View source for HAEM4Backup:Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN)

==Primary Author(s)*==

Linsheng Zhang, MD, PhD

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==Graphical Data Links==

'''MDS, MDS/MPN and MPN Tables''' - A comprehensive list of copy number alterations and CN-LOH detectable by CMA testing with strong diagnostic, prognostic and treatment implications in MDS, MDS/MPN and MPN. Tables derived from Kanagal-Shawanna et al., 2018 [PMID [https://www.ncbi.nlm.nih.gov/pubmed/?term=30377088 30377088]] with permission from Cancer Genetics. ''See [[MDS, MDS/MPN and MPN Tables: Recurrent Genomic Alterations Detected by Chromosomal Microarray]].''

No specific recurrent genetic abnormalities are identified for this category of myeloid neoplasms.

==General Disease Overview / Description of Cancer Category==

The MDS/MPNs include a category of myeloid malignancies with overlapping characteristics of myelodysplastic syndrome ([[Myelodysplastic Syndromes (MDS)|MDS]]) and myeloproliferative neoplasm ([[Myeloproliferative Neoplasms (MPN)|MPN]]) at the time of initial presentation (disease onset).

==WHO Classification Pages (Includes Links to Content)==

*[[Chronic Myelomonocytic Leukemia (CMML)]]
*[[Atypical Chronic Myeloid Leukemia (aCML), BCR-ABL1 Negative]]
*[[Juvenile Myelomonocytic Leukemia (JMML)]]
*[[Myelodysplastic/Myeloproliferative Neoplasms with Ring Sideroblasts and Thrombocytosis (MDS/MPN-RS-T)|MDS/MPN with Ring Sideroblasts and Thrombocytosis (MDS/MPN-RS-T)]]
*[[Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN), Unclassifiable|MDS/MPN, Unclassifiable]]

==Other Related Pages (Includes Links to Content)==

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==Additional Information<ref>Arber DA, et al., (2017). Introduction and overview of the classification of myeloid neoplasms, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p23.</ref>==

Features of MDS : normal or low blood cell counts; dysplastic changes

Features MPN : Elevated blood cell counts in at least one lineage; hypercellular bone marrow; hepatosplenomegaly

The blasts are < 20% in the blood (leukocytes) and bone marrow (nucleated cells).

Mutations in genes encoding encode components of RAS dependent pathways, and epigenetic regulators such as ''TET2'' and ''ASXL1'', are common, supporting clonal processes; however, diagnostic or specific mutations for this entity of myeloid neoplasms are not identified.

The following situations may have the same or similar clinical and laboratory presentations with MDS/MPN, however, they are exclude from this category:

#Previously diagnosed MPN developed cytopenia(s): classification must be based on initial presentation; avoid “reclassification”.
#Cases with well-defined fusions that can be classified otherwise: ''[[Chronic Myeloid Leukemia (CML), BCR-ABL1 Positive|BCR-ABL1]]''; fusion involving ''[[Myeloid/Lymphoid Neoplasms with PDGFRA Rearrangement|PDGFRA]], [[Myeloid/Lymphoid Neoplasms with PDGFRB Rearrangement|PDGFRB]]'', or ''[[Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement|FGFR1]]''; or ''[[Myeloid/Lymphoid Neoplasms with PCM1-JAK2|PCM1-JAK2]]''.

==References==
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==Notes==
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome.

<nowiki>*</nowiki>The hierarchical tumour classification structure displayed on this page is reproduced from the [https://tumourclassification.iarc.who.int/welcome/ WHO Classification of Tumours] with permission from the copyright holder, ©International Agency for Research on Cancer.