymphoma
  DIS5VHUX: Spleen neoplasm
  DISLZ4AO: Marginal zone lymphoma
• ICD Code:
  ICD-11: ICD-11: 2A85.0
  ICD-10: ICD-10: C88.7
  Expand ICD-11: 'XH3FE9
• Disease Identifiers:
  MONDO ID: MONDO_0017604
  UMLS CUI: C1367654
  MedGen ID: 277950
  Orphanet ID: 300912
  SNOMED CT ID: 128803008

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Umbralisib	DMYRBO1	Approved	NA	[1]

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
INCB50465	DMZJP2T	Phase 2	NA	[2]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 16 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
LMO2	TTFX379	Limited	Biomarker	[3]
MME	TT5TKPM	Limited	Biomarker	[3]
MYD88	TTB6Q2O	Limited	Genetic Variation	[4]
MALT1	TTCI81G	Disputed	Genetic Variation	[5]
TNFAIP3	TT5W0IO	Disputed	Genetic Variation	[6]
CASP10	TTX5HEK	moderate	Genetic Variation	[7]
IFNGR2	TT13TL0	moderate	Genetic Variation	[8]
PTPRJ	TTWMKXP	moderate	Altered Expression	[9]
BCL6	TTC9YX5	Strong	Genetic Variation	[10]
FCER2	TTCH6MU	Strong	Biomarker	[11]
GPR34	TTVXSTQ	Strong	Genetic Variation	[12]
LAIR1	TTSI7A8	Strong	Biomarker	[13]
MS4A1	TTUE541	Strong	Biomarker	[14]
BIRC3	TTAIWZN	Definitive	Genetic Variation	[15]
IKZF3	TTCZVFZ	Definitive	Genetic Variation	[16]
IL2RA	TT10Y9E	Definitive	Genetic Variation	[16]

This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
SLC35B2	DT81RKJ	Limited	Biomarker	[17]

This Disease Is Related to 16 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
FCRL1	OTXOYT29	Limited	Altered Expression	[18]
GCSAM	OTZNOPYK	Limited	Biomarker	[3]
MEF2B	OT880SE6	Limited	Biomarker	[19]
MNDA	OTCTKR47	Limited	Biomarker	[20]
C1QC	OTLE5U1P	Strong	Genetic Variation	[21]
CARD11	OTRCTLYC	Strong	Biomarker	[22]
FCRL4	OT3DVTRV	Strong	Biomarker	[23]
KNTC1	OTI2OOFN	Strong	Biomarker	[24]
NDUFS4	OTJKUYEE	Strong	Biomarker	[25]
RHOH	OT1J9SEB	Strong	Biomarker	[26]
TLR10	OTQ1KVJO	Strong	Genetic Variation	[27]
BTNL2	OTTTEMZA	Definitive	Genetic Variation	[28]
FBXW8	OTJG15EO	Definitive	Genetic Variation	[16]
KALRN	OT8WRCBH	Definitive	Genetic Variation	[16]
MGAT5	OTU4DD4G	Definitive	Genetic Variation	[16]
NCOA5	OTOGWTWB	Definitive	Genetic Variation	[16]

References

• [1] Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health Human Services. 2021
• [2] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [3] The efficacy of HGAL and LMO2 in the separation of lymphomas derived from small B cells in nodal and extranodal sites, including the bone marrow.Am J Clin Pathol. 2011 May;135(5):697-708. doi: 10.1309/AJCP7Z2BIBUNQPLZ.
• [4] CD13 expression in B cell malignancies is a hallmark of plasmacytic differentiation.Br J Haematol. 2019 Feb;184(4):625-633. doi: 10.1111/bjh.15584. Epub 2018 Sep 10.
• [5] Targeted deep sequencing of gastric marginal zone lymphoma identified alterations of TRAF3 and TNFAIP3 that were mutually exclusive for MALT1 rearrangement.Mod Pathol. 2018 Sep;31(9):1418-1428. doi: 10.1038/s41379-018-0064-0. Epub 2018 May 15.
• [6] The mutational landscape of ocular marginal zone lymphoma identifies frequent alterations in TNFAIP3 followed by mutations in TBL1XR1 and CREBBP.Oncotarget. 2017 Mar 7;8(10):17038-17049. doi: 10.18632/oncotarget.14928.
• [7] Genetic variants in caspase genes and susceptibility to non-Hodgkin lymphoma.Carcinogenesis. 2007 Apr;28(4):823-7. doi: 10.1093/carcin/bgl196. Epub 2006 Oct 27.
• [8] Cytokine polymorphisms in Th1/Th2 pathway genes, body mass index, and risk of non-Hodgkin lymphoma.Blood. 2011 Jan 13;117(2):585-90. doi: 10.1182/blood-2010-07-295097. Epub 2010 Oct 15.
• [9] CD148 and CD27 are expressed in B cell lymphomas derived from both memory and nave B cells.Leuk Lymphoma. 2002 Sep;43(9):1855-8. doi: 10.1080/1042819021000006385.
• [10] An update in treating transformed lymphoma.Best Pract Res Clin Haematol. 2018 Sep;31(3):251-261. doi: 10.1016/j.beha.2018.07.008. Epub 2018 Jul 25.
• [11] Delineation of the breakpoint at 18q21.1 in a cell line (Karpas1106) derived from mediastinal B-cell lymphoma by fluorescence in situ hybridization with multiple YAC clones.Int J Cancer. 1998 Sep 25;78(1):100-5. doi: 10.1002/(sici)1097-0215(19980925)78:1<100::aid-ijc16>3.0.co;2-f.
• [12] t(X;14)(p11.4;q32.33) is recurrent in marginal zone lymphoma and up-regulates GPR34.Haematologica. 2012 Feb;97(2):184-8. doi: 10.3324/haematol.2011.052639. Epub 2011 Nov 4.
• [13] Unclassifiable Isolated Monoclonal Lymphocytosis: Comprehensive Description of a Retrospective Cohort.Cancers (Basel). 2019 Oct 4;11(10):1495. doi: 10.3390/cancers11101495.
• [14] Marginal Zone Lymphoma: State-of-the-Art Treatment.Curr Treat Options Oncol. 2019 Dec 5;20(12):90. doi: 10.1007/s11864-019-0687-5.
• [15] Marginal zone lymphoma-derived interfollicular diffuse large B-cell lymphoma harboring 20q12 chromosomal deletion and missense mutation of BIRC3 gene: a case report.Diagn Pathol. 2016 Dec 19;11(1):137. doi: 10.1186/s13000-016-0588-x.
• [16] Genetic overlap between autoimmune diseases and non-Hodgkin lymphoma subtypes.Genet Epidemiol. 2019 Oct;43(7):844-863. doi: 10.1002/gepi.22242. Epub 2019 Aug 13.
• [17] Genetically predicted longer telomere length is associated with increased risk of B-cell lymphoma subtypes.Hum Mol Genet. 2016 Apr 15;25(8):1663-76. doi: 10.1093/hmg/ddw027. Epub 2016 Feb 9.
• [18] Evaluation of CD307a expression patterns during normal B-cell maturation and in B-cell malignancies by flow cytometry.Cytometry B Clin Cytom. 2018 Jul;94(4):588-595. doi: 10.1002/cyto.b.21631. Epub 2018 Mar 9.
• [19] Comparison of Myocyte Enhancer Factor 2B Versus Other Germinal Center-associated Antigens in the Differential Diagnosis of B-Cell Non-Hodgkin Lymphomas.Am J Surg Pathol. 2018 Mar;42(3):342-350. doi: 10.1097/PAS.0000000000001015.
• [20] Myeloid Cell Nuclear Differentiation Antigen (MNDA) Positivity in Primary Follicles: Potential Pitfall in the Differential Diagnosis With Marginal Zone Lymphoma.Appl Immunohistochem Mol Morphol. 2020 May/Jun;28(5):384-388. doi: 10.1097/PAI.0000000000000738.
• [21] Polymorphisms in complement system genes and risk of non-Hodgkin lymphoma.Environ Mol Mutagen. 2012 Mar;53(2):145-51. doi: 10.1002/em.21675. Epub 2011 Dec 15.
• [22] Dicentric chromosome 3 associated with binucleated lymphocytes in atypical B-cell chronic lymphoproliferative disorder.Leuk Lymphoma. 2002 Sep;43(9):1749-54. doi: 10.1080/1042819021000006501.
• [23] IRTA1 and MNDA Expression in Marginal Zone Lymphoma: Utility in Differential Diagnosis and Implications for Classification.Am J Clin Pathol. 2019 Feb 4;151(3):337-343. doi: 10.1093/ajcp/aqy144.
• [24] Change of Serum IgG4 in Patients with Ocular Adnexal Marginal Zone B Cell Lymphoma Associated with IgG4-Related Ophthalmic Disease After Treatment.J Ocul Pharmacol Ther. 2017 Sep;33(7):543-548. doi: 10.1089/jop.2016.0175. Epub 2017 May 17.
• [25] An open-label phase 2 trial of entospletinib in indolent non-Hodgkin lymphoma and mantle cell lymphoma.Br J Haematol. 2019 Jan;184(2):215-222. doi: 10.1111/bjh.15552. Epub 2018 Sep 5.
• [26] Aberrant somatic hypermutations in thyroid lymphomas.Leuk Res. 2009 May;33(5):649-54. doi: 10.1016/j.leukres.2008.10.007. Epub 2008 Nov 18.
• [27] A pooled investigation of Toll-like receptor gene variants and risk of non-Hodgkin lymphoma.Carcinogenesis. 2009 Feb;30(2):275-81. doi: 10.1093/carcin/bgn262. Epub 2008 Nov 24.
• [28] A genome-wide association study of marginal zone lymphoma shows association to the HLA region.Nat Commun. 2015 Jan 8;6:5751. doi: 10.1038/ncomms6751.