Details of Disease

Disease Name

MALT lymphoma

lymphoma, mucosa-associated lymphoid type; gastric lymphoma, primary; extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma); familial primary gastric lymphoma; lymphoma, MALT, somatic; lymphoma of mucosa-associated lymphoid tissue; Extranodal marginal zone B-cell lymphoma; Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue; Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue; mucosa-associated lymphatic tissue lymphoma; Immunocytoma; mucosa-associated lymphoid tissue lymphoma; MALT-lymphoma; MALToma; Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma); MALT lymphoma

Definition

An indolent, extranodal type of non-Hodgkin lymphoma composed of small B-lymphocytes (centrocyte-like cells). The gastrointestinal tract is the most common site of involvement. Other common sites of involvement include lung, head and neck, ocular adnexae, skin, thyroid, and breast. Gastric involvement is associated with the presence of H. pylori infection. (WHO, 2001)

Disease Hierarchy

DISYKSRF: Genetic disease

DISN6V4S: Lymphoma

DISKAXO0: Neoplasm of mature B-cells

DISLZ4AO: Marginal zone lymphoma

DIS1AVVE: MALT lymphoma

Disease Identifiers

MONDO ID

MONDO_0007650

MESH ID

D018442

UMLS CUI

C0242647

MedGen ID

66942

Orphanet ID

52417

SNOMED CT ID

277622004

General Information of Disease (ID: DIS1AVVE)

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 14 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Bortezomib	DMNO38U	Approved	Small molecular drug	[1]
Doxorubicin	DMVP5YE	Approved	Small molecular drug	[2]
Fludarabine	DMVRLT7	Approved	Small molecular drug	[3]
Gemcitabine	DMSE3I7	Approved	Small molecular drug	[4]
Lenalidomide	DM6Q7U4	Approved	Small molecular drug	[5]
Mycophenolate mofetil	DMPQAGE	Approved	Small molecular drug	[6]
Mycophenolic acid	DMRBMAU	Approved	NA	[7]
Oprelvekin	DMTSRI6	Approved	NA	[7]
Pegfilgrastim	DM7UP8X	Approved	Small molecular drug	[7]
Rituximab	DM1YVZT	Approved	Antibody	[8]
Tacrolimus	DMZ7XNQ	Approved	Small molecular drug	[9]
Thalidomide	DM70BU5	Approved	Small molecular drug	[10]
Vidarabine	DM0N85H	Approved	Small molecular drug	[11]
Vincristine	DMINOX3	Approved	Small molecular drug	[12]
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⏷ Show the Full List of 14 Drug(s)

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 24 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CXCR3	TT1UCIJ	Limited	Altered Expression	[13]
IFNGR2	TT13TL0	Limited	Genetic Variation	[14]
LAIR1	TTSI7A8	Limited	Biomarker	[15]
LMO2	TTFX379	Limited	Biomarker	[16]
NRG4	TTWAGKJ	Limited	Altered Expression	[17]
STK4	TTCPLVN	Limited	Genetic Variation	[18]
TNFRSF13B	TTL9OD4	Limited	Biomarker	[19]
TNFRSF13C	TT7NJSE	Limited	Genetic Variation	[20]
BTLA	TTER58P	moderate	Altered Expression	[21]
CASP10	TTX5HEK	moderate	Genetic Variation	[22]
CCR8	TTE836A	moderate	Altered Expression	[23]
KDM4C	TTV8CRH	moderate	Genetic Variation	[24]
PIM2	TT69J2Z	moderate	Altered Expression	[25]
PTPRJ	TTWMKXP	moderate	Altered Expression	[26]
TCL1A	TTUKRDV	moderate	Altered Expression	[27]
CD19	TTW640A	Strong	Altered Expression	[28]
FCER2	TTCH6MU	Strong	Biomarker	[29]
FOXP1	TT0MUCI	Strong	Altered Expression	[30]
GPR34	TTVXSTQ	Strong	Genetic Variation	[31]
MADCAM1	TTBD6I7	Strong	Biomarker	[32]
MAP3K20	TTTUZ3O	Strong	Genetic Variation	[33]
MS4A1	TTUE541	Strong	Biomarker	[34]
SELL	TT2IYXF	Strong	Biomarker	[32]
SPN	TTOZAX0	Strong	Altered Expression	[35]
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⏷ Show the Full List of 24 DTT(s)

This Disease Is Related to 2 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
SLC27A5	DT0TQS3	Limited	Genetic Variation	[36]
SLC35B2	DT81RKJ	Limited	Biomarker	[37]
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This Disease Is Related to 29 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CYP4V2	OTQKNK0D	Limited	Biomarker	[38]
DDX6	OTKWXVDY	Limited	Biomarker	[39]
GCSAM	OTZNOPYK	Limited	Biomarker	[16]
N4BP1	OTN2TL6N	Limited	Biomarker	[40]
NGDN	OTBXTCN5	Limited	Biomarker	[41]
PARP9	OT7K4494	Limited	Genetic Variation	[36]
PTPRD	OTZPJ3GX	Limited	Altered Expression	[42]
REEP5	OTZU4TJI	Limited	Biomarker	[43]
TNFRSF10D	OTOSRDJT	Limited	Altered Expression	[44]
CHST2	OTV11IZF	moderate	Biomarker	[32]
CNN3	OTJTAXAP	moderate	Genetic Variation	[24]
GKN2	OTR9DOYG	moderate	Genetic Variation	[45]
HOXC5	OTPQTLKZ	moderate	Altered Expression	[46]
MICB	OTS2DVDW	moderate	Genetic Variation	[47]
MINDY3	OT6YZPWC	moderate	Altered Expression	[48]
NAIP	OTLA925F	moderate	Biomarker	[49]
NCR2	OT2H13BX	moderate	Biomarker	[50]
PWWP3A	OTXQVL4U	moderate	Altered Expression	[51]
SSB	OTCCTPBR	moderate	Biomarker	[52]
TCL1B	OT4CSO39	moderate	Altered Expression	[27]
TENM2	OTPLUYEF	moderate	Genetic Variation	[24]
CD79A	OTOJC8DV	Strong	Altered Expression	[53]
ITGAX	OTOGIMHE	Strong	Altered Expression	[54]
KNTC1	OTI2OOFN	Strong	Biomarker	[29]
MNDA	OTCTKR47	Strong	Biomarker	[55]
POTEF	OTV3WXYE	Strong	Biomarker	[56]
SLC7A4	OTAVC6QS	Strong	Genetic Variation	[57]
SMUG1	OT2YIOCQ	Strong	Biomarker	[58]
FCRL4	OT3DVTRV	Definitive	Biomarker	[59]
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⏷ Show the Full List of 29 DOT(s)

References

1	Bortezomib FDA Label
2	Doxorubicin FDA Label
3	Fludarabine FDA Label
4	Gemcitabine FDA Label
5	Lenalidomide FDA Label
6	Mycophenolate mofetil FDA Label
7	A standard database for drug repositioning. Sci Data. 2017 Mar 14;4:170029.
8	Diagnosis and Treatment for Gastric Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma. J Clin Med. 2022 Dec 23;12(1):120.
9	Tacrolimus FDA Label
10	Thalidomide FDA Label
11	Vidarabine FDA Label
12	Vincristine FDA Label
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15	Significant association between TNFAIP3 inactivation and biased immunoglobulin heavy chain variable region 4-34 usage in mucosa-associated lymphoid tissue lymphoma.J Pathol. 2017 Sep;243(1):3-8. doi: 10.1002/path.4933. Epub 2017 Aug 7.
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17	The role of neuregulin4 and HER4 in gastrointestinal malignant lymphoma.Mol Med Rep. 2011 Nov-Dec;4(6):1151-5. doi: 10.3892/mmr.2011.542. Epub 2011 Jul 26.
18	Successful Curative Therapy With Rituximab and Allogeneic Haematopoietic Stem Cell Transplantation for MALT Lymphoma Associated With STK4-Mutated CD4+ Lymphocytopenia.Pediatr Blood Cancer. 2016 Sep;63(9):1657-9. doi: 10.1002/pbc.26048. Epub 2016 May 10.
19	Nodal marginal zone lymphoma: gene expression and miRNA profiling identify diagnostic markers and potential therapeutic targets.Blood. 2012 Jan 19;119(3):e9-e21. doi: 10.1182/blood-2011-02-339556. Epub 2011 Nov 22.
20	A BAFF receptor His159Tyr mutation in Sjgren's syndrome-related lymphoproliferation.Arthritis Rheumatol. 2015 Oct;67(10):2732-41. doi: 10.1002/art.39231.
21	High expression of the inhibitory receptor BTLA in T-follicular helper cells and in B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia.Am J Clin Pathol. 2009 Oct;132(4):589-96. doi: 10.1309/AJCPPHKGYYGGL39C.
22	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.
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25	PIM2 inhibition as a rational therapeutic approach in B-cell lymphoma.Blood. 2011 Nov 17;118(20):5517-27. doi: 10.1182/blood-2011-03-344374. Epub 2011 Sep 21.
26	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.
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29	Immunophenotypic profiles for distinguishing orbital mucosa-associated lymphoid tissue lymphoma from benign lymphoproliferative tumors.Jpn J Ophthalmol. 2017 Jul;61(4):354-360. doi: 10.1007/s10384-017-0513-1. Epub 2017 Apr 18.
30	FOXP1 directly represses transcription of proapoptotic genes and cooperates with NF-B to promote survival of human B cells.Blood. 2014 Nov 27;124(23):3431-40. doi: 10.1182/blood-2014-01-553412. Epub 2014 Sep 29.
31	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.
32	Induction of high endothelial venule-like vessels expressing GlcNAc6ST-1-mediated L-selectin ligand carbohydrate and mucosal addressin cell adhesion molecule 1 (MAdCAM-1) in a mouse model of "Candidatus Helicobacter heilmannii"-induced gastritis and gastric mucosa-associated lymphoid tissue (MALT) lymphoma.Helicobacter. 2010 Dec;15(6):538-48. doi: 10.1111/j.1523-5378.2010.00801.x.
33	Frequency of chromosomal aneuploidies and deletions of the RB and TP53 genes in MALT lymphomas harboring the t(14;18)(q32;q21).Cancer Genet Cytogenet. 2006 Jan 1;164(1):81-3. doi: 10.1016/j.cancergencyto.2005.06.012.
34	Pre-Therapeutic Total Lesion Glycolysis on [(18)F]FDG-PET Enables Prognostication of 2-Year Progression-Free Survival in MALT Lymphoma Patients Treated with CD20-Antibody-Based Immunotherapy.Mol Imaging Biol. 2019 Dec;21(6):1192-1199. doi: 10.1007/s11307-019-01329-2.
35	Marginal zone lymphomas in children and the young adult population; characterization of genetic aberrations by FISH and RT-PCR.Mod Pathol. 2010 Jun;23(6):866-73. doi: 10.1038/modpathol.2010.63. Epub 2010 Mar 19.
36	Detection of MALT1 gene rearrangements in BAL fluid cells for the diagnosis of pulmonary mucosa-associated lymphoid tissue lymphoma.Chest. 2012 Jan;141(1):176-182. doi: 10.1378/chest.11-0068. Epub 2011 Jul 14.
37	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.
38	Proposed rituximab biosimilar BCD-020 versus reference rituximab for treatment of patients with indolent non-Hodgkin lymphomas: An international multicenter randomized trial.Hematol Oncol. 2020 Feb;38(1):67-73. doi: 10.1002/hon.2693. Epub 2020 Jan 13.
39	t(11;14)(q23;q32) involving IGH and DDX6 in nodal marginal zone lymphoma.Genes Chromosomes Cancer. 2013 Jan;52(1):33-43. doi: 10.1002/gcc.22004. Epub 2012 Sep 11.
40	N4BP1 restricts HIV-1 and its inactivation by MALT1 promotes viral reactivation.Nat Microbiol. 2019 Sep;4(9):1532-1544. doi: 10.1038/s41564-019-0460-3. Epub 2019 May 27.
41	High prevalence of Chlamydophila psittaci subclinical infection in Italian patients with Sjgren's syndrome and parotid gland marginal zone B-cell lymphoma of MALT-type.Clin Exp Rheumatol. 2014 Jan-Feb;32(1):61-5. Epub 2014 Jan 20.
42	The genetics of nodal marginal zone lymphoma.Blood. 2016 Sep 8;128(10):1362-73. doi: 10.1182/blood-2016-02-696757. Epub 2016 Jun 22.
43	Analysis of microsatellite instability in gastric mucosa-associated lymphoid tissue lymphoma.Leuk Lymphoma. 2013 Apr;54(4):812-8. doi: 10.3109/10428194.2012.723211. Epub 2012 Sep 14.
44	The potential evasion of immune surveillance in mucosa associated lymphoid tissue lymphoma by DcR2-mediated up-regulation of nuclear factor-B.Leuk Lymphoma. 2015 May;56(5):1440-9. doi: 10.3109/10428194.2014.953149. Epub 2014 Nov 5.
45	Specificity of polymerase chain reaction monoclonality for diagnosis of gastric mucosa-associated lymphoid tissue (MALT) lymphoma: direct comparison to Southern blot gene rearrangement.Dig Dis Sci. 1998 Feb;43(2):290-9. doi: 10.1023/a:1018842002926.
46	HOXC4, HOXC5, and HOXC6 expression in non-Hodgkin's lymphoma: preferential expression of the HOXC5 gene in primary cutaneous anaplastic T-cell and oro-gastrointestinal tract mucosa-associated B-cell lymphomas.Blood. 1997 Nov 15;90(10):4116-25.
47	Eight novel MICB alleles, including a null allele, identified in gastric MALT lymphoma patients.Tissue Antigens. 2004 Sep;64(3):276-80. doi: 10.1111/j.1399-0039.2004.00286.x.
48	Bcl10 expression, rearrangement and mutation in MALT lymphoma: correlation with expression of nuclear factor-kappaB.Int J Oncol. 2001 Aug;19(2):283-9.
49	Mutations in apoptosis genes: a pathogenetic factor for human disease.Mutat Res. 2001 Jul;488(3):211-31. doi: 10.1016/s1383-5742(01)00057-6.
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51	Immunohistochemical expression of cereblon and MUM1 as potential predictive markers of response to lenalidomide in extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma).Hematol Oncol. 2018 Feb;36(1):62-67. doi: 10.1002/hon.2472. Epub 2017 Aug 22.
52	Autoimmunity and extranodal lymphocytic infiltrates in lymphoproliferative disorders.J Intern Med. 1999 Mar;245(3):277-86. doi: 10.1046/j.1365-2796.1999.0443f.x.
53	Chronic active B-cell-receptor signalling in diffuse large B-cell lymphoma.Nature. 2010 Jan 7;463(7277):88-92. doi: 10.1038/nature08638.
54	Macrophages and Dendritic Cells Are the Predominant Cells Infected in Measles in Humans.mSphere. 2018 May 9;3(3):e00570-17. doi: 10.1128/mSphere.00570-17. eCollection 2018 May-Jun.
55	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.
56	Translocation t(11;18)(q21;q21) in gastric B-cell lymphomas.Cancer Sci. 2009 May;100(5):881-7. doi: 10.1111/j.1349-7006.2009.01128.x. Epub 2009 Mar 23.
57	Immunoglobulin VH genes in thymic MALT lymphoma are biased toward a restricted repertoire and are frequently unmutated.J Pathol. 2006 Feb;208(3):415-22. doi: 10.1002/path.1889.
58	FDG PET/CT as a diagnostic and prognostic tool for the evaluation of marginal zone lymphoma.Hematol Oncol. 2019 Apr;37(2):168-175. doi: 10.1002/hon.2578. Epub 2019 Mar 18.
59	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.