RATIONALE AND OBJECTIVES

Individuals with myeloproliferative neoplasia (MPN) have blood cell parameters representing abnormal proliferation of the cell line/lines affected. Considering the implication of symptom burden scores to treatment response and disease progression, with the same implication among changes in blood cell parameters, a question of correlation between the two variables becomes inevitable. This study aims to determine the correlation of controlled blood counts, severity of symptoms and quality of life of individuals with MPN.

This is a cross-sectional analytical study and a sub-study from the Filipino myeloproliferative neoplasia quality of life (MPN-QOL) multicenter study. Secondary data obtained from the parent study will be used as primary data of this sub-study. Comparative analyses were conducted using Chi-Square Test of Homogeneity or Fisher’s Exact Test. Association analysis used Cramer’s V coefficient.

Data in this study has shown 52.65 years old as the average age of participants. Most participants had mild symptom burden at 60.53% with the most common symptom being fatigue. Comparative analysis showed the absence of identified statistical difference in the overall symptom burden severity among the three types of MPN.

In this study, there was no statistically significant correlation between the severity of symptom burden or quality of life, and the degree of blood count control among the three types of MPN. In practice, controlling hematologic parameters has been a goal to achieve among patients with MPN. This study suggests symptom control and quality of life is not necessarily affected by blood count control.

OBJECTIVE: To analyze the occurrence of arterial events and platelet parameters in patients with polycythemia vera (PV) and essential thrombocythemia (ET), and to explore the characteristics of platelet parameters in patients with PV and ET and their relationship with arterial complications. METHODS: The clinical and laboratory data of newly diagnosed PV and ET patients who visited the Department of Hematology, Beijing Anzhen Hospital, Capital Medical University from August 2017 to August 2022 were retrospectively analyzed. RESULTS: 86 MPN patients (46 males and 40 females) were enrolled, including 44 PV patients and 42 ET patients, with an median age of 61(23-83) years. The mutation rate of JAK2V617F gene, the number of megakaryocytes in bone marrow, the incidence of splenomegaly, and the levels of white blood cell count (WBC), hemoglobin (HGB), hematocrit (HCT), platelet distribution width (PDW), mean platelet volume (MPV), platelet-large cell ratio (P-LCR) in PV patients were significantly higher than those in ET patients (P < 0.05), while the levels of PLT and PCT were significantly lower than those in ET patients (P < 0.01). 22 cases (50%) of PV patients were complicated with arterial events, of which 12 had arterial stenosis in≥2 locations. Among arterial events, the PDW of PV patients with ischemic stroke was greater than that of PV patients without ischemic stroke (P =0.003), and the PDW of PV patients with arterial stenosis in≥2 locations was greater than that of PV patients with arterial stenosis in≤1 location (P =0.037). 23 cases (54.8%) of ET patients were complicated with arterial events, and 7 cases had arterial stenosis in≥2 locations. In arterial events, the PCT of ET patients complicated with ischemic stroke was greater than that of ET patients without ischemic stroke (P =0.037), and the PCT of ET patients with≥2 locations of arterial stenosis was greater than that of ET patients with≤1 location of arterial stenosis (P =0.049). The binary logistic regression analysis showed that elevated PDW and PCT were risk factors for ischemic stroke in PV and ET patients, respectively (P < 0.05). CONCLUSION The platelet parameters of PV and ET patients exhibit significantly different characteristics. Elevated PDW and PCT can predict a higher risk of ischemic stroke in PV and ET patients, respectively.
Humans ; Middle Aged ; Female ; Male ; Aged ; Retrospective Studies ; Adult ; Thrombocythemia, Essential/blood* ; Myeloproliferative Disorders/complications* ; Aged, 80 and over ; Polycythemia Vera/blood* ; Blood Platelets ; Platelet Count ; Young Adult ; Mean Platelet Volume

Ropeginterferon α-2b (Ropeg), a novel, long-acting pegylated prolene alpha interferon, is the first interferon specifically approved for the treatment of patients with polycythemia vera (PV), and has been found in clinical trials and experience to induce hematologic remission, control disease-related symptoms, and reduce JAK2V617F allelic burden in patients with myeloproliferative neoplasms (MPNs). It has a lower incidence and severity of adverse drug reactions than pegylated interferon alpha and hydroxyurea and a longer dosing interval. Some patients with lowrisk PV and myelofibrosis can benefit from it. This article reviews the latest progress of Ropeg in MPN.
Humans ; Interferon-alpha/therapeutic use* ; Myeloproliferative Disorders/drug therapy* ; Polyethylene Glycols/therapeutic use* ; Recombinant Proteins/therapeutic use* ; Interferon alpha-2 ; Polycythemia Vera/drug therapy*

OBJECTIVE: To understand the biological characteristics of polycythemia vera (PV) patients with myeloid fibroplasia, and further analyze the risk factors affecting myeloid fibroplasia in PV patients, so as to provide ideas for predicting the occurrence of myeloid fibroplasia in PV patients. METHODS: Forty patients with PV in the Department of Hematology, Xiyuan Hospital of China Academy of Chinese Medical Sciences were collected and divided into two groups, with (hyperplasia group) and without (Non-proliferative group) hyperplasia of bone marrow fibers. The differences of basic clinical characteristics, blood routine, biochemistry, bone marrow cells, coagulation function and other indicators between the two groups were compared, and the independent risk factors affecting the proliferation of bone marrow fibrous tissue in PV patients were further analyzed by multivariate regression. RESULTS: Compared with Non-proliferative group, the JAK2 mutation rate (95% vs 70%，P=0.037), eosinophilic cell count (0.19 vs 0.11, P=0.047) and eosinophilic percentage (1.84 vs 1.27, P=0.001) in PV patients with hyperplasia were significantly increased, triglycerides (1.55 vs 1.91, P=0.038) and low-density lipoprotein (1.50 vs 3.08, P=0.000) were significantly reduced, bone marrow hematopoietic volume (0.85 vs 0.6, P=0.001), granulocyte/erythrocyte ratio (3.40 vs 1.89, P=0.033), lymphocyte/erythrocyte ratio (0.60 vs 0.42, P=0.033), and granulocyte+lymphocyte/erythrocyte ratio (3.72 vs 2.37, P=0.026) were significantly increased, thrombin time (18.84 vs 18.12, P=0.043) was significantly prolonged. Multivariate regression analysis results showed that peripheral blood eosinophil ≥2% and low-density lipoprotein ≤2 mmol/L were independent risk factors for bone marrow fibrous tissue hyperplasia in PV patients (P<0.05). CONCLUSION Increased proportion of peripheral blood eosinophils and decreased low density lipoprotein are risk factors for bone marrow fibrous tissue hyperplasia in PV patients.
Humans ; Bone Marrow/pathology* ; Polycythemia Vera ; Hyperplasia/pathology* ; Granulocytes/pathology* ; Janus Kinase 2/genetics* ; Risk Factors ; Lipoproteins, LDL ; Polycythemia/pathology*

Calreticulin/genetics* ; DNA Methylation ; Female ; Humans ; Janus Kinase 2/genetics* ; Mutation ; Myeloproliferative Disorders/genetics* ; Polycythemia Vera/genetics*

Objective: To compare clinical and laboratory features between JAK2 exon12 and JAK2 V617F mutated polycythemia vera (PV) . Method: We collected data from 570 consecutive newly-diagnosed subjects with PV and JAK2 mutation, and compared clinical and laboratory features between patients with JAK2 exon12 and JAK2 V617F mutation. Results: 543 (95.3%) subjects harboured JAK2 V617F mutation (JAK2 V617F cohort) , 24 (4.2%) harboured JAK2 exon12 mutations (JAK2 exon12 cohort) , and 3 (0.5%) harboured JAK2 exon12 and JAK2 V617F mutations. The mutations in JAK2 exon12 including deletion (n=10, 37.0%) , deletion accompanied insertion (n=10, 37.0%) , and missense mutations (n=7, 25.9%) . Comparing with JAK2 V617F cohort, subjects in JAK2 exon12 cohort were younger [median age 50 (20-73) years versus 59 (25-91) years, P=0.040], had higher RBC counts [8.19 (5.88-10.94) ×10(12)/L versus 7.14 (4.11-10.64) ×10(12)/L, P<0.001] and hematocrit [64.1% (53.7-79.0%) versus 59.6% (47.2%-77.1%) , P=0.001], but lower WBC counts [8.29 (3.2-18.99) ×10(9)/L versus 12.91 (3.24-38.3) ×10(9)/L, P<0.001], platelet counts [313 (83-1433) ×10(9)/L versus 470 (61-2169) ×10(9)/L, P<0.001] and epoetin [0.70 (0.06-3.27) versus 1.14 (0.01-10.16) IU/L, P=0.002] levels. We reviewed bone marrow histology at diagnosis in 20 subjects with each type of mutation matched for age and sex. Subjects with JAK2 exon12 mutations had fewer loose megakaryocyte cluster (40% versus 80%, P=0.022) compared with subjects with JAK2 V617F. The median follow-ups were 30 months (range 4-83) and 37 months (range 1-84) for cohorts with JAK2 V617F and JAK2 exon12, respectively. There was no difference in overall survival (P=0.422) and thrombosis-free survival (P=0.900) . Conclusions: Compared with patients with JAK2 V617F mutation, patients with JAK2 exon12 mutation were younger, and had more obvious erythrocytosis and less loose cluster of megakaryocytes.
Adult ; Aged ; Aged, 80 and over ; Bone Marrow/pathology* ; Exons ; Humans ; Janus Kinase 2/genetics* ; Middle Aged ; Mutation ; Mutation, Missense ; Polycythemia Vera/genetics* ; Young Adult

OBJECTIVE: To evaluate the efficacy and safety of ruxolitinib in patients with polycythemia vera (PV). METHODS: The clinical data of patients with PV treated with ruxolitinib in Peking Union Medical College Hospital from January 1, 2013 to December 31, 2019 were retrospectively analyzed. The starting dose of oral ruxolitinib was 10 mg twice daily and could be increased after 3 months of treatment if hematocrit (HCT) control was not achieved. HCT control was defined as HCT<45% in the absence of phlebotomy. RESULTS: Thirty-three patients (17 males and 16 females) were treated with ruxolitinib at a median age of 50 (21-72) years. JAK2V617F and JAK2exon12 alleles were detected in 31 and 2 patients, respectively. Before treatment, median hemoglobin level was 187 (166-208) g/L, median white blood cell and platelet level was 10.4 (5.0-15.8)×10⁹/L and 457(237-677)×10⁹/L, respectively. Totally 17 patients (51.5%) who were resistant to or intolerant of hydroxyurea were treated with ruxolitinib as second-line therapy, and 16 patients (48.5%) were treated with ruxolitinib as first-line therapy voluntarily. The median time since PV diagnosis to treatment of ruxolitinib was 47 (3-188) months. By December 31, 2019, all the patients continued to receive ruxolitinib. The median duration of ruxolitinib exposure was 19 (2-91) months. Both in the first-line therapy group and second-line therapy group, 15 cases (accounting for 93.8% and 88.2%, respecitvely) achieved HCT control. The median time from start of therapy to HCT control was 2.2 (0.8-11.6) months. One patient (3.0%) had disease progression after HCT control. The most common hematologic adverse events included anemia and thrombocytopenia, according to CTCAE classification, including 1 case of grade 1 anemia (3.0%) and 1 case of grade 2 thrombocytopenia (3.0%). There was no thromboembolic event occurred during the therapy of ruxolitinib. CONCLUSION The remission rate of HCT in PV patients treated with ruxolitinib is high, and adverse reactions are rare. Ruxolitinib is effective in HCT control and generally well tolerated in patients with PV.
Adult ; Aged ; Anemia ; Female ; Hemoglobins/therapeutic use* ; Humans ; Hydroxyurea/therapeutic use* ; Male ; Middle Aged ; Nitriles ; Polycythemia Vera/drug therapy* ; Pyrazoles ; Pyrimidines ; Retrospective Studies ; Thrombocytopenia ; Young Adult

OBJECTIVE: To explore the relationship between clinical features, peripheral blood cell count, coagulation function, gene mutation and hemorrhagic events and thrombotic events in essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis(PMF) patients. METHODS: Clinical data of 78 patients with ET, PV, and PMF who were admitted to the Second Affiliated Hospital of Chongqing Medical University between September 2019 and August 2020 were retrospectively analyzed. Information about sex, age, gene mutation, peripheral blood cell count, coagulation function, and hemorrhagic and thrombotic events was included, and the influence of these data on the occurrence of hemorrhagic and thrombotic events was estimated. RESULTS: Among the 78 patients with myeloproliferative neoplasms, there were 47 cases of ET, 15 cases of PV, and 16 cases of PMF.A total of 10 patients (12.82%) experienced hemorrhagic events and 27 (34.62%) experienced thrombotic events. Male,patients aged ≥ 60 years, and patients with a JAK2V617F mutation were more likely to experience thrombotic events (P<0.05). Patients with thrombotic events had higher platelet (PLT) counts and fibrinogen (FIB) levels than patients without hemorrhagic-thrombotic events (P<0.05).White blood cell (WBC) count, red blood cell (RBC) count, hemoglobin (HGB) level, prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time (TT) and international normalized ratio (INR) showed no statistical difference between patients with thrombotic events and patients without hemorrhagic-thrombotic events (P>0.05). There was also no significant difference in the above-mentioned indexes between patients with hemorrhagic events and patients without hemorrhagic-thrombotic events (P>0.05). Among JAK2V617F positive myeloproliferative neoplasm patients, male patients were more likely to have thrombotic events (P<0.05), and patients with thrombotic events had higher platelet counts than those without hemorrhagic-thrombotic events (P<0.05). There was no significant difference in age, white blood cell count, red blood cell count, hemoglobin level, PT, APTT, FIB, TT or INR between patients with thrombotic events and patients without hemorrhagic-thrombotic events (P>0.05). CONCLUSION Sex, age, JAK2V617F mutation and platelet count have a certain value for predicting thrombosis in patients with myeloproliferative neoplasms.
Hemoglobins/genetics* ; Hemorrhage ; Humans ; Janus Kinase 2/genetics* ; Male ; Mutation ; Myeloproliferative Disorders/genetics* ; Polycythemia Vera/genetics* ; Retrospective Studies ; Thrombocythemia, Essential ; Thrombosis

OBJECTIVE: To investigate the relationship between JAK2 gene mutation and clinical indicators in patients with myeloproliferative neoplasms (MPN). METHODS: 122 MPN patients in the Department of Hematology, Xiyuan Hospital, China Academy of Chinese Medical Sciences from September 2017 to January 2020 were retrospectively analyzed. The relationship between JAK2 gene mutation and sex, age, peripheral blood cell count, splenomegaly, and thrombosis and bleeding events were analyzed. RESULTS: In 122 patients with MPN, the patients with polycythemia vera (PV) accounted for 36 (29.5%), the patients with essential thrombocythemia (ET) accounted for 56 (45.9%), the patients with myelofibrosis (MF) accounted for 30 (24.6%). The JAK2 gene mutation rate in MPN patients was 64.6% (79/122), and the JAK2 gene mutation rate in PV, ET and MF groups were 77.7% (28/36), 60.7% (34/56) and 56.7% (17/30), the JAK2 gene mutation rate of the patients in PV group was statistically significant as compared with those in the ET group (P＜0.05). The hemoglobin (Hb) count of the patients in JAK2 gene mutation group was higher than those in wild-type group ［(150.0±39.6)g/L vs (129.4±38.9)g/L, P＜0.05］; the white blood cell (WBC) count of the patients in JAK2 gene mutation group was higher than those in the wild type group ［(9.5±4.7)×10 CONCLUSION The mutation rate of JAK2 gene in MPN patients is higher, and the mutation rate of JAK2 gene in PV patients is higher than that in ET and MF patients; JAK2 gene mutations in MPN patients are related to hemogram index; the incidence of splenomegaly is the highest in MF patients, and splenomegaly is related to the occurrence of JAK2 gene mutations in MF patients.
Humans ; Janus Kinase 2/genetics* ; Mutation Rate ; Myeloproliferative Disorders/genetics* ; Polycythemia Vera ; Retrospective Studies

OBJECTIVE: To analyze the disease types, clinical manifestations, efficacy and outcome of JAK2 V617F and BCR-ABL double-mutant myeloproliferative neoplasms (MPN), and provide a reference for the diagnosis, treatment and prognosis of MPN. METHODS: The clinical characteristics, diagnosis, therapeutic efficacy and outcome of JAK2 V617F and BCR-ABL double-mutant MPN were analyzed comprehensitively by combining a clinical case diagnosed and treated in our hospital with literature cases from CNKI and PubMed databases. RESULTS: A total of 38 related literatures were retrieved from the two databases by searching "JAK2 V617F" and "BCR-ABL" as key words from 1990 to 2019, and 59 cases were involved. Among all the 60 cases, 41 were males (68.3%) with a median age of 61 (32-77) years old, while 19 were females (31.7%) with a median age of 58 (21-82) years old. The BCR-ABL fusion gene and JAK2 V617F mutation were found simultaneously in 21 cases (35%), 19 cases (31.7%) with JAK2 V617F mutation were found during the treatment of Philadelphia chromosome (Ph)-positive chronic myelogenous leukemia (CML). Ph CONCLUSION As cases of BCR-ABL and JAK2 V617F double-mutant MPN are reported, simultaneous detection of JAK2 V617F mutation and BCR-ABL fusion gene in MPN patients is necessary to avoid misdiagnosis and missed diagnosis.
Adult ; Aged ; Aged, 80 and over ; Female ; Fusion Proteins, bcr-abl/genetics* ; Humans ; Janus Kinase 2/genetics* ; Male ; Middle Aged ; Myeloproliferative Disorders/genetics* ; Polycythemia Vera ; Thrombocythemia, Essential ; Young Adult