SCLL
MCID: CHR375
MIFTS: 27

Chromosome 8p11 Myeloproliferative Syndrome (SCLL)

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases

Aliases & Classifications for Chromosome 8p11 Myeloproliferative Syndrome

MalaCards integrated aliases for Chromosome 8p11 Myeloproliferative Syndrome:

Name: Chromosome 8p11 Myeloproliferative Syndrome 57 29 13 72
Stem Cell Leukemia/lymphoma 57 59
Myeloid/lymphoid Neoplasm Associated with Fgfr1 Rearrangement 59
Myeloid and Lymphoid Neoplasms with Fgfr1 Rearrangement 72
Stem Cell Leukemia/lymphoma; Scll 57
8p11 Myeloproliferative Syndrome 59
Scll 57

Classifications:



External Ids:

OMIM 57 613523
ICD10 via Orphanet 34 D47.1
Orphanet 59 ORPHA168953
UMLS 72 C2827362 C3150773

Summaries for Chromosome 8p11 Myeloproliferative Syndrome

OMIM : 57 The 8p11 myeloproliferative syndrome is a rare aggressive condition characterized in its typical form by the occurrence, either simultaneously or sequentially, of a BCR/ABL-negative myeloproliferative disorder and a lymphoma, usually a precursor T-lymphoblastic lymphoma. The disease most often terminates in acute myeloid leukemia (Goradia et al., 2008). (613523)

MalaCards based summary : Chromosome 8p11 Myeloproliferative Syndrome, also known as stem cell leukemia/lymphoma, is related to 8p11 myeloproliferative syndrome and myeloproliferative neoplasm. An important gene associated with Chromosome 8p11 Myeloproliferative Syndrome is SCLL (Chromosome 8p11 Myeloproliferative Syndrome). The drugs Tacrolimus and Methotrexate have been mentioned in the context of this disorder. Affiliated tissues include myeloid and t cells.

Related Diseases for Chromosome 8p11 Myeloproliferative Syndrome

Graphical network of the top 20 diseases related to Chromosome 8p11 Myeloproliferative Syndrome:



Diseases related to Chromosome 8p11 Myeloproliferative Syndrome

Symptoms & Phenotypes for Chromosome 8p11 Myeloproliferative Syndrome

Clinical features from OMIM:

613523

Drugs & Therapeutics for Chromosome 8p11 Myeloproliferative Syndrome

Drugs for Chromosome 8p11 Myeloproliferative Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 28)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492 6473866
2
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
3
Mesna Approved, Investigational Phase 2 3375-50-6 598
4
Clofarabine Approved, Investigational Phase 2 123318-82-1 119182
5
leucovorin Approved Phase 2 58-05-9 143 6006
6
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
7
Lenograstim Approved, Investigational Phase 2 135968-09-1
8
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
9
Busulfan Approved, Investigational Phase 2 55-98-1 2478
10
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
11 Thymoglobulin Phase 2
12 Folic Acid Antagonists Phase 2
13 Alkylating Agents Phase 2
14 Antilymphocyte Serum Phase 2
15 Nucleic Acid Synthesis Inhibitors Phase 2
16 Vitamin B9 Phase 2
17 Adjuvants, Immunologic Phase 2
18 Folate Phase 2
19 Immunosuppressive Agents Phase 2
20 Vitamin B Complex Phase 2
21 Protective Agents Phase 2
22 Dermatologic Agents Phase 2
23 Immunologic Factors Phase 2
24 Calcineurin Inhibitors Phase 2
25 Antirheumatic Agents Phase 2
26 Antimetabolites Phase 2
27 Antineoplastic Agents, Alkylating Phase 2
28 Antimetabolites, Antineoplastic Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Thiotepa-Clofarabine-Busulfan With Allogeneic Stem Cell Transplant for High Risk Malignancies Completed NCT00857389 Phase 2 Thiotepa;Clofarabine;Busulfan;Thymoglobulin (ATG);G-CSF (Filgrastim);Tacrolimus;Methotrexate;Cyclophosphamide;Mesna
2 An Open Label Study to Investigate the Maximum Tolerated Dose of RO5045337 in Patients With Acute Myelogenous Leukemia (AML), Acute Lymphocytic Leukemia (ALL), Chronic Myelogenous Leukemia (CML) in Blast Phase, or Refractory Chronic Lymphocytic Leukemia/Small Cell Lymphocytic Lymphoma (CLL / SCLL) Completed NCT00623870 Phase 1 RO5045337

Search NIH Clinical Center for Chromosome 8p11 Myeloproliferative Syndrome

Genetic Tests for Chromosome 8p11 Myeloproliferative Syndrome

Genetic tests related to Chromosome 8p11 Myeloproliferative Syndrome:

# Genetic test Affiliating Genes
1 Chromosome 8p11 Myeloproliferative Syndrome 29

Anatomical Context for Chromosome 8p11 Myeloproliferative Syndrome

MalaCards organs/tissues related to Chromosome 8p11 Myeloproliferative Syndrome:

41
Myeloid, T Cells

Publications for Chromosome 8p11 Myeloproliferative Syndrome

Articles related to Chromosome 8p11 Myeloproliferative Syndrome:

(show all 40)
# Title Authors PMID Year
1
Modeling the human 8p11-myeloproliferative syndrome in immunodeficient mice. 38 8
20554971 2010
2
The 8p11 myeloproliferative syndrome: review of literature and an illustrative case report. 38 8
18787627 2008
3
PKC412 inhibits the zinc finger 198-fibroblast growth factor receptor 1 fusion tyrosine kinase and is active in treatment of stem cell myeloproliferative disorder. 38 8
15448205 2004
4
Consistent fusion of ZNF198 to the fibroblast growth factor receptor-1 in the t(8;13)(p11;q12) myeloproliferative syndrome. 8
9716603 1998
5
The t(8;13) atypical myeloproliferative disorder: further analysis of the ZNF198 gene and lack of evidence for multiple genes disrupted on chromosome 13. 8
9694738 1998
6
Fibroblast growth factor receptor 1 is fused to FIM in stem-cell myeloproliferative disorder with t(8;13). 8
9576949 1998
7
The t(8;13)(p11;q11-12) rearrangement associated with an atypical myeloproliferative disorder fuses the fibroblast growth factor receptor 1 gene to a novel gene RAMP. 8
9499416 1998
8
FGFR1 is fused with a novel zinc-finger gene, ZNF198, in the t(8;13) leukaemia/lymphoma syndrome. 8
9425908 1998
9
About the t(8;13)(p11;q12) clinico-pathologic entity. 8
8608264 1996
10
A new myeloproliferative disorder associated with chromosomal translocations involving 8p11: a review. 8
7564500 1995
11
A syndrome of lymphoblastic lymphoma, eosinophilia, and myeloid hyperplasia/malignancy associated with t(8;13)(p11;q11): description of a distinctive clinicopathologic entity. 8
7661940 1995
12
T-cell lymphoblastic lymphoma with eosinophilia associated with subsequent myeloid malignancy. 8
1599015 1992
13
RAC1/2 activation promotes FGFR1 driven leukemogenesis in stem cell leukemia/lymphoma syndrome. 38
31221776 2019
14
Loss of the BCR-FGFR1 GEF Domain Suppresses RHOA Activation and Enhances B-Lymphomagenesis in Mice. 38
30413411 2019
15
FGFR1 fusion kinase regulation of MYC expression drives development of stem cell leukemia/lymphoma syndrome. 38
29720732 2018
16
Distinct signaling programs associated with progression of FGFR1 driven leukemia in a mouse model of stem cell leukemia lymphoma syndrome. 38
30439482 2018
17
miR-339 Promotes Development of Stem Cell Leukemia/Lymphoma Syndrome via Downregulation of the BCL2L11 and BAX Proapoptotic Genes. 38
29735550 2018
18
8p11 Stem Cell Leukemia/Lymphoma Syndrome without Myeloproliferation: A Rare Clinical Entity. 38
29622897 2018
19
Mutation in the FGFR1 tyrosine kinase domain or inactivation of PTEN is associated with acquired resistance to FGFR inhibitors in FGFR1-driven leukemia/lymphomas. 38
28646488 2017
20
Development of ZMYM2-FGFR1 driven AML in human CD34+ cells in immunocompromised mice. 38
27005999 2016
21
[8p11 myeloproliferative syndrome]. 38
23998615 2013
22
Ponatinib suppresses the development of myeloid and lymphoid malignancies associated with FGFR1 abnormalities. 38
22781593 2013
23
Src activation plays an important key role in lymphomagenesis induced by FGFR1 fusion kinases. 38
21937681 2011
24
Constitutive Notch pathway activation in murine ZMYM2-FGFR1-induced T-cell lymphomas associated with atypical myeloproliferative disease. 38
21527531 2011
25
Biphenotypic hematologic malignancy: a case report of the 8p11 myeloproliferative syndrome in a child. 38
20562652 2010
26
Chromosome 8p11.2 translocations: prevalence, FISH analysis for FGFR1 and MYST3, and clinicopathologic correlates in a consecutive cohort of 13 cases from a single institution. 38
20143402 2010
27
Genetic fingerprinting of the development and progression of T-cell lymphoma in a murine model of atypical myeloproliferative disorder initiated by the ZNF198-fibroblast growth factor receptor-1 chimeric tyrosine kinase. 38
19506298 2009
28
A novel cytogenetic aberration found in stem cell leukemia/lymphoma syndrome. 38
17928887 2008
29
14-3-3 Integrates prosurvival signals mediated by the AKT and MAPK pathways in ZNF198-FGFR1-transformed hematopoietic cells. 38
17389761 2007
30
Oncogenes in myeloproliferative disorders. 38
17351342 2007
31
Classification of chronic myeloid disorders: from Dameshek towards a semi-molecular system. 38
16781478 2006
32
The t(8;17)(p11;q23) in the 8p11 myeloproliferative syndrome fuses MYO18A to FGFR1. 38
15800673 2005
33
The 8p11 myeloproliferative syndrome: a distinct clinical entity caused by constitutive activation of FGFR1. 38
11919391 2002
34
ZNF198-FGFR1 transforming activity depends on a novel proline-rich ZNF198 oligomerization domain. 38
10887137 2000
35
Cloning and mapping of members of the MYM family. 38
10486218 1999
36
CD56+CD7+ stem cell leukemia/lymphoma with D2-Jdelta1 rearrangement. 38
10435360 1999
37
Electron microscopical analysis of myeloperoxidase of leukemic cells in CD7+ stem cell leukemia/lymphoma. 38
10359066 1999
38
T-stem cell leukemia/lymphoma with both myeloid lineage conversion and T-specific delta recombination. 38
9379684 1997
39
Translocation t(8;13)(p11;q11-12) in stem cell leukemia/lymphoma of T-cell and myeloid lineages. 38
7535088 1995
40
CD7+ stem cell leukemia/lymphoma. Features of a subgroup without circulating blast cells. 38
7685244 1993

Variations for Chromosome 8p11 Myeloproliferative Syndrome

Expression for Chromosome 8p11 Myeloproliferative Syndrome

Search GEO for disease gene expression data for Chromosome 8p11 Myeloproliferative Syndrome.

Pathways for Chromosome 8p11 Myeloproliferative Syndrome

GO Terms for Chromosome 8p11 Myeloproliferative Syndrome

Sources for Chromosome 8p11 Myeloproliferative Syndrome

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