Details of Disease

General Information of Disease (ID: DISCGTZY)

• Disease Name: Splenic marginal zone lymphoma
• Synonyms: splenic marginal zone lymphoma with villous lymphocytes; SLVL; splenic marginal zone B-cell lymphoma with villous lymphocytes; marginal zone lymphoma of spleen; marginal zone lymphoma of the spleen; splenic lymphoma with circulating villous lymphocytes; splenic marginal zone B-cell lymphoma; SMZL; splenic marginal zone lymphoma
• Definition: Splenic marginal zone lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement in the spleen, bone marrow and, frequently, the blood. It usually presents with splenomegaly, lymphocytosis, anemia and/or thrombocytopenia. Hepatitis C virus and autoimmune manifestations, such as autoimmune hemolytic anemia and autoimmune thrombocytopenia, could be associated.
• Disease Hierarchy:
  DISLZ4AO: Marginal zone lymphoma
  DISCGTZY: Splenic marginal zone lymphoma
• Disease Identifiers:
  MONDO ID: MONDO_0019462
  UMLS CUI: C0349632
  MedGen ID: 138108
  Orphanet ID: 86854
  SNOMED CT ID: 1.17E+14

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 13 Approved Drug(s)

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

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 7 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
IKBKB	TTJ3E9X	Limited	Genetic Variation	[12]
NOTCH2	TT82FVD	Limited	Biomarker	[13]
BIRC3	TTAIWZN	Strong	Altered Expression	[14]
FCER2	TTCH6MU	Strong	Genetic Variation	[15]
MALT1	TTCI81G	Strong	Biomarker	[16]
PDE10A	TTJW4LU	Strong	Genetic Variation	[17]
TCL1A	TTUKRDV	Strong	Altered Expression	[18]

This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
SLC35B2	DT81RKJ	Strong	Genetic Variation	[19]

This Disease Is Related to 6 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CYP4V2	OTQKNK0D	Limited	Biomarker	[20]
AMOTL1	OT40G45S	Strong	Genetic Variation	[21]
CENPX	OTG1RAKJ	Strong	Altered Expression	[22]
KLF2	OTIP1UFX	Strong	Biomarker	[23]
POU2F2	OTPV0J0C	Strong	Biomarker	[24]
SLC7A4	OTAVC6QS	Strong	Genetic Variation	[25]

References

• [1] Doxorubicin FDA Label
• [2] Fludarabine FDA Label
• [3] Gemcitabine FDA Label
• [4] Lenalidomide FDA Label
• [5] Mycophenolate mofetil FDA Label
• [6] A standard database for drug repositioning. Sci Data. 2017 Mar 14;4:170029.
• [7] Treatment of splenic marginal zone lymphoma. Best Pract Res Clin Haematol. 2017 Mar-Jun;30(1-2):139-148.
• [8] Tacrolimus FDA Label
• [9] Thalidomide FDA Label
• [10] Vidarabine FDA Label
• [11] Vincristine FDA Label
• [12] IB kinase (IKBKB) mutations in lymphomas that constitutively activate canonical nuclear factor B (NFB) signaling.J Biol Chem. 2014 Sep 26;289(39):26960-26972. doi: 10.1074/jbc.M114.598763. Epub 2014 Aug 8.
• [13] Splenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization.Med Oncol. 2018 Aug 2;35(9):118. doi: 10.1007/s12032-018-1183-7.
• [14] Alteration of BIRC3 and multiple other NF-B pathway genes in splenic marginal zone lymphoma.Blood. 2011 Nov 3;118(18):4930-4. doi: 10.1182/blood-2011-06-359166. Epub 2011 Aug 31.
• [15] Splenic marginal zone lymphomas presenting with splenomegaly and typical immunophenotype are characterized by allelic loss in 7q31-32.Mod Pathol. 2003 Dec;16(12):1210-7. doi: 10.1097/01.MP.0000095895.19756.77.
• [16] Establishment and characterization of the new splenic marginal zone lymphoma-derived cell line UCH1 carrying a complex rearrangement involving t(8;14) and chromosome 3.Leuk Lymphoma. 2007 Apr;48(4):767-73. doi: 10.1080/10428190601094768.
• [17] Whole exome sequencing of microdissected splenic marginal zone lymphoma: a study to discover novel tumor-specific mutations.BMC Cancer. 2015 Oct 24;15:773. doi: 10.1186/s12885-015-1766-z.
• [18] TCL1: a shared tumor-associated antigen for immunotherapy against B-cell lymphomas.Blood. 2012 Aug 23;120(8):1613-23. doi: 10.1182/blood-2011-09-382838. Epub 2012 May 29.
• [19] An unusual case of indolent B-cell lymphoma with distinct chronic lymphocytic leukemia and marginal zone differentiation according to the site of involvement.Leuk Lymphoma. 2005 Sep;46(9):1369-74. doi: 10.1080/10428190500138039.
• [20] 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.
• [21] A study of the mutational landscape of pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma.Mod Pathol. 2016 Oct;29(10):1212-20. doi: 10.1038/modpathol.2016.102. Epub 2016 Jun 24.
• [22] Clonal expansion and functional exhaustion of monoclonal marginal zone B cells in mixed cryoglobulinemia: the yin and yang of HCV-driven lymphoproliferation and autoimmunity.Autoimmun Rev. 2013 Jan;12(3):430-5. doi: 10.1016/j.autrev.2012.08.016. Epub 2012 Aug 23.
• [23] Splenic marginal zone lymphoma: from genetics to management.Blood. 2016 Apr 28;127(17):2072-81. doi: 10.1182/blood-2015-11-624312. Epub 2016 Mar 17.
• [24] Classical Hodgkin lymphoma concurrently evolving in a patient with marginal zone B-cell lymphoma of the spleen.Ann Diagn Pathol. 2008 Jun;12(3):212-6. doi: 10.1016/j.anndiagpath.2006.12.005. Epub 2007 Sep 14.
• [25] Splenic lymphoma with villous lymphocytes involves B cells with extensively mutated Ig heavy chain variable region genes.Blood. 1995 Mar 15;85(6):1603-7.