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Journal of Clinical Medicine Jun 2024Polycythemia vera (PV) is a chronic hematologic neoplasm commonly treated with hydroxyurea (HU). We utilized the advanced digitalized database of Maccabi Healthcare...
Polycythemia vera (PV) is a chronic hematologic neoplasm commonly treated with hydroxyurea (HU). We utilized the advanced digitalized database of Maccabi Healthcare Services to retrospectively investigate the clinical and economic implications of HU intolerance in the routine clinical care of PV patients in Israel. We collected data on demographics, physician visits, hospitalizations, laboratory results, medication purchases, cardiovascular and thrombotic events, mental health, economic outcomes, and mortality. Outcomes included cardiovascular and other thrombotic events, disease progression, mental health events, economic outcomes, and overall mortality. Of the 830 patients studied, 3 (0.4%) were resistant to HU treatment, 318 (38.3%) were intolerant to HU treatment, and 509 (61.3%) were stable on HU treatment. The venous thrombosis rate was significantly higher among HU-intolerant compared to HU-stable patients (1.58 vs. 0.47 per 100 person-years [PY], respectively; < 0.001). The rate of progression to myelofibrosis was 6 vs. 0.9 per 100 PY in HU-intolerant patients vs. HU-stable patients, respectively ( < 0.001), and the rate of progression to acute myeloid leukemia (AML) was 1.16 vs. 0.2 per 100 PY in HU-intolerant patients vs. HU-stable patients, respectively ( < 0.001). The phlebotomy requirement, mortality rate, and total hospitalization days among HU-intolerant patients were significantly higher than in HU-stable patients ( = 0.049, < 0.001, < 0.001, respectively). More mental health-related events were noted in HU-intolerant patients vs. HU-stable patients ( = 0.007), and the total healthcare cost ratio was 2.65 for the HU-intolerant patients compared with HU-stable patients. This study suggests that HU-intolerant patients are more likely to have worse outcomes than HU-stable patients, highlighting the need for the close monitoring of these patients for disease-related complications or progression.
PubMed: 38929918
DOI: 10.3390/jcm13123390 -
International Journal of Molecular... Jun 2024Myeloproliferative neoplasms (MPNs), namely, polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are clonal stem cell disorders...
Assessment of Total Antioxidant Capacity, 8-Hydroxy-2'-deoxy-guanosine, the Genetic Landscape, and Their Associations in -Negative Chronic and Blast Phase Myeloproliferative Neoplasms.
Myeloproliferative neoplasms (MPNs), namely, polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are clonal stem cell disorders defined by an excessive production of functionally mature and terminally differentiated myeloid cells. MPNs can transform into secondary acute myeloid leukemia (sAML/blast phase MPN) and are linked to alterations in the redox balance, i.e., elevated concentrations of reactive oxygen species and markers of oxidative stress (OS), and changes in antioxidant systems. We evaluated OS in 117 chronic phase MPNs and 21 sAML cases versus controls by measuring total antioxidant capacity (TAC) and 8-hydroxy-2'-deoxy-guanosine (8-OHdG) concentrations. TAC was higher in MPNs than controls ( = 0.03), particularly in ET ( = 0.04) and PMF ( = 0.01). -positive MPNs had higher TAC than controls ( = 0.002) and triple-negative MPNs ( = 0.01). PMF patients who had treatment expressed lower TAC than therapy-free subjects ( = 0.03). 8-OHdG concentrations were similar between controls and MPNs, controls and sAML, and MPNs and sAML. We noted associations between TAC and MPNs (OR = 1.82; = 0.05), i.e., ET (OR = 2.36; = 0.03) and PMF (OR = 2.11; = 0.03), but not sAML. 8-OHdG concentrations were not associated with MPNs (OR = 1.73; = 0.62) or sAML (OR = 1.89; = 0.49). In conclusion, we detected redox imbalances in MPNs based on disease subtype, driver mutations, and treatment history.
Topics: Humans; Male; Female; 8-Hydroxy-2'-Deoxyguanosine; Middle Aged; Aged; Myeloproliferative Disorders; Antioxidants; Adult; Oxidative Stress; Aged, 80 and over; Blast Crisis; Fusion Proteins, bcr-abl; Primary Myelofibrosis
PubMed: 38928358
DOI: 10.3390/ijms25126652 -
SAGE Open Medical Case Reports 2024Although disseminated cryptococcosis can occur occasionally, it is most commonly seen in immunodeficient patients. In 2005, a 43-year-old man was diagnosed with...
Although disseminated cryptococcosis can occur occasionally, it is most commonly seen in immunodeficient patients. In 2005, a 43-year-old man was diagnosed with polycythemia vera. Following in 2018, he experienced an unknown-cause fever and headache. To establish the source of the symptoms, a magnetic resonance imaging scan of the brain was performed, which indicated meningeal and gyral-leptomeningeal thickening and several localized T2 hyperintense lesions measuring up to 10 × 14 mm in diameter. was then cultivated from cerebrospinal fluid. Serum IgM antibodies against West Nile Virus were positive. After 8 weeks of treatment with amphotericin B and fluconazole, the overall condition improved, and the cerebrospinal fluid control culture became negative. The symptoms returned shortly after discontinuing antifungal therapy, necessitating the reintroduction of fluconazole. Currently, the patient is stable and responding positively to ruxolitinib. Here, it is demonstrated how a patient with polycythemia vera due to immunological weakness might develop disseminated cryptococcosis of the brain after West Nile virus infection.
PubMed: 38895656
DOI: 10.1177/2050313X241262145 -
Journal of Clinical Medicine Jun 2024Patients with myeloproliferative neoplasms (MPNs) experience a high disease-related symptom burden. A specific instrument to evaluate quality of life (QoL), i.e., the...
Translation, Cultural Adaptation, and Validation into Romanian of the Myeloproliferative Neoplasm Symptom Assessment Form-Total Symptom Score (MPN-SAF TSS or MPN-10) Questionnaire.
Patients with myeloproliferative neoplasms (MPNs) experience a high disease-related symptom burden. A specific instrument to evaluate quality of life (QoL), i.e., the MPN Symptom Assessment Form Total Symptom Score (MPN-SAF TSS; MPN-10), was developed. We conducted the translation, cultural adaptation, and validation into Romanian of the MPN-10. We translated the MPN-10 and tested its psychometric properties. We recruited 180 MPN patients: 66 polycythemia vera (36.67%), 61 essential thrombocythemia (33.89%), 51 primary and secondary myelofibrosis (SMF) (28.33%), and 2 MPN-unclassifiable (1.11%). The mean TSS was 19.51 ± 16.51 points. Fatigue, inactivity, and concentration problems were the most cumbersome symptoms. We detected scoring differences between MPN subtypes regarding weight loss ( < 0.001), fatigue ( = 0.006), early satiety ( = 0.007), night sweats ( = 0.047), pruritus ( = 0.05), and TSS ( = 0.021). There were strong positive associations between TSS and inactivity, fatigue, and concentration problems, and moderate negative correlations between QoL scores and all MPN-10 items. Cronbach's α internal consistency coefficient was 0.855. The Kaiser-Meyer-Olkin construct validity test result was 0.870 and the Bartlett Sphericity Test was significant ( < 0.001). Symptom scores were loaded into one single factor according to the exploratory factor analysis. The Romanian MPN-10 version displayed excellent psychometric properties and is a reliable instrument for assessing symptom burden and QoL in Romanian MPN patients.
PubMed: 38892995
DOI: 10.3390/jcm13113284 -
Physiological Reports Jun 2024Large-volume therapeutic phlebotomy is the mainstay of hemochromatosis treatment and offers an opportunity to investigate the hemodynamic changes during acute...
Large-volume therapeutic phlebotomy is the mainstay of hemochromatosis treatment and offers an opportunity to investigate the hemodynamic changes during acute hypovolemia. An otherwise healthy 64-year-old male with hemochromatosis participated. On nine separate visits, 1000 mL therapeutic phlebotomy was performed. On one occasion, pre- and post-phlebotomy orthostatic challenge with 27° reverse Trendelenburg position was administered. Mean arterial pressure, heart rate, and stroke volume were measured continuously during the procedures. The patient's tolerance to the interventions was continuously evaluated. The procedures were well tolerated by the patient. Mean arterial pressure was maintained during hemorrhage and following phlebotomy in both supine and reverse Trendelenburg positions, primarily through an increase in heart rate and systemic vascular resistance. The present study found that 1000 mL therapeutic phlebotomy in a patient with hemochromatosis may be acceptably and safely used to model hemorrhage. The approach demonstrates high clinical applicability and ethically robustness in comparison with volunteer studies.
Topics: Humans; Male; Phlebotomy; Middle Aged; Polycythemia; Hemochromatosis; Heart Rate; Hemorrhage
PubMed: 38844733
DOI: 10.14814/phy2.16035 -
Radiology Case Reports Aug 2024We report a case of acute myocardial infarction in a patient with polycythemia rubra vera, who has been treated with hydroxyurea. The patient presented with chest pain...
We report a case of acute myocardial infarction in a patient with polycythemia rubra vera, who has been treated with hydroxyurea. The patient presented with chest pain extending to both arms accompanied by nausea and sweating. Hemoglobin was 18.1 mg/dL, hematocrit 53.2%, white blood cells 9600/mm³, and platelets 745,000/mm³. The levels of specific cardiac injury markers were increased, troponin I increased to 110 ng/mL and creatine kinase-MB to 361 U/l, respectively. Electrocardiography showed sinus rhythm with ST-segment elevation in leads V2-6, D1, and aVL as well as ST depression in D2, D3 and aVF. Echocardiography demonstrated hypokinesis of the interventricular septum and lateral wall with mildly reduced left ventricle (LV) ejection fraction (EF≈45%). Coronary angiography revealed proximal-LAD subtotal occlusion and 80% mid-LAD stenosis with distal-LAD vasospasm. Percutaneous coronary intervention was performed with a drug-eluting stent in mid- and proximal-LAD. Hypercoagulable state of polycythemia rubra vera may be complicated with acute myocardial infarction, in addition to the vasospastic effect and endothelium lesions of hydroxyurea regardless its favorable effect as a standard therapy.
PubMed: 38827039
DOI: 10.1016/j.radcr.2024.05.014 -
Pulmonology Jun 2024
PubMed: 38825549
DOI: 10.1016/j.pulmoe.2024.05.003 -
Clinical methemoglobinemia secondary to administration of hydroxyurea at therapeutic doses in a dog.Journal of Veterinary Internal Medicine Jun 2024Methemoglobinemia secondary to administration of hydroxyurea is only reported in veterinary medicine as a result of accidental ingestion of high doses, and once at...
Methemoglobinemia secondary to administration of hydroxyurea is only reported in veterinary medicine as a result of accidental ingestion of high doses, and once at therapeutic dose in human medicine. A 2.5-year-old female spayed mixed breed dog was presented for acute signs of neurologic disease and diagnosed with severe erythrocytosis without an identified underlying cause, leading to suspicion of polycythemia vera. The dog was managed with phlebotomies, supportive care, and administration of hydroxyurea. Within 2 h of administration of hydroxyurea (37 mg/kg) administration, respiratory distress with cyanosis, and methemoglobinemia developed. Signs resolved within 24 h but recurred after a second administration of lower dosage of hydroxyurea (17 mg/kg) 20 days later. The dog remained asymptomatic except for mild cyanosis but was humanely euthanized for lack of relevant improvement of signs of neurologic disease. This case report documents the repeated occurrence of methemoglobinemia in a dog after administration of hydroxyurea at therapeutic doses.
PubMed: 38822748
DOI: 10.1111/jvim.17127 -
World Journal of Clinical Cases May 2024Acute upper gastrointestinal bleeding is a common medical emergency that has a 10% hospital mortality rate. According to the etiology, this disease can be divided into...
BACKGROUND
Acute upper gastrointestinal bleeding is a common medical emergency that has a 10% hospital mortality rate. According to the etiology, this disease can be divided into acute varicose veins and nonvaricose veins. Bleeding from esophageal varices is a life-threatening complication of portal hypertension. Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10 mmHg. Cirrhosis is the most common cause of portal hypertension, and thrombosis of the portal system not associated with liver cirrhosis is the second most common cause of portal hypertension in the Western world. Primary myeloproliferative disorders are the main cause of portal venous thrombosis, and somatic mutations in the gene () can be found in approximately 90% of polycythemia vera, 50% of essential thrombocyrosis and 50% of primary myelofibrosis.
CASE SUMMARY
We present a rare case of primary myelofibrosis with gastrointestinal bleeding as the primary manifestation that presented as portal-superior-splenic mesenteric vein thrombosis. Peripheral blood tests revealed the presence of the mutation. Bone marrow biopsy ultimately confirmed the diagnosis of myelofibrosis (MF-2 grade).
CONCLUSION
In patients with acute esophageal variceal bleeding due to portal hypertension and vein thrombosis without cirrhosis, the possibility of myeloproliferative neoplasms should be considered, and the mutation test should be performed.
PubMed: 38817215
DOI: 10.12998/wjcc.v12.i15.2621 -
Journal of Personalized Medicine Apr 2024To investigate inflammation indices and erythropoietin levels for their potential role in distinguishing polycythemia vera from secondary polycythemia and to compare...
AIM
To investigate inflammation indices and erythropoietin levels for their potential role in distinguishing polycythemia vera from secondary polycythemia and to compare different parameter combinations in terms of the diagnostic accuracy.
METHODS
This retrospective cohort was created from patients assessed for polycythemia from January 2020 to December 2023. Polycythemia vera diagnosis was made according to the 2016 World Health Organization criteria ( = 145). Those who did not fulfill the criteria were defined as having secondary polycythemia ( = 84).
RESULTS
The neutrophil lymphocyte ratio, platelet lymphocyte ratio and systemic immune-inflammation index were significantly higher in the polycythemia vera group ( < 0.001 for all). Erythropoietin had the highest area under the curve in the analysis to distinguish groups, followed by the systemic immune-inflammation index. The platelet lymphocyte ratio (≥135) had the highest specificity to detect polycythemia vera, followed closely by the systemic immune-inflammation index. The sensitivity for polycythemia vera detection was highest with the erythropoietin and systemic immune-inflammation index combination, followed by erythropoietin and the neutrophil lymphocyte ratio. All the single and combinatory variables exhibited significant performance in predicting polycythemia vera after adjusting for age and sex. However, the erythropoietin and systemic immune-inflammation index combination had the highest odds ratio, followed by erythropoietin alone.
CONCLUSION
These are promising findings supporting the usability of these biomarkers, especially the systemic immune-inflammation index, as minor criteria in the diagnosis of polycythemia vera. It is especially crucial to note that using erythropoietin in combination with these markers may improve diagnostic accuracy.
PubMed: 38793053
DOI: 10.3390/jpm14050471