Background: This study aimed to evaluate the clinical and prognostic associations of the systemic inflammatory index (SII) in polycythemia vera (PV) patients. SII integrates information on absolute neutrophil (ANC), lymphocyte (ALC), and platelet counts into one index (calculated as ANCxALC/platelet count) and was previously shown to predict thrombotic and mortality risks in the general population. Methods: A total of 279 PV patients treated in several hematologic centers in Croatia and Serbia was retrospectively evaluated. Results: The median SII for the overall cohort was 1960. Higher SII stratified at the specific cut-off points was significantly associated with shorter time to thrombosis (TTT; p = 0.004) driven by arterial thrombotic events, and shorter overall survival (OS; p < 0.001). Higher SII was able to refine the European Leukemia Net-defined high-risk patient subgroup for both thrombotic and survival risks, especially in individuals over 60 years of age. SII and all other evaluated CBC components and indices (leukocytes, ANC, ALC, platelets, neutrophil to lymphocyte ratio (NLR), and platelet to lymphocyte ratio (PLR)) demonstrated low-to-modest prognostic properties, whereas SII outperformed other parameters with respect to TTT and OS prognostications. Discussion: The presented results complement prior studies evaluating the prognostic performance of different CBC components for thrombotic and survival risk predictions and offer more options to personalize PV treatments.

Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation with thrombocytosis. The aim of our study was to determine significant clinical-laboratory parameters at presentation to differentiate prePMF from ET as well as to develop and validate a predictive diagnostic prePMF model. This retrospective study included 464 patients divided into ET (289 pts) and prePMF (175 pts) groups. The model was built using data from a development cohort (229 pts; 143 ET, 86 prePMF), which was then tested in an internal validation cohort (235 pts; 146 ET, 89 prePMF). The most important prePMF predictors in the multivariate logistic model were age ≥ 60 years (RR = 2.2), splenomegaly (RR = 13.2), and increased lactat-dehidrogenase (RR = 2.8). Risk scores were assigned according to derived relative risk (RR) for age ≥ 60 years (1 point), splenomegaly (2 points), and increased lactat-dehidrogenase (1 point). Positive predictive value (PPV) for pre-PMF diagnosis with a score of ≥points was 69.8%, while for a score of ≥3 it was 88.2%. Diagnostic performance had similar values in the validation cohort. In MPN patients with thrombocytosis at presentation, the application of the new model enables differentiation of pre-PMF from ET, which is clinically relevant considering that these diseases have different prognoses and treatments.

Introduction: Myeloproliferative neoplasms (MPN) are typically diagnosed in patients around 60 years of age; however, in clinical practice, patients <40 years are often encountered. Aim: Assessment of clinical-laboratory characteristics, thrombosis incidence, and therapeutic approaches in patients with MPN 18 cm. This indicates the need for better stratification and the use of different methods for disease risk assessment, such as next-generation sequencing (NGS).

Background: The prevalence and the prognostic impact of chronic obstructive pulmonary disease (COPD) and asthma in patients with myeloproliferative neoplasms (MPNs) are unknown. Methods: This retrospective multicenter cohort analyzed the prevalence and prognostic implications of COPD and asthma in 246 patients with essential thrombocythemia (ET) and polycythemia vera (PV). Results: A total of 6.5% and 1.6% patients had COPD or asthma, respectively, without statistically significant differences with respect to disease phenotype. The presence of COPD/asthma was more frequently associated with active/prior smoking (p = 0.021) and constitutional symptoms (p = 0.001). After the median follow-up of 47.5 months, the presence of COPD/asthma was univariately associated with an inferior time to thrombosis (TTT; median 135 vs. 190 months, 95% confidence interval (CI) 1.8–29.5, hazard ratio-HR 7.75, p = 0.005), mainly driven by venous (HR 37.3, 95% CI 3.2–43.6, p = 0.003) and not arterial events (HR 1.77, 95% CI 0.40–7.78, p = 0.445, p = 0.445). Statistically significant interactions existed between COPD/asthma, female sex (HR 3.94, 95% CI 1.01–11.02), ET phenotype (HR 7.1, 95% CI 15.3–16.7), JAK2 positive status (HR 4.17, 95% CI 1.04–6.9), hydroxyurea use (HR 4.67, 95% CI 1.10–7.43), and the presence of other cardiovascular risk factors (HR 8.1, 95% CI 1.55–10.72) with overall thrombotic risk (interaction p < 0.050 for all analyses). Finally, the negative effect of COPD/asthma on TTT persisted in the multivariate analysis (HR 6.54, p = 0.010) independently of older (>60 years) age (p = 0.030) when being adjusted for other clinically meaningful variables. There was no effect of COPD/asthma on overall survival. Conclusions: These results provide an important signal regarding the potentially inferior outcomes in ET/PV patients presenting with these common respiratory disorders and may help to further personalize MPN management.

Background: The occurrence of myeloproliferative neoplasms (MPNs) that evolve into each other is well-described, as is this occurrence of lymphoproliferative neoplasms (LPNs). However, less is known about rare MPN/LPN coexistence, and the aim of our study was to analyze charachteristics of these patients after long term follow-up. Methods: Fourteen patients with MPN/LPN coexistence were diagnosed and treated according to guidelines at a single university center across two decades. Results: The overall median age was 53 years (22–69). MPNs patients with subsequent LPNs had a shorter period of second malignancy development and a more aggressive course of LPN, which can cause fatal outcomes. Polycythemia vera and chronic lymphocytic leukemia were most commonly associated (36%). The JAK2V617F mutation had 2/3 and cytogenetic abnormalities occurred in 1/3 of patients. MPN/LPN coexistence cases had significantly higher thrombotic potential (42.8%) and a higher third malignancy accruement frequency (21.4%) versus those without such malignancies. Conclusions: Considering the younger ages at MPN diagnosis, it is recommended to check regularly for blood lymphocytosis or lymphadenopathy occurrences and organomegaly progression faster than expected for MPN, with the aim of timely LPN diagnoses. The presence of molecular-cytogenetic abnormalities in a majority of patients indicate possible genetic instability and increased risk of development of multiple neoplasms, thus elevating thrombotic risk.

Introduction. Thymic hyperplasia is a rare condition caused by increase in cellular thymic mass, and in some cases is associated with pure red cell aplasia (PRCA). Case report. The patient, an 18-year-old female, was referred to hematologist due to severe transfusion dependent anemia. After full hematological work up included bone marrow biopsy a diagnosis of PRCA was made. Immunological and virological analyses were unremarkable. With history of myasthenia gravis in family and known association between this condition and thymoma, a chest magnet resonance imaging was performed revealing thymic hyperplasia, with successful clinical outcome after total thymectomy using video-assisted thoracic surgery approach. Pathohistological examination confirmed thymus hyperplasia with a simple intrathymic cyst. Following thymectomy, after 3 months the patient's hemoglobin values normalized which still persist. Conclusion. Acquired thymic hyperplasia is associated with the occurrence of autoimmune disorders. Our findings support the role of thymectomy in PRCA associated with thymic hyperplasia, but further research is needed to optimize management strategies for this rarity.