Details of Disease

General Information of Disease (ID: DIS7MP4T)

• Disease Name: Nodal marginal zone lymphoma
• Synonyms: nodal marginal zone lymphoma; nodal marginal zone B-cell lymph.; NMZL; nodal marginal zone B-cell lymphoma; Monocytoid B-cell lymphoma
• Disease Class: 2A85: Mature B-cell lymphoma
• Definition: Nodal marginal zone B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma, characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement of the lymph nodes, sometimes the bone marrow, and rarely the blood. Clinically it presents with disseminated peripheral, abdominal and/or thoracic lymphadenopathy. Cytopenia and bulky tumors (greater than 5 cm) are rare. Association with Hepatitis C virus and chronic inflammation has been reported.
• Disease Hierarchy:
  DISLZ4AO: Marginal zone lymphoma
  DIS7MP4T: Nodal marginal zone lymphoma
• ICD Code:
  ICD-11: ICD-11: 2A85.0
  ICD-10: ICD-10: C88.7
  Expand ICD-11: '2A85.0
  Expand ICD-10: 'C88.7
• Disease Identifiers:
  MONDO ID: MONDO_0019465
  UMLS CUI: C0855139
  MedGen ID: 208959
  Orphanet ID: 86867
  SNOMED CT ID: 277623009

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 15 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]
Ublituximab	DMHTY9W	Approved	Antibody	[11]
Vidarabine	DM0N85H	Approved	Small molecular drug	[12]
Vincristine	DMINOX3	Approved	Small molecular drug	[13]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 3 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CYP4V2	OTQKNK0D	Limited	Biomarker	[14]
DDX6	OTKWXVDY	Limited	Biomarker	[15]
MNDA	OTCTKR47	moderate	Altered Expression	[16]

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] Nodal marginal zone lymphoma. Leuk Lymphoma. 2014 Jun;55(6):1240-50.
• [9] Tacrolimus FDA Label
• [10] Thalidomide FDA Label
• [11] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [12] Vidarabine FDA Label
• [13] Vincristine FDA Label
• [14] 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.
• [15] 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.
• [16] Identification of MNDA as a new marker for nodal marginal zone lymphoma.Leukemia. 2009 Oct;23(10):1847-57. doi: 10.1038/leu.2009.108. Epub 2009 May 28.