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Medical Dictionary for Regulatory Activities Terminology (MedDRA)
| Id | http://purl.bioontology.org/ontology/MEDDRA/20000217
http://purl.bioontology.org/ontology/MEDDRA/20000217
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| Preferred Name | Myelodysplastic syndrome (SMQ) |
| Definitions |
Myelodysplastic syndrome (MDS) = heterogeneous group of clonal disorders of hematopoiesis. Characterized by: hyper- or hypocellular bone marrow; abnormal cell morphology; abnormal maturation; peripheral blood cytopenias; ineffective blood cell production. All three myeloid cell lineages (erythrocytic, granulocytic, and megakaryocytic) may be involved. Considered a premalignant condition as some patients progress to AML. May present with: anemia, thrombocytopenia, neutropenia. Diagnostic approach includes: complete blood count with differential, peripheral blood smear, bone marrow studies. Treatment: supportive, including transfusions; possible bone marrow stimulation; cytotoxic chemotherapy; bone marrow transplantation has a limited role. May be caused by exposure to: cytotoxic chemotherapy; radiation; viral infection; genotoxic chemicals (e.g., benzene). May also be no known exposure. Cytogenetics include: normal karyotype; balanced chromosomal abnormality and generation of fusion oncogenes; complex karyotypes. Primarily affects the elderly. French-American-British classification: refractory anemia (RA); RA with ringed sideroblasts (RARS); RA with excess blasts (RAEB; 6-20% myeloblasts); RAEB in transition to AML (RAEB-T; 21-30% myeloblasts); chronic myelomonocytic leukemia. 2008 WHO classification: refractory cytopenia with unilineage dysplasia; RARS; RAEB; refractory cytopenia with multilineage dysplasia; MDS with isolated deletion of 5q; MDS, unclassifiable; childhood MDS
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| Type | http://www.w3.org/2002/07/owl#Class |
All Properties
| definition | Myelodysplastic syndrome (MDS) = heterogeneous group of clonal disorders of hematopoiesis. Characterized by: hyper- or hypocellular bone marrow; abnormal cell morphology; abnormal maturation; peripheral blood cytopenias; ineffective blood cell production. All three myeloid cell lineages (erythrocytic, granulocytic, and megakaryocytic) may be involved. Considered a premalignant condition as some patients progress to AML. May present with: anemia, thrombocytopenia, neutropenia. Diagnostic approach includes: complete blood count with differential, peripheral blood smear, bone marrow studies. Treatment: supportive, including transfusions; possible bone marrow stimulation; cytotoxic chemotherapy; bone marrow transplantation has a limited role. May be caused by exposure to: cytotoxic chemotherapy; radiation; viral infection; genotoxic chemicals (e.g., benzene). May also be no known exposure. Cytogenetics include: normal karyotype; balanced chromosomal abnormality and generation of fusion oncogenes; complex karyotypes. Primarily affects the elderly. French-American-British classification: refractory anemia (RA); RA with ringed sideroblasts (RARS); RA with excess blasts (RAEB; 6-20% myeloblasts); RAEB in transition to AML (RAEB-T; 21-30% myeloblasts); chronic myelomonocytic leukemia. 2008 WHO classification: refractory cytopenia with unilineage dysplasia; RARS; RAEB; refractory cytopenia with multilineage dysplasia; MDS with isolated deletion of 5q; MDS, unclassifiable; childhood MDS |
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| prefLabel | Myelodysplastic syndrome (SMQ)
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| SMQ STATUS | A
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| type | |
| tui | T185
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| notation | 20000217
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| SMQ LEVEL | 1
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| Semantic type UMLS property | |
| SMQ ALGO | N
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| Scope Statement | NOTE: SMQ Myelodysplastic syndrome focuses on cases likely to represent this condition; hence, broad concepts that could represent findings in MDS - e.g., leukopenia - but could also represent a host of unrelated conditions are specifically excluded from this SMQ. The user may wish to apply Myelodysplastic syndrome (SMQ) initially to search for cases of interest; the user may optionally wish to also apply Haematopoietic cytopenias (SMQ) to extend the range of cases that represent cytopenias and that may also describe forms of MDS.
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| cui | C3666007
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| SMQ SOURCE | Besa, EC, Woermann, UJ, Krishnan, K. Myelodysplastic syndrome. eMedicine, 10 October 2011; http://emedicine.medscape.com/article/207347-overview. Tefferi, A and Vardiman, JW. Myelodysplastic syndromes. N Engl J Med 2009, 361(19): 1872 - 1885
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