Polycythemia Vera Presenting As Acute Non-hemorrhagic Infarction and Chronic Bleed: Deception Between the Lines.

Patients with polycythemia vera (PV) develop various complications due to hyper-viscous blood, causing events such as ischemic stroke. There are other associated complications due to the dysfunction of platelet activity, causing hemorrhages. In our unusual case, we present a patient who came to the OPD complaining of slurring speech. An MRI was done and was suggestive of acute lacunar infarcts with changes in chronic bleed. CBC and hematocrit were consistent for PV, with the genetic marker JAK2 being positive.

Acute effects of apnea bouts on hemoglobin concentration and hematocrit: a systematic review and meta-analysis.

Objective: This study aimed to systematically analyze the existing literature and conduct a meta-analysis on the acute effects of apnea on the hematological response by assessing changes in hemoglobin (Hb) concentration and hematocrit (Hct) values. Methods: Searches in Pubmed, The Cochrane Library, and Web of Science were carried out for studies in which the main intervention was voluntary hypoventilation, and Hb and Hct values were measured. Risk of bias and quality assessments were performed. Results: Nine studies with data from 160 participants were included, involving both subjects experienced in breath-hold sports and physically active subjects unrelated to breath-holding activities. The GRADE scale showed a "high" confidence for Hb concentration, with a mean absolute effect of 0.57 g/dL over control interventions. "Moderate" confidence appeared for Hct, where the mean absolute effect was 2.45% higher over control interventions. Hb concentration increased to a greater extent in the apnea group compared to the control group (MD = 0.57 g/dL [95% CI 0.28, 0.86], Z = 3.81, p = 0.0001) as occurred with Hct (MD = 2.45% [95% CI 0.98, 3.93], Z = 3.26, p = 0.001). Conclusions: Apnea bouts lead to a significant increase in the concentration of Hb and Hct with a high and moderate quality of evidence, respectively. Further trials on apnea and its application to different settings are needed.

Impact of Plateletpheresis on the Hemoglobin, Hematocrit, and Total Red Blood Cell Count: An Updated Meta-Analysis.

Plateletpheresis has become a pivotal part of transfusion medicine. With the increasing demand for plateletpheresis, donor safety is an area of concern because plateletpheresis alters donor hematological parameters. For a better understanding of plateletpheresis, a systemic review is needed to study more evidence-based aspects of plateletpheresis. Electronic databases PubMed, Google Scholar, and Cochrane Library were used to find articles from January 1, 1980, to May 23, 2024. The random effect model was used to meta-analyze the effect of plateletpheresis on hematocrit, hemoglobin, and red blood cell (RBC) count. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline was followed. A total of 24 studies were found; the effect of plateletpheresis on hemoglobin, hematocrit, and RBC count was studied in the following respective numbers of donors: 3,374, 3,374, and 690. A decrease of hemoglobin, hematocrit, and RBC count was observed after plateletpheresis having a weighted mean difference (WMD) of 0.50 (95%CI = -0.72 to -0.27), WMD of -1.36 (95%CI = -2.05 to -0.66), and WMD of -0.18 (95%CI = -0.23 to -0.12), respectively. Plateletpheresis shows a decrease in the value of hematological parameters such as hemoglobin, hematocrit, and erythrocyte count due to blood loss in the kits employed in the procedure; cell lysis was also seen because of exposure of erythrocytes to stress or change in osmotic pressure. Thus, strict criteria for donation must be developed for better safety of the donors. Improved automated cell separators for plateletpheresis should be made available in blood banks to ensure good quality hematologic products. Our findings suggest that the duration of the procedure should be decreased.

Effect of the Administration of Cordyceps militaris Mycelium Extract on Blood Markers for Anemia in Long-Distance Runners.

In the present study, we conducted a placebo-controlled, double-blind, parallel-group comparison trial in which an extract of Cordyceps militaris (CM) mycelium was administered to long-distance runners for 16 weeks during the pre-season training period and blood test markers for anemia were investigated. The results indicated that the change rates of serum ferritin levels were moderately increased in the CM group (n = 11) but decreased in the placebo group (n = 11) during the study period, and the levels were significantly increased in the CM group compared with those in the placebo group at 4 weeks and 8 weeks after the test food intake (p < 0.05). Moreover, the change rates of hemoglobin and hematocrit were significantly increased in the CM group compared with those in the placebo group at 8 weeks after the test food intake (p < 0.05). These observations suggest that the intake of test food containing Cordyceps militaris mycelium extract is expected to effectively maintain the hemoglobin and hematocrit levels in long-distance runners, possibly via the suppression of the decrease in iron storage, which is reflected by serum ferritin, during pre-season training. Furthermore, the levels of creatine kinase were increased above the normal range in both the placebo and CM groups at registration. Interestingly, the creatine kinase levels were significantly decreased in the CM group compared with those in the placebo group at 16 weeks after the test food intake (p < 0.05). These results suggest that Cordyceps militaris mycelium extract exhibits a protective action on the muscle damage observed in long-distance runners and may suppress muscle injury. Together, these observations suggest that Cordyceps militaris mycelium extract exhibits an improving effect on the markers for not only anemia, but also muscle injury in long-distance runners during pre-season training.

Perioperative Dyselectrolytemia in Patients Undergoing Transurethral Resection of the Prostate Using 0.9% Normal Saline Irrigation.

BACKGROUND: The choice of irrigation fluid used in transurethral resection of the prostate (TURP) has a significant impact on serum electrolyte levels. Among the many available options, 0.9% normal saline (NS) is considered to be more physiological. MATERIAL AND METHODS: This observational study was conducted on 60 adult males aged 50-70 years, classified as American Society of Anesthesiologists grade 1 and 2, undergoing TURP with 0.9% NS irrigation under spinal anesthesia achieved with a mixture of 0.5% heavy bupivacaine. The patients' hematocrit and serum electrolyte levels were obtained after six hours and compared with preoperative values. RESULTS: Hematocrit reduced from 40.32 ± 6.27 to 31.07 ± 5.40 (p < 0.001). Both serum sodium and potassium decreased from 136.77 ± 3.27 to 128.31 ± 5.91 and from 4.02 ± 0.26 to 3.81 ± 0.36, respectively (p < 0.001). However, serum chloride showed only a minimal increase from 101.58 ± 2.88 to 102.25 ± 1.66 (p < 0.12). CONCLUSION: Although the changes in serum sodium and potassium were statistically significant, they did not have any physiological consequences in our study. However, this emphasizes the importance of vigilant electrolyte monitoring to identify and mitigate the risk of electrolyte disturbances during TURP surgeries.

Joint clinical determinants for bivariate hematological parameter among TB/HIV co-infected adults under TB/HIV treatment in university of Gondar comprehensive specialized hospital: Retrospective panel data study.

BACKGROUND: Worldwide ranking above HIV/AIDS, tuberculosis is continues to have a significant effect on public health and the leading cause of death due to high progression of HIV. The objective of current study was identify joint clinical determinants that affecting bivariate hematological parameter among TB/HIV co-infected adults under TB/HIV treatment in university of Gondar comprehensive specialized hospital. METHOD: The result of these study was conducted at university of Gondar comprehensive specialized hospital, Gondar, Ethiopia by using a retrospective cohort follow up study from September 2015-march 2022 G.C. The source of data in this study was secondary data obtained from patients chart. Bayesian approach of longitudinal linear mixed effect sub model was used in panel data set to get wide range of information about TB/HIV co-infected patients. RESULT: Out of 148 co-infected participants more than half of the patients (56.1%) and (52.7%) accounted for CPT and INH non users, of which 10.8% and 10.3% had the outcome of mortality respectively. The random intercept and slope model were selected for repeated measure hemoglobin level and hematocrit based on deviance information criteria (DIC), and probability of direction (Pd) under the full model. CONCLUSION: Current study revealed that clinical predictors red blood cell count, platelet cell count, fair and good treatment adherence, other ART regiment, IPT drug users, and viral load count < 10,000 copies/mL, were associated with high hemoglobin level concentration while, lymphocyte count, WHO clinical stage-IV,1e ART regiment, and patients with OIs results for low hemoglobin level concentration. Likewise, red blood cell count, platelet cell count, fair and good treatment adherence, IPT drug users, and viral load count < 10,000 copies/mL co-infected patients had high hematocrit, while lymphocyte count, WHO clinical stage-III,1c ART regiment, and patients with OIs significantly leads to low hematocrit. Health professionals give more attention to these important predictors to reduce progression of disease when the co-infected patients come back again in the hospital. In addition, health staff should conduct health related education for individuals to examine continuous check-up of co-infected patients.

Diagnostic Accuracy of Hounsfield Unit Value and Hounsfield Unit to Hematocrit Ratio in Predicting Cerebral Venous Sinus Thrombosis: A Retrospective Case-Control Study.

OBJECTIVE: Non-contrast computed tomography (CT) of the brain is a primary neuroimaging modality in emergency patients suspected of having cerebral venous sinus thrombosis (CVST). The objective of the study was to determine the diagnostic accuracy of Hounsfield unit (HU) values and the ratio of HU to hematocrit value (HU/Htc) in predicting CVST in suspected patients. MATERIAL AND METHODS: A retrospective, case-control study was done in a tertiary care institute which included 35 patients with CVST constituted as cases and 41 patients without CVST as controls on the basis of magnetic resonance venography (MRV). Non-contrast CT brain of all 76 subjects were assessed by two experienced radiologists independently. HU values of dural venous sinuses were calculated in both groups, and HU/Htc ratio was also determined. Statistical Package for Social Sciences (SPSS) version 25.0 (SPSS© for Windows, IBM© Corp.) was used for statistical analysis. Independent samples t-test was applied to compare the means of continuous variables. The diagnostic values were computed using the Calculator 1 tool on clinical research calculators tab on vassarstats.net. The predictive values of HU and HU/Htc ratio were estimated by the receiver operating characteristic (ROC) curve analysis. RESULTS: In CVST group, the mean Hounsfield Unit (HU) value was "75.9±3.9 (mean±SD)", while in control group, it was 57.78±4.65 (mean±SD), p < 0.001. The mean HU/Htc ratio was 1.98±0.42 (mean±SD) in the CVST group and 1.51±0.12 (mean±SD) in the control group (p < 0.001). Optimum cut-off HU value was calculated as 68, with 97% sensitivity and 100% specificity. For HU/Htc ratio, optimum cut-off was calculated as 1.69, yielding 71.4% sensitivity and 100% specificity on the basis of ROC curves. The difference was not statistically significant in hemoglobin and hematocrit (Htc) values between the cases and controls. CONCLUSION: The quantitative measurements like HU value and HU/Htc ratio provide an easily obtainable metric in patients with suspected CVST on non-contrast CT brain, thus enhancing the role of non-contrast CT brain in diagnosing CVST.

Hematocrit drift and outcomes in surgical patients with aneurysmal subarachnoid hemorrhage.

BACKGROUND: There is a paucity of conclusive evidence regarding the impact of downward drift in hematocrit levels among patients who have undergone surgical clipping for aneurysmal subarachnoid hemorrhage (aSAH). This study endeavors to explore the potential association between hematocrit drift and mortality in this specific patient population. METHODS: A cohort study was conducted, encompassing adult patients diagnosed with aSAH at a university hospital. The primary endpoint was follow-up mortality. Propensity score matching was employed to align patients based on their baseline characteristics. Discrimination capacity across various models was assessed and compared using net reclassification improvement (NRI). RESULTS: Among the 671 patients with aSAH in the study period, 118 patients (17.6%) experienced an in-hospital hematocrit drift of more than 25%. Following adjustment with multivariate regression analysis, patients with elevated hematocrit drift demonstrated significantly increased odds of mortality (aOR: 2.12, 95% CI: 1.14 to 3.97; P = 0.019). Matching analysis yielded similar results (aOR: 2.07, 95% CI: 1.05 to 4.10; P = 0.036). The inclusion of hematocrit drift significantly improved the NRI (P < 0.0001) for mortality prediction. When in-hospital hematocrit drift was served as a continuous variable, each 10% increase in hematocrit drift corresponded to an adjusted odds ratio of 1.31 (95% CI 1.08-1.61; P = 0.008) for mortality. CONCLUSIONS: In conclusion, the findings from this comprehensive cohort study indicate that a downward hematocrit drift exceeding 25% independently predicts mortality in surgical patients with aSAH. These findings underscore the significance of monitoring hematocrit and managing anemia in this patient population.

Association of iron supplementation, HFE and AMPD1 polymorphisms and biochemical iron metabolism parameters in the performance of a men's World Tour cycling team: A pilot study.

BACKGROUND: Nutritional strategies with iron supplementation have been shown to be effective in preventing the decline of blood biochemical parameters and sports performance. The aim of the study was to describe biochemical iron metabolism parameters in association with iron supplementation and HFE and AMPD1 polymorphisms in a Union Cycliste Internationale (UCI) World Tour cycling team to evaluate performance during a whole season METHODS: Twenty-eight professional men cyclists took part in this longitudinal observational pilot study. AMPD1 c.34 C>T (rs17602729) and HFE c.187 C>G (rs1799945) polymorphisms were genotyped using Single Nucleotide Primer Extension (SNPE). All the professional cyclists took oral iron supplementation throughout the season. Four complete blood analyses were carried out corresponding to UCI controls in January (1st), April (2nd), June (3rd) and October (4th). Data on participation in three-week Grand Tours, kms of competition and wins were analyzed. RESULTS: In performance, especially in wins, there was a significant effect in HFE on biochemical hemoglobin (F = 4.255; p = 0.021) and biochemical hematocrit (F = 5.335; p = 0.009) and a hematocrit biochemical × genotype interaction (F = 3.418; p = 0.041), with higher values in professional cyclist with GC genotype. In AMPD1 there were significant effects in the biochemical iron x genotype interaction in three-week Grand Tours (F = 3.874; p = 0.029) and wins (F = 3.930; p = 0.028) CONCLUSIONS: Blood biochemical iron metabolism parameters could be related to performance in the season due to increasing hemoglobin and hematocrit concentration under iron supplementation, associated with winning in the professional cyclists with GC genotype of the HFE polymorphism.

Optimal hematocrit theory - A review.

In humans and higher animals, a trade-off between a sufficiently high concentration of erythrocytes (hematocrit), to bind oxygen and sufficiently low blood viscosity to allow rapid blood flow has been achieved during evolution. The optimal value lies between the extreme cases of pure blood plasma, which cannot practically transport any oxygen, and 100\% hematocrit, which would imply very slow blood flow or none at all. As oxygen delivery to tissues is the main task of the cardiovascular system, it is reasonable to expect that maximum oxygen delivery has been achieved during evolution. Optimal hematocrit theory, based on this optimality principle, has been successful in predicting hematocrit values of about 0.3-0.5, which are indeed observed in humans and many animal species. Similarly, the theory can explain why higher than normal hematocrit, ranging from 0.5 to 0.7, can promote better exertional performance. Here we present a review of theoretical approaches to the calculation of the optimal hematocrit value under different conditions and discuss them in a broad physiological context. Several physiological and medical implications are outlined, e.g. in view of blood doping, temperature adaptation, and life at high altitudes.

Hematocrit Predicts Poor Prognosis in Patients with Acute Ischemic Stroke or Transient Ischemic Attack.

This study aims to investigate the association between HCT (Hematocrit) levels and adverse outcomes in patients with acute ischemic stroke (AIS) or transient ischemic attack (TIA); 14,832 participants from the China National Stroke Registry-III with AIS or TIA were analyzed. Participants were categorized into quartiles based on baseline HCT levels. The primary outcome was poor functional outcomes (modified Rankin Scale ≥ 3) during three months, with secondary outcomes including all-cause death, stroke recurrence, and combined vascular events. Logistic regression or Cox regression models were used to assess the relationship between HCT and clinical outcomes. Compared to the third quartile, patients in the lowest quartile group showed increased risk of poor functional outcome (adjusted OR: 1.35, 95% CI: 1.15-1.58, p < 0.001), patients in the lowest quartile had a higher risk of all-cause death (adjusted HR: 1.68, 95% CI: 1.06-2.68, p = 0.028), as did those in the highest quartile (adjusted HR: 2.02, 95% CI: 1.26-3.25, p = 0.004). Sensitivity analysis shows that the association of HCT with all-cause death weakened, while the association with poor functional outcome was strengthened after excluding patients with recurrent stroke. Our results indicated that HCT level could be used as a short-term predictor for poor functional outcomes and all-cause death in patients with AIS or TIA.

Effect of Fluid Intake on Acute Changes in Plasma Volume: A Randomized Controlled Crossover Pilot Trial.

Plasma volume (PV) undergoes constant and dynamic changes, leading to a large intra-day variability in healthy individuals. Hydration is known to induce PV changes; however, the response to the intake of osmotically different fluids is still not fully understood. In a randomized controlled crossover trial, 18 healthy individuals (10 females) orally received an individual amount of an isotonic sodium-chloride (ISO), Ringer (RIN), or glucose (GLU) solution. Hemoglobin mass (Hbmass) was determined with the optimized carbon monoxide re-breathing method. Fluid-induced changes in PV were subsequently calculated based on capillary hemoglobin concentration ([Hb]) and hematocrit (Hct) before and then every 10 minutes until 120 min (t0-120) after the fluid intake and compared to a control trial arm (CON), where no fluid was administered. Within GLU and CON trial arms, no statistically significant differences from baseline until t120 were found (p > 0.05). In the ISO trial arm, PV was significantly increased at t70 (+138 mL, p = 0.01), t80 (+191 mL, p < 0.01), and t110 (+182 mL, p = 0.01) when compared to t0. Moreover, PV in the ISO trial arm was significantly higher at t70 (p = 0.02), t110 (p = 0.04), and t120 (p = 0.01) when compared to the same time points in the CON trial arm. Within the RIN trial arm, PV was significantly higher between t70 and t90 (+183 mL, p = 0.01) and between t110 (+194 mL, p = 0.03) and t120 (+186 mL, p < 0.01) when compared to t0. These results demonstrated that fluids with a higher content of osmotically active particles lead to acute hemodilution, which is associated with a decrease in [Hb] and Hct. These findings underpin the importance of the hydration state on PV and especially on PV constituent levels in healthy individuals.

Comparison of hematologic variables between dogs with congenital intrahepatic and extrahepatic portosystemic shunts.

BACKGROUND: Dogs with congenital intrahepatic portosystemic shunt (IHPSS) are predisposed to gastrointestinal inflammation, ulceration, and bleeding, unlike dogs with congenital extrahepatic portosystemic shunt (EHPSS). Limited information is available about hematologic differences between dogs with IHPSS and dogs with EHPSS. OBJECTIVE: Compare hemogram variables between dogs with IHPSS and EHPSS. We hypothesized that hematologic variables would differ between the 2 populations, with a higher frequency and severity of anemia and microcytosis in dogs with IHPSS. ANIMALS: Twenty-six client-owned dogs with IHPSS and 35 client-owned dogs with EHPSS. METHODS: Retrospective cross-sectional study. Dogs were included if a CBC was performed before shunt attenuation. Contingency analysis was performed to determine if the frequency of clinical signs and of hematologic variables below the reference range differed between groups. Hematologic and selected biochemical variables were compared between groups using an analysis of covariance with age as a covariate. RESULTS: Gastrointestinal clinical signs (IHPSS, 81% vs EHPSS, 34%; P = .01), anemia (31% vs 6%; P = .01), microcytosis (77% vs 29%; P = .002), and hypochromia (77% vs 49%; P = .03) were more common in dogs with IHPSS than in dogs with EHPSS. Dogs with IHPSS had lower packed cell volume (34% vs 41%, P = .04), hemoglobin concentration (11.5 g/dL vs 13.7 g/dL, P = .03), mean corpuscular volume (57 fL vs 65 fL; P = .001), and mean corpuscular hemoglobin concentration (32 g/dL vs 33 g/dL; P = .04) than dogs with EHPSS. CONCLUSIONS AND CLINICAL IMPORTANCE: Dogs with IHPSS had a higher frequency of anemia, microcytosis, and hypochromia and exhibited more gastrointestinal clinical signs.

Exogenous erythropoietin increases hematological status, fat oxidation, and aerobic performance in males following prolonged strenuous training.

This study investigated the effects of EPO on hemoglobin (Hgb) and hematocrit (Hct), time trial (TT) performance, substrate oxidation, and skeletal muscle phenotype throughout 28 days of strenuous exercise. Eight males completed this longitudinal controlled exercise and feeding study using EPO (50 IU/kg body mass) 3×/week for 28 days. Hgb, Hct, and TT performance were assessed PRE and on Days 7, 14, 21, and 27 of EPO. Rested/fasted muscle obtained PRE and POST EPO were analyzed for gene expression, protein signaling, fiber type, and capillarization. Substrate oxidation and glucose turnover were assessed during 90-min of treadmill load carriage (LC; 30% body mass; 55 ± 5% V̇O2peak) exercise using indirect calorimetry, and 6-6-[2H2]-glucose PRE and POST. Hgb and Hct increased, and TT performance improved on Days 21 and 27 compared to PRE (p < 0.05). Energy expenditure, fat oxidation, and metabolic clearance rate during LC increased (p < 0.05) from PRE to POST. Myofiber type, protein markers of mitochondrial biogenesis, and capillarization were unchanged PRE to POST. Transcriptional regulation of mitochondrial activity and fat metabolism increased from PRE to POST (p < 0.05). These data indicate EPO administration during 28 days of strenuous exercise can enhance aerobic performance through improved oxygen carrying capacity, whole-body and skeletal muscle fat metabolism.

Impact of etiology, sex, diabetes mellitus and remission status on erythrocytic profile in patients with cushing's syndrome: a large population database study.

PURPOSE: The study aimed to characterize the erythrocytic profile in patients with cushing's syndrome (CS) versus controls from the normal population according to etiology, sex, presence of diabetes mellitus (DM) and hypercortisolemia remission status. METHODS: This retrospective cohort analysis compared erythrocytic parameters between patients with CS of pituitary (CD) and adrenal (aCS) etiology and age, sex, body mass index (BMI) and socioeconomic status-matched controls in a 1:5 ratio. Laboratory values at baseline were calculated as mean values during the year preceding CS diagnosis, and over one year thereafter. RESULTS: The cohort included 397 CS patients (68.26% female; mean age 51.11 ± 16.85 years) and 1970 controls. Patients with CS had significantly higher baseline median levels of hemoglobin (Hgb) (13.70 g/dL vs. 13.12 g/dL [p < 0.0001]) and hematocrit (Hct) (41.64% vs. 39.80% [p < 0.0001]) compared to controls. These differences were observed for both CD and aCS and for both sexes. Patients who attained remission had Hgb and Hct levels comparable to controls (13.20 g/dL and 40.08% in patients with CD and aCS vs. 13.20 g/dL and 39.98% in controls). Meanwhile, those with persistent/recurrent disease maintained elevated levels. Patients with comorbid DM had similar Hgb but higher Hct (p = 0.0419), while patients without DM showed elevated erythrocytic values compared to controls (p < 0.0001). CONCLUSION: Our data illustrates that erythrocytic parameters are directly influenced by glucocorticoid excess as Hgb and Hct are higher in patients with CS, and normalize after remission. We have identified the influence of DM on erythrocytic parameters in patients with CS for the first time.

Contemporary Challenges in Polycythemia Vera Management From the Perspective of Patients and Physicians.

Although polycythemia vera (PV) is a chronic and incurable disease, effective management can allow most patients to maintain functional lives with near-normal life expectancy. However, there remain several inter-related factors that contribute to many ongoing challenges associated with the management of PV, which this review aims to explore. First, as a disease hallmarked by constitutive activation of the JAK/STAT pathway, PV is often accompanied by inflammatory symptoms that negatively impact quality of life. Next, patients often require recurrent therapeutic phlebotomies to maintain their hematocrit below the 45% threshold that has been associated with a decreased risk of thrombotic events. The need to closely monitor hematocrit and perform conditional therapeutic phlebotomies ties patients to the healthcare system, thereby limiting their autonomy. Furthermore, many patients describe therapeutic phlebotomies as burdensome and the procedure is often poorly tolerated, further contributing to quality-of-life decline. Phlebotomy needs can be reduced by utilizing cytoreductive therapy; however, standard first-line cytoreductive options (i.e., hydroxyurea and interferon) have not been shown to significantly improve symptom burden. Collectively, current PV management, while reducing thrombotic risk, often has a negative impact on patient quality of life. As researchers continue to advance towards the goal of developing a disease-modifying therapy for patients with PV, pursuit of nearer-term opportunities to shift the current treatment paradigm towards improving symptoms without compromising quality of life is also warranted, for example, by reducing or eliminating the frequent use of phlebotomy.

Predictors of Progression in a Series of 81 Adult Patients Surgically Managed for an Intracranial Hemangioblastoma: Implications for the Postoperative Follow-Up.

The aim was to identify predictors of progression in a series of patients managed for an intracranial hemangioblastoma, in order to guide the postoperative follow-up modalities. The characteristics of 81 patients managed for an intracranial hemangioblastoma between January 2000 and October 2022 were retrospectively analyzed. The mean age at diagnosis was of 48 ± 16 years. Eleven (14%) patients had von Hippel-Lindau disease. The most frequent tumor location was the cerebellar hemispheres (n = 51, 65%) and 11 (14%) patients had multicentric hemangioblastomas. A gross total resection was achieved in 75 (93%) patients. Eighteen (22%) patients had a local progression, with a median progression-free survival of 56 months 95% CI [1;240]. Eleven (14%) patients had a distant progression (new hemangioblastoma and/or growth of an already known hemangioblastoma). Local progression was more frequent in younger patients (39 ± 14 years vs. 51 ± 16 years; p = 0.005), and those with von Hippel-Lindau disease (n = 8, 44% vs. n = 3, 5%, p < 0.0001), multiple cerebral locations (n = 3, 17% vs. n = 2, 3%, p = 0.02), and partial tumoral resection (n = 4, 18% vs. n = 1, 2%, p = 0.0006). Therefore, it is advisable to propose a postoperative follow-up for at least 10 years, and longer if at least one predictor of progression is present.

Hematocrit control and thrombotic risk in patients with polycythemia vera treated with ruxolitinib in clinical practice.

Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by unregulated red blood cell production resulting in elevated hemoglobin and/or hematocrit levels. Patients often have symptoms such as fatigue, pruritus, and painful splenomegaly, but are also at risk of thrombosis, both venous and arterial. Ruxolitinib, a selective Janus kinase inhibitor, is approved by the US Food and Drug Administration as second-line cytoreductive treatment after intolerance or inadequate response to hydroxyurea. Although ruxolitinib has been widely used in this setting, limited data exist in the literature on ruxolitinib treatment patterns and outcomes among patients with PV in routine clinical practice. We report a retrospective, observational, cohort study of patients treated for PV with ruxolitinib across three US centers (academic and regional practice) from December 2014-December 2019. The study included 69 patients, with a median follow-up duration of 3.7 years (95% CI, 2.9-4.4). Our data demonstrate very high rates of hematocrit control (88% of patients by three months and 89% by six months); few patients required dose adjustments or suspension. No arterial thromboses were observed; however, the follow-up duration does not allow for the generation of meaningful conclusions from this. Three patients had thrombotic events; one was in the setting of a second malignancy, one post-operative, and a third related to prolonged immobility. We also found that 28% of patients initiated ruxolitinib as a result of poorly controlled platelet counts, second only to hydroxyurea intolerance (46%) as a reason to start therapy. In clinical practice, ruxolitinib continues to be effective in controlling hematocrit levels after three and six months of treatment in patients and is associated with low thrombotic risk.

Neuronal nitric oxide synthase required for erythropoietin modulation of heart function in mice.

Introduction: Erythropoietin (EPO) acts primarily in regulating red blood cell production mediated by high EPO receptor (EPOR) expression in erythroid progenitor cells. EPO activity in non-erythroid tissue is evident in mice with EPOR restricted to erythroid tissues (ΔEPORE) that become obese, glucose-intolerant, and insulin-resistant. In animal models, nitric oxide synthase (NOS) contributes to EPO activities including erythropoiesis, neuroprotection, and cardioprotection against ischemia-reperfusion injury. However, we found that extended EPO treatment to increase hematocrit compromised heart function, while the loss of neuronal NOS (nNOS) was protective against the deleterious activity of EPO to promote heart failure. Methods: Wild-type (WT) mice, ΔEPORE mice, and nNOS-knockout mice (nNOS-/-) were placed on a high-fat diet to match the ΔEPORE obese phenotype and were treated with EPO for 3 weeks. Hematocrit and metabolic response to EPO treatment were monitored. Cardiac function was assessed by echocardiography and ultrasonography. Results: ΔEPORE mice showed a decrease in the left ventricular outflow tract (LVOT) peak velocity, ejection fraction, and fractional shortening, showing that endogenous non-erythroid EPO response is protective for heart function. EPO treatment increased hematocrit in all mice and decreased fat mass in male WT, demonstrating that EPO regulation of fat mass requires non-erythroid EPOR. EPO treatment also compromised heart function in WT mice, and decreased the pulmonary artery peak velocity (PA peak velocity), LVOT peak velocity, ejection fraction, and fractional shortening, but it had minimal effect in further reducing the heart function in ΔEPORE mice, indicating that the adverse effect of EPO on heart function is not related to EPO-stimulated erythropoiesis. ΔEPORE mice had increased expression of heart failure-associated genes, hypertrophic cardiomyopathy-related genes, and sarcomeric genes that were also elevated with EPO treatment in WT mice. Male and female nNOS-/- mice were protected against diet-induced obesity. EPO treatment in nNOS-/- mice increased the hematocrit that tended to be lower than WT mice and decreased the PA peak velocity but did not affect the LVOT peak velocity, ejection fraction, and fractional shortening, suggesting that nNOS is required for the adverse effect of EPO treatment on heart function in WT mice. EPO treatment did not change expression of heart failure-associated gene expression in nNOS-/- mice. Discussion: Endogenous EPO has a protective effect on heart function. With EPO administration, in contrast to the protective effect to the cardiac injury of acute EPO treatment, extended EPO treatment to increase hematocrit in WT mice adversely affected the heart function with a corresponding increase in expression of heart failure-associated genes. This EPO activity was independent of EPO-stimulated erythropoiesis and required EPOR in non-erythroid tissue and nNOS activity, while nNOS-/- mice were protected from the EPO-associated adverse effect on heart function. These data provide evidence that nNOS contributes to the negative impact on the heart function of high-dose EPO treatment for anemia.

Impact of systematic variations in hematocrit and platelet count on thrombelastometry tissue factor activated assay parameters.

BACKGROUND: Thromboelastogram testing is increasingly being used to manage patients with massive bleeding. An earlier study found that the test results were influenced by the hematocrit (Hct) and platelet (PLT) concentrations. This study sought to determine if these factors confounded the results of a different manufacturer's thromboelastography testing. METHODS: Using freshly collected whole blood from volunteers and stored red blood cells (RBC) and plasma, the whole blood was manipulated to achieve different Hct values and PLT concentrations. Each reconstituted whole blood sample was tested in triplicate on the ROTEM Delta device and the ExTEM results were recorded. RESULTS: Many of the ExTEM results varied according to the Hct and PLT concentration. In particular, the ExTEM clot formation time (CFT) was abnormally long when the Hct was 45% and the PLT concentration was ≤75 × 109/L, normalizing only when the PLT count was ≥100 × 109/L. CFT samples with Hct 25% and 35% were also abnormal with low PLT concentrations but normalized at lower PLT concentrations compared to the Hct 45% samples. The ExTEM angle also demonstrated abnormal results when the Hct was 45% and the PLT concentration was ≤50 × 109/L. The ExTEM A10 and maximum clot firmness (MCF) tests tended to also be abnormal when the Hct was between 25% and 45% and the platelet concentrations were below 75 × 109/L. CONCLUSION: While thromboelastogram testing is gaining popularity for managing bleeding patients, clinicians should be aware of these confounding factors when making transfusion decisions based on their results.