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Different Types of Leukemias, Lymphomas, and Myelomas
Published in Tariq I Mughal, John M Goldman, Sabena T Mughal, Understanding Leukemias, Lymphomas, and Myelomas, 2017
Tariq I Mughal, John M Goldman, Sabena T Mughal
A prognostic scoring has been proposed by the International Myelodysplastic Syndrome Risk Analysis Workshop (Table 4.9). It attempts to assign a score in accordance to the clinical and biological features. Patients are assigned a score based upon the cytogenetic abnormalities, the percentage of blasts in the marrow and the number of lineages affected in the cytopenia and then stratified into four prognostic groups: low score (median survival 5.7 years), intermediate score subgroup 1 (median survival 3.5 years), intermediate score subgroup 2 (median survival 1.2 years), and high score (median survival 0.4 year). This system appears to have gained international approval and should prove useful. Among patients with MDS, those with certain associated or underlying genetic events appear to have a unique natural history such that it is sometimes convenient to consider them as separate clinical entities. These include the 5q– syndrome, which is characterized by a relatively indolent clinical course.
Cytogenetics
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Deletion of 5q occurs in MDSs and AMLs [14,28–35]. Patients with MDS and del(5q) can be grouped into those with isolated del(5q) (good prognosis), del(5q) with additional abnormality [prognosis worse than for isolated del(5q)], and del(5q) with complex karyotype (poor prognosis). The 5q− syndrome is a distinct category of MDS defined by less than 5% blasts, isolated deletion of the long arm of chromosome 5, and low probability of transformation to AML [29,30,32–37]. MDS cases with 5q− plus additional abnormalities, including del(12p), del(7q), del(14q), i(11q), and i(17q), show a neoplastic evolution in a short period of time [38]. Patients with MDS associated with del(5q) respond well to lenalidomide treatment, although the target for the drug is unknown.
CHRONIC LEUKAEMIAS AND MYELODYSPLASTIC DISEASE
Published in James Bishop, Cancer Facts, 1999
o 5q- syndrome: While the 5q- karyotype is not specific for MDS, when seen it defines patients with a syndrome characterised by macrocytic anaemia, leucopenia, and normal or raised platelet count with uncommon progression to leukaemia.
VEXAS: is it time to reshape the nosology of clonal hematopoiesis?
Published in Expert Review of Hematology, 2023
Pierre Sujobert, Laetitia Largeaud, Yvan Jamilloux, Maël Heiblig, Olivier Kosmider
Nosology, like all classification strategies, attempts to find an optimal trade-off between recognizing singularities or similarities. If one pays attention mostly to singularities, the classification will recognize many different entities (with low number of patients in each class), and this can be problematic for the recruitment of patients in clinical trials. On the other hand, if the recognition of similarities is the guiding principle of classification, patients with different conditions could be wrongly recognized as similar. This may also hamper clinical and therapeutic research by masking positive effects that may be limited to an unrecognized subgroup. This tension in classification principles also concerns MDS. Hence, after the creation of the MDS entity (based on the recognition of similarities), subtypes were formally recognized by the different updates of the WHO classification (based on the recognition of singularities) [17–19]. Some of these subtypes such as 5q-syndrome or refractory anemia with ring sideroblasts were supported by specificities at the cytological, cytogenetic, and/or molecular levels and were validated by specific responses to therapies (such as lenalidomide in 5q-) [20]. The latest version of the WHO classification confirmed the consensus on these categories [15]. However, these refinements have not called into question the fundamental definition of what an MDS is.
Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia
Published in Hematology, 2021
Huaquan Wang, Haiyue Niu, Tian Zhang, Limin Xing, Zonghong Shao, Rong Fu
Our study found that there were 6 low- and intermediate-risk MDS patients with PRCA, 1 male and 5 females, with a median age of 63.5 (50-75) years. It accounted for 7.7% (6/78) of all diagnosed PRCA cases and 1.67% (6/359) of diagnosed MDS cases during the same period. All patients had severe anemia and were red blood cell (RBC) transfusion dependence. The median RBC transfusion was 4 (2-8) U /8 weeks. The reticulocytes are severely reduced. The number of white blood cells and platelets were normal. The bone marrow erythroid precursors were severely reduced, with a median of 1.25 (0.5-2.0) %. Serum erythropoietin (EPO) and ferritin levels were significantly increased. One patient had a 5q- and 20q- chromosome karyotype, and one patient had a 20q-chromosome karyotype during follow-up, one patient had 1q21. TET2, GATA2, KRAS and DNMT3A mutations were found in the patients. None of the 6 patients was positive for STAT mutations. According to the WHO 2016 MDS diagnostic criteria, one patient was 5q syndrome, and five patients had MDS-MLD (Tables 1 and 2).
The clinical characteristics and therapy response of patients with acquired pure red cell aplasia
Published in Hematology, 2018
Rong Fu, Tian Zhang, Bingnan Liu, Jia Song, Guojin Wang, Lijuan Li, Huaquan Wang, Limin Xing, Yuhong Wu, Jing Guan, Zonghong Shao
The hemoglobin level, erythrocyte and reticulocyte counts decreased obviously in all patients with acquired PRCA, while leukocytes, PLT, mean corpuscular volume and mean corpuscular hemoglobin concentration were normal. Although most patients had hyperplasia marrow (sternum: 86.36% of patients, iliac: 78.69% of patients), erythroid progenitor was absence in all patients. These data were lower in secondary PRCA compared with idiopathic PRCA (Table 1). Bone marrow biopsies showed the same results as the bone marrow smears. One case had mild fiber hyperplasia. Chromosome karyotype analysis was performed in 40 patients, and all of results were normal except one patient who developed to 5q-syndrome after a year. The 20/30 patients (66.7%) had iron overload (extracellular iron grade ≥2+) examined by bone marrow iron stain. Ringed sideroblasts were found in 1 patient. No positive finding in staining for glycogen (PAS) of nucleated red blood cells.