Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty patients with Myelodysplastic Syndrome (MDS) were diagnosed in University Hospital, Kuala Lumpur over a 5 year period. They were subclassified using the French American British (FAB) criteria. 90% of the patients were above 40 years old and the sex ratio was about equal. The predominant presenting symptom was anaemia and there was paucity of physical signs at presentation. Patients with 'aggressive' subtypes of MDS i.e. refractory anaemia with excess blasts (RAEB), refractory anaemia with excess blasts in transformation (RAEB(-)+) and chronic myelomonocytic leukaemia (CMML) had more frequent thrombocytopenia and neutropenia and their marrow pictures frequently had dysmegakaryopoiesis and dysgranulopoiesis as compared to more the "benign" subtypes i.e. refractory anaemia (RA) and refractory leukaemic anaemia with ringed sideroblasts (RARS). Four patients had leukaemic transformation and all of them came from the 'aggressive' subtypes. The current views on treatment of MDS are discussed.
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PMID:Myelodysplastic syndrome: a review from University Hospital, Kuala Lumpur. 219 85

Recombinant human erythropoietin (r-EPO) has been used in Myelodysplastic Syndrome (MDS) patients with anaemia since the early nineties. In low-risk MDS patients, other haemopoietic growth factors (HGFs) (granulocyte-colony stimulating factor, G-CSF, granulocyte-macrophage-colony stimulating factor, GM-CSF, and interleukin 3, IL-3) have been used to synergise the effects of r-EPO on erythroid growth and to increase neutrophil count in patients with severe neutropenia. In high-risk MDS, or in patients with post-MDS AML, myeloid HGFs have been used to push blasts into the S-phase, thus increasing their sensitivity to antiblastic drugs. Several trials have shown that r-EPO can increase haemoglobin levels and improve QoL in patients with anaemia associated to MDS. The selection of patients with a high probability of response to HGFs is based on the careful consideration of several clinical and biological parameters, i.e., among others, basal EPO and transfusional needs, disease duration, FAB or WHO subtypes, and IPSS score. Treatment of anaemic MDS patients with HGFs should become "patient oriented" and different types, schedules, and duration of treatment have to be designed according to the specific criteria which most likely predict, for each individual patient, the best chance of responding favourably to therapy.
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PMID:Haemopoietic growth factors in myelodysplastic syndromes: towards patient-oriented therapy? 1594 26

In comparison with the 2008 World Health Organization "Blue Book" on hematopoietic neoplasms, a small number of changes have been made in the classification. In the lower-risk patients, Refractory Cytopenias with Multilineage Dysplasia and Ring Sideroblasts (RCMD-RS) has been separated from RCMD to recognize the importance of the SF3B1 mutation. Often there has been confusion as to the degree of morphologic dysplasia and/or cytopenias to define some of the lower-risk subtypes. In addition, the type of dysplasia or cytopenias is not always concordant. Therefore, it seems prudent to apply the more general term "myelodysplastic syndrome (MDS)" with single- or multiple-lineage dysplasia. Refractory neutropenia or thrombocytopenia has been deemphasized because most patients have multilineage dysplasia. In the higher-risk patients, the terms Refractory Anemia with Excess Blasts (RAEB) 1 or 2 have been simplified to Myelodysplastic Syndrome-Excess Blasts (MDS-EB) 1 or 2 to emphasize the importance of the percentage of blasts that dictate therapy. Patients with Chronic Myelomonocytic Leukemia (CMML) can be classified into 3 groups: CMML 0, 1, or 2, based on the percentage of blasts (< 5%, 5%-9%, 10%-19%). Cases with 50% or more erythroid precursors will not have the percentage of blasts based on the nonerythroid precursors. Previous cases of acute erythroid leukemia (myeloid:erythroid type) can be included in the acute myeloid leukemia (AML) subtype: AML with MDS-related features.
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PMID:Changes in the Updated 2016: WHO Classification of the Myelodysplastic Syndromes and Related Myeloid Neoplasms. 2769 33