Myelodysplastic Syndromes Treatment (PDQ®): Treatment - Health Professional Information [NCI]-Pathol
Myelodysplastic Syndromes Treatment (PDQ®): Treatment - Health Professional Information [NCI]-Pathologic and Prognostic Systems for Myelodysplastic Syndromes
Myelodysplastic Syndromes Treatment (PDQ®): Treatment - Health Professional Information [NCI] Guide
Unclassifiable myelodysplastic syndrome (MDS-U)
The cellular subtype MDS-U lacks findings appropriate for classification as RA, RARS, RCMD, or RAEB. Blasts in the blood and bone marrow are not increased.
Myelodysplastic syndrome associated with an isolated del(5q) chromosome abnormality
This MDS cellular subtype, the 5q- syndrome, is associated with an isolated del(5q) cytogenetic abnormality. Blasts in both blood and bone marrow are less than 5%. This subtype is associated with a long survival. Karyotypic evolution is uncommon. Additional cytogenetic abnormalities may be associated with a more aggressive MDS cellular subtype or may evolve to AML.
Therapy-related myeloid neoplasms
The latest version of the WHO pathologic classification system identifies patients with therapy-related MDS or AML and places them in the same category as "therapy-related myeloid neoplasms." This group of disorders evolves in patients who were previously treated with chemotherapy or radiation therapy for other cancers and in whom there is a clinical suspicion that the prior therapy caused the myeloid neoplasm. Classic chemotherapy agents associated with these disorders include alkylating agents, topoisomerase inhibitors, and purine nucleoside analogs.
Chronic myelomonocytic leukemia (CMML)
Although previously classified with the myelodysplastic syndromes, CMML is now assigned to a group of overlap myelodysplastic/myeloproliferative neoplasms. (Refer to the PDQ summary on Myelodysplastic/ Myeloproliferative Neoplasms for more information.)
Prognostic Scoring Systems
A variety of pathologic and risk classification systems have been developed to predict the overall survival of patients with MDS and the evolution from MDS to AML. Major prognostic classification systems include the International Prognostic Scoring System (IPSS), revised as the IPSS-R;[3] the WHO Prognostic Scoring System (WPSS); and the MD Anderson Cancer Center Prognostic Scoring Systems.[4,5] Clinical variables in these systems have included bone marrow and blood myeloblast percentage, specific cytopenias, transfusion requirements, age, performance status, and bone marrow cytogenetic abnormalities.
IPSS
The IPSS incorporates bone marrow blast percentage, number of peripheral blood cytopenias, and cytogenetic risk group.
IPSS-R
Compared with the IPSS, the IPSS-R updates and gives greater weight to cytogenetic abnormalities and severity of cytopenias, while reassigning the weighting for blast percentages.[3]
Myelodysplastic Syndromes Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Pathologic and Prognostic Systems for Myelodysplastic Syndromes
Myelodysplastic Syndromes Treatment (PDQ®): Treatment - Health Professional Information [NCI] Guide
- General Information About Myelodysplastic Syndromes
- Pathologic and Prognostic Systems for Myelodysplastic Syndromes
- Treatment for Myelodysplastic Syndromes
- Relapsed or Refractory Myelodysplastic Syndromes
- Current Clinical Trials
- Changes to This Summary (12 / 03 / 2012)
- About This PDQ Summary
- Get More Information From NCI
Table 1. Myelodysplastic Syndromes: Comparison of the FAB and WHO Classifications continued...
Unclassifiable myelodysplastic syndrome (MDS-U)
The cellular subtype MDS-U lacks findings appropriate for classification as RA, RARS, RCMD, or RAEB. Blasts in the blood and bone marrow are not increased.
Myelodysplastic syndrome associated with an isolated del(5q) chromosome abnormality
This MDS cellular subtype, the 5q- syndrome, is associated with an isolated del(5q) cytogenetic abnormality. Blasts in both blood and bone marrow are less than 5%. This subtype is associated with a long survival. Karyotypic evolution is uncommon. Additional cytogenetic abnormalities may be associated with a more aggressive MDS cellular subtype or may evolve to AML.
Therapy-related myeloid neoplasms
The latest version of the WHO pathologic classification system identifies patients with therapy-related MDS or AML and places them in the same category as "therapy-related myeloid neoplasms." This group of disorders evolves in patients who were previously treated with chemotherapy or radiation therapy for other cancers and in whom there is a clinical suspicion that the prior therapy caused the myeloid neoplasm. Classic chemotherapy agents associated with these disorders include alkylating agents, topoisomerase inhibitors, and purine nucleoside analogs.
Chronic myelomonocytic leukemia (CMML)
Although previously classified with the myelodysplastic syndromes, CMML is now assigned to a group of overlap myelodysplastic/myeloproliferative neoplasms. (Refer to the PDQ summary on Myelodysplastic/ Myeloproliferative Neoplasms for more information.)
Prognostic Scoring Systems
A variety of pathologic and risk classification systems have been developed to predict the overall survival of patients with MDS and the evolution from MDS to AML. Major prognostic classification systems include the International Prognostic Scoring System (IPSS), revised as the IPSS-R;[3] the WHO Prognostic Scoring System (WPSS); and the MD Anderson Cancer Center Prognostic Scoring Systems.[4,5] Clinical variables in these systems have included bone marrow and blood myeloblast percentage, specific cytopenias, transfusion requirements, age, performance status, and bone marrow cytogenetic abnormalities.
IPSS
The IPSS incorporates bone marrow blast percentage, number of peripheral blood cytopenias, and cytogenetic risk group.
IPSS-R
Compared with the IPSS, the IPSS-R updates and gives greater weight to cytogenetic abnormalities and severity of cytopenias, while reassigning the weighting for blast percentages.[3]
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