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Paper 1
Published in Aalia Khan, Ramsey Jabbour, Almas Rehman, nMRCGP Applied Knowledge Test Study Guide, 2021
Aalia Khan, Ramsey Jabbour, Almas Rehman
The IPSS is an objective measure of symptoms to grade severity (mild, moderate and severe) and is very helpful when assessing symptoms of benign prostatic hypertrophy (nocturia, double voiding, urgency, frequency, straining) in deciding whether a referral to the urologist is warranted. More information on www.eguidelines.co.uk
Leukemias
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
The 1997 International Prognostic Scoring System (IPSS), based on the karyotype, blast percentage, and number of cytopenias, served well until 2012, when it was revised.125 The revised IPSS (IPSS-R) stratified patients into five prognostic groups, based on cytogenetics but not molecular characteristics (Table 28.15), and was broadly accepted.126 It is also better able to capture fatigue severity compared with IPSS.127 Refinements are in progress to integrate validated genetic and immunologic information to further improve prognostic discrimination and probability of response to specific therapy, including allo-SCT.122 As an illustration, mutations in TP53, RUNX1, and ASXL1 predict for poor prognosis, mutations involving SF3B1 are associated with favorable prognosis, and mutations involving PPM1D may inform of risk of relapse from allo-SCT. Indeed, research has shown that clinical outcomes of patients with TP53 mutated MDS are heterogenous and affected by other factors.128
Myelodysplastic Syndromes and Myelodysplastic/Myeloproliferative Neoplasms
Published in Tariq I. Mughal, Precision Haematological Cancer Medicine, 2018
Historically the widely adopted International Prognostic Scoring System (IPSS), was proposed by Peter Greenberg in 1997; and based on the karyotype, blast percentage and the number of cytopenias, and permitted the stratification of MDS into four prognostic groups. It was revised in 2012 to expand the number and weighted impact of cytogenetic abnormalities, blast percentages and severity, as well as number of cytopenias. This revised IPSS (IPSS-R) stratifies patients into five prognostic groups (Table 8.2); however, it does not incorporate molecular characteristics. Refinements are now in progress to integrate validated genetic and immunologic information to further improve prognostic discrimination and probability of response to specific therapy, including allo-SCT. It is likely that the new prognostic models will include mutations in TP53, EZH2, ETV6, RUNX1, ASXL1 and SRSF2 to predict for poor prognosis, and those in SF3B1 to be associated with favourable prognosis. Mutations of TP53 are the strongest adverse risk factors even in patients with complex karyotypes. The clinical utility of mutations in TP53 and its associated regulator, PPM1D, have also been found to inform for risk of relapse from allo-SCT and the selection of a conditioning regimen.
The prognostic role of lymphocyte to monocyte ratio (LMR) in patients with Myelodysplastic Neoplasms
Published in Hematology, 2023
Chuanyang Lu, Qiuni Chen, Jiaxin Li, Chunling Wang, Liang Yu
MDS is a heterogeneous group of clonal hematopoietic stem-cell disorders characterized by ineffective and dysplastic hematopoietic differentiation and variable risk that progressed to acute myeloid leukemia. The fifth edition of the WHO Classification replaced myelodysplastic syndromes with myelodysplastic neoplasms and emphasized MDS as neoplastic disease. According to the National Cancer Institute, MDS patients did not have a significantly higher five-year survival rate than AML patients (36.9% versus 30.5%). The most widely used prognostic scoring systems for MDS today are IPSS-R [19]. However, it has a few limitations. For example, the 5th edition of the WHO Classification emphasized that MDS is a genetically-defined disease type [2]. However, factors associated with gene mutations were not included in the IPSS-R. Recently, a new clinical molecular prognostic model incorporating somatic gene mutations has been developed under the name IPSS-M. However, neither IPSS-R nor IPSS-M included lymphocyte and monocyte counts, which were demonstrated to have predictive prognostic value in cancer patients. LMR has been demonstrated prognostic role in patients with solid tumors in the past few years. Besides, existing research focuses on the predicting value of LMR for hematologic malignancies such as MM and DLBCL [15,16]. However, the prognostic significance of LMR in MDS was unclear. Thus, the retrospective study was conducted to investigate the correlation between LMR and the prognosis of MDS patients.
How low risk are low risk myelodysplastic syndromes?
Published in Expert Review of Hematology, 2022
Amy E. DeZern, William Brian Dalton
The IPSS-R captures the clinical prognostic variables of number and depth of cytopenias and bone marrow blasts, but there is evidence that other clinical features are relevant to progression in MDS (Table 2). First, red blood cell transfusion dependence (RBC-TD) was found to be an independent predictor of poor prognosis by the developers of the WPSS [10]. Subsequently, RBC-TD was shown to add prognostic value to the IPSS-R [27]. It is interesting to consider why RBC-TD would be prognostic independent of hemoglobin levels, given the latter largely triggers the former. One answer is that it won’t, if lower hemoglobin thresholds are simply incorporated into scoring [16]. Another possibility is that RBC-TD may serve as a surrogate for other medical comorbidities that influence outcomes [10,28]. It is also possible that RBC-TD reflects a patient-specific property distinct from the mere presence of severe anemia, a sort of ‘anemia-tolerance index’ that bears on prognosis. Whatever the mechanism, RBC-TD is a feature than can upstage an otherwise IPSS-R lower-risk patient.
Low serum albumin level deteriorates prognosis in azacitidine-treated myelodysplastic syndromes patients – results of the PALG study ‘PolAZA’
Published in Hematology, 2021
Krzysztof Mądry, Karol Lis, Andrzej Tukiendorf, Paweł Szwedyk, Katarzyna Kapelko-Słowik, Edyta Subocz, Aleksandra Gołos, Wioletta Makowska, Anna Masternak, Anna Kopińska, Magdalena Czemerska, Sara Zawadzka-Leska, Patrycja Rusicka, Joanna Drozd-Sokołowska, Elżbieta Wiater, Jadwiga Hołojda, Bartłomiej Pogłódek, Piotr Centkowski, Anna Waszczuk-Gajda, Rafał Machowicz, Janusz Hałka, Tomasz Czerw, Grzegorz Basak, Jadwiga Dwilewicz-Trojaczek
We collected data on 315 patients treated with azacitidine: AML with 20–30% bone marrow blasts, n = 66 (21%); MDS, n = 210 (67%); CMML, n = 39 (12%) (Table 1). Median age was 69 years (54 years in patients referred for allo-SCT), 61% were male. Patients with available IPSS were in the intermediate-2 (58%) and high (42%) risk categories. In 12% of patients, IPSS was not precisely determined due to lack of one out of three mandatory components. Median duration of MDS/AML/CMML before the start of azacitidine treatment was 5 weeks, 18% of patients had previously received chemotherapy – low-dose cytarabine. Among 276 (88%) patients with available cytogenetic results, 113 (41%) had a normal karyotype. The IPSS-R cytogenetic risk included 7 (2%) patients with very good risk, 130 (47%) patients with good risk, 55 (20%) patients with intermediate risk, 64 (23%) with poor risk and 20 (7%) patients with very poor risk. The most frequent cytogenetic abnormalities were: complex karyotype in 57 (21%), monosomal and complex karyotype in 44 (16%), isolated trisomy 8 in 15 (5%), isolated del 5 in 6 (2%), isolated del 7 in 7 (2%), isolated monosomy 7 in 8 (3%) and deletion 20 in 7 (2%).