Stocktaking: Blood Research Selected on May 21, 2020

1Non-myoglobin IIA mutation and MYH9-related thrombocytopenia
https://doi.org/10.1182/blood.2019003064cytoblasts (MKs) are precursor cells of platelets, non-muscular myoglobin II (MYH9) mutations can lead to macroplateplate reduction, characterized by a decrease in the number of platelets, resulting in a decrease in the volume of clotting disorders, called clotting disordersMyoglobulin 9-Related Disorders (MYH9-RD)researchers studied the distribution of MK in BM, matrix derivative factors (SDF) through myH9-RD mouse model (NMIIAR702C/-GFP----), NMIIAD1424N-/-and-NMIIAE1841K--) and in vitro experiments 1 of the chemokines, NMIIA activity and double wire assemblyThe results showed that different MYH9-RD mutations inhibited mk migration in BM and did not affect the formation of double filaments, but caused different viscosity patterns and NMIIA shrinkage activity, depending on the mutation2: AML patients carry high-frequency RUNX1 embryo mutationhttps://doi.org/10.1182/blood.2019003357RUNX1 mutations are found in about 10% of adult AMLAlthough most OF THE RUNX1 MUTATIONS IN THIS DISEASE ARE THOUGHT TO BE ACQUIRED LATERTHAN ALBEIT, THEY MAY ALSO BE GERM LINE MUTATIONSIn fact, the blastocyst RUNX1 mutation causes autosomal dominant familial platelet disease, which is prone to hematologic malignancies (RUNX1-FPD, FPD/AML, FPDMM), of which about 44% of patients may develop AML or bone marrow hyperplasia syndromeThe researchers used Leucegene RUNX1 AML patient cohorts to study the ratio of embryos to acquired RUNX1 mutationsMolecular analysis showed that in AML, which carries RUNX1 embryo mutations, NRAS mutations and other known mutations in the genes that activate various signaling pathways were more frequentIn addition, two patients with RUNX1 and CEBPA germ mutations (mother and son) developed the disease at the age of 59 and 27 at the same time (AML), respectively the predictive model of initial treatment in early CLL patients https://doi.org/10.1182/blood.2019003453 most patients with chronic lymphocytic leukemia (CLL) are diagnosed at an early stage and treated with active monitoring The individual course of disease in early CLL patients is heterogeneous, and it is difficult to predict the probability of treatment at the time of diagnosis recently researchers developed an international prognosis score (IPS-E) to predict the first treatment time (TTFT) for early asymptomatic diseases in Patients with CLL The study analyzed data from 4,933 early CLL patients in 11 international queues to establish and validate prognosis scores The three covariates are consistent lysing in a consistent and independent correlation with TTFT: the unmutated IGHV gene, the absolute number of lymphocytes , 15 x109/l, and the apparent lymph nodes IPS-E is the sum of these three covariates (1 point each) and separates patients with low risk (0 points), medium risk (1 point) and high risk (2-3 points), showing significantly different TTFT Nine queues rated by the Binet system and one queue rated by the Rai system verified the accuracy of the rating The c index for the training series is 0.74 and the verification series is 0.70 A meta-analysis of training and validation queues showed that the cumulative risk of five-year starting treatment in low- and medium-risk and high-risk patients was 8.4%, 28.4% and 61.2%, respectively 4 HSCT treatment and long-term prognostic in children with malignant blood diseases
https://doi.org/10.1182/blood.2019003858 researchers recently compared the prevalence of symptoms, health-related quality of life (HRQOL) and risk factors in adult survivors of hetic systemic malignancies treated with HSCT in childhood with those in the general treatment and non-cancer control group from St Survivors of childhood blood system malignancies in the Jude Lifetime Queue Study (HSCT group: 112 (70% allogeneic, 30% self-enbeing; routine treatment group: 1106) and non-cancer control (242) completed questionnaires assessing the total score of 10 symptom domains and SF-36 HRQOL Chronic health conditions (CHCs) were verified through clinical evaluation The multivariate logic regression analysis showed that HSCT survivors had significantly higher rates of sensory, motor, lung and memory symptoms and lower physical HRQOL scores than non-cancer control groups The prevalence of all symptoms of HSCT and routine treatment survivors was similar to the HRQOL score, but the cumulative prevalence of specific symptoms in HSCT survivors was significantly higher than the risk of re-vision, dry eyes and visual difficulties when wearing glasses In addition to pain and anxiety, organ-specific CHCs were significantly associated with a high prevalence of all symptoms in adult survivors of childhood cancer 5: on the characteristics of multiple myeloma mild chain cast kidney disease https://doi.org/10.1182/blood.2019003807 multiple myeloma mild-chain cast kidney disease (LCCN) often leads to severe, irreversible acute kidney injury Severe kidney damage affects the distribution of chemotherapy, its tolerance, and the survival of the patient Whether the results of kidney biopsy are helpful for clinical evaluation and prediction of the kidneys and prognosis in PATIENTs with LCCN are uncertain researchers conducted retrospective analysis of 178 biopsy-confirmed LCCN patients from 10 centres in Europe and North America, clinical performance, chemotherapy protocols, blood reactions, kidneys, and patient prognosis A detailed pathological review and analysis, including the degree of light-chain protein deposition, was carried out to study the correlation with initial performance and prognosis The patient's median eGFR was 13 x 11 mL/min/1.73 m2, and 82% of patients had stage 3 acute kidney injury The cortical region is deposited on average at 3.2/mm2 Interstitial lesions of the renal tube are common: acute renal tube injury (94%), renal nephritis (82%), rupture of the renal tube (62%), cytocellular reaction (60%), cortical and myelin inflammation (95% and 75%) Myelin inflammation, cytoblastic response and deposition are associated with eGFR at the time of LCCN diagnosis The median follow-up was increased to an average of 43 to 30 mL/min/1.73m2 over a 22-month follow-up period Age, beta-2-microglobulin, optimal blood response, number of deposition cortical/area, and degree of interstitial fibrosis/tube atrophy (IFTA) were all associated with higher eGFR independence during follow-up The eGFR value is associated with overall survival and not with blood reaction Source: MedSci Original