| Objective To evaluate the symptom burden in patients with BCR/ABL-negative myelogenous proliferative neoplasms(MPN),to analyze the risk factors for thrombotic events,and the correlation between symptom burden and thrombotic events,then to guide the individualized treatment and reduce thrombosis events,to improve the quality of life.Methods1.The clinical data of 108 patients with BCR/ABL-negative myeloproliferative neoplasms who were diagnosed and treated from November 2007 to November 2017 in the First Affiliated Hospital of Jinan University were collected and summarized,including age distribution,distribution of blood tests,and symptoms.Distribution,clinical features of JAK2 gene mutations.2.The MPN-10 scale was completed in 50 patients with MPN.The three subtypes of myelofibrosis,polycythemia vera,and essential thrombocythemia were assessed using the MPN-10 scale,respectively.Feeling,abdominal discomfort,poor mobility,lack of concentration,night sweats,pruritus,bone pain,fever,weight loss,and symptom score.The scores are divided into four levels Q1-Q4(Q1:<8;Q2:8-17;Q3:18-31;Q4:≥32),ranging from 0-100 points.The risk factors of 29 PMF patients were evaluated by DIPSSsy system.3.108 cases of BCR/ABL-negative MPN patients were divided into thrombotic event group and non-thrombotic event group.WBC,HB,PLT,JAK gene,gender,age,hypertension,diabetes,history of thrombosis,subtypes were analyzed.And other indicators are related to the occurrence of thrombosis.X2 test and t test were used to determine whether there were statistically significant differences between the two groups under the influence of different factors.Logistic regression analysis was used to analyze whether there were significant differences in the occurrence of different symptoms and thrombotic events in different subtypes.4.The hemorheology of 43/108 patients were detected.The correlation between the high-cut,mid-cut,and low-cut viscosity indexes in hemorheology and the occurrence of thrombotic events were univariately analyzed.Result1.Clinical characteristics:(1)Basic features: 24/108 PV patients(22.2%),55/108 ET patients(50.9%),and 29/108 PMF patients(26.9%),with a median age of 59.5 year-old.(2)Age distribution: MPN was found in any age group of adults.The proportion of 50-59-year-old group and 70-79-year-old group was the highest,both accounting for 46.3%.Most of the patients were older than 40 year-old,accounting for 80.0%.(3)Symptom distribution: Among 108 patients,52 were males and 56 were females.The median age was 59.5 year-old.76 patients(70.4%)were diagnosed with dizziness,fatigue,chest tightness,shortness of breath,and other symptoms of anemia;due to hepatosplenomegaly 48 patients(44.4%);11 patients(10.2%)attending gastrointestinal symptoms such as abdominal distention,abdominal pain,vomiting,etc.,6 patients(5.6%)due to limb numbness;bleeding due to gums,gastrointestinal bleeding,and cyanosis of both lower extremities A total of 12 patients(11.1%)had bleeding problems,6(5.6%)had headaches,1 had a rash(0.9%),and 3(2,8%)had no symptoms.(4)Gene distribution In 108 patients,the total positive rate of JAK2 gene expression was 51.8%.Among them,the positive rates of JAK2 gene expression in patients with PV,ET and PMF were 70.8%,56.4% and 27.6%,respectively.(5)HB and HCT were significantly higher in PV patients than in the other two types,with an average of(184.97±33.32)g/L,(56.66±9.41)g/L,and WBC and PLT of(12.74±5.36)× 109/L,(389.06±236.81)×109/L;platelet counts of patients with ET increased significantly(mean: 880.64±354.68)×109/L,mean WBC,HB,and HCT were(14.03±9.17)×109/L,respectively.(124.72±29.59)g/L,(38.77±7.85)%;mean WBC,HB,PLT,HCT in patients with PMF were(10.69±9.30)×109/L,(95.14±46.86)g/L,(277.95).±270.36)×109/L,(30.42±14.15)%.2.Symptom burden scores:(1)The subtype symptom burden score PV patients had fatigue(90.9%),poor mobility(72.7%),abdominal discomfort(45.5%),pruritus(45.5%),bone pain(45.5 %),weight loss(45.5%),inattention(36.4%),night sweats(36.4%),early satiety(27.3%),fever(18.2%).The highest and lowest load symptoms were fatigue [(2.64 ± 1.69)points] and fever [(0.73 ± 1.68)points].ET patients had fatigue(73.1%),poor mobility(42.3%),early satiety(38.5%),pruritus(34.6%),bone pain(34.6%),and 20 to 30% of patients had inattention Concentration(26.9%),night sweats(26.9%),abdominal discomfort(23.1%),weight loss(23.1%),and fever(3.9%)were the lowest.As with PV patients,the highest fatigue load [(3.35±2.37)points] and the lowest fever [(1.11±2.13)points].Patients with PMF had fatigue(84.6%),poor mobility(84.6%),early satiety(76.9%),abdominal discomfort(61.5%),and the remaining symptoms were all less than 50%.Attention was not concentrated.Symptoms of pruritus and pruritus were [(0.54±1.45)points],[(0.15±0.38)points],and the highest rate of fatigue(84.6%)was highest.(2)Total burden: The scores of 50 patients with MPNevaluated by MPN-10 scale were among 0-90,the total burden of three subtypes were PMF(14.49±15.95),PV(12.83±14.52)and ET(13.77±19.77)respectively.(3)Risk stratification 29 patients with PMF were evaluated with the DIPSS system,with a score of 3 points and 4 points in the intermediate-risk group,with 9 cases(62.1%);≤2 points in the middle-risk group,a total of 10 cases(34.5).%),high-risk group,1 case(3.4%);no low-risk patients.2 The total load of 50 patients completed the MPN-10 assessment scale cumulative score between 0 and 90,the total load of each subtype was PMF [(14.49±15.95)points],PV [(12.83±14.52)points] and ET [(13.77±19.77)points].3.Analysis of risk factors for thrombotic events: 30/108 thrombotic event group,78/108 non-thrombotic event group,age ≥60 years(P=0.008),history of hypertension(P= 0.000),JAK2V617 F gene mutation(P=0.034),previous thrombus history(P=0.009)were more likely to have thrombosis.Among the three MPN subtypes,the incidence of thrombosis was highest in PV patients(50%),followed by ET patients(29%)and MF(7%),with a statistically significant difference(P=0.002).Sex,diabetes,hemoglobin,platelet ≥450 X10^9/L(PV,PMF),platelet ≥1000X10^9/L(ET),WBC ≥10X10^9/L were less have thrombosis.In addition to the fatigue and thrombotic events in the MPN-10 scores of PV patients,there was a statistically significant difference(P<0.05).The rest of the score items and total scores were not statistically significant with thrombotic events.4.Correlation between blood viscosity and thrombotic events 43/108 blood viscosity indexes were detected.The apparent viscosity of the thrombosis event group increased at different shear rates and was significantly related to the occurrence of thrombotic events(P<0.05).).Conclusions 1.The median age of MPN was 59.5 year-old.The proportion of the50-59-year-old group and the 70-79-year-old group is the highest.The total positive rate of JAK2 gene expression was 51.8%.The order was PV 70.8%,ET 56.4% and PMF 27.6%.The common causes of the visit were dizziness,fatigue,chest tightness,shortness of breath and other symptoms of anemia,hepatosplenomegaly,bloating,abdominal pain,vomiting and other gastrointestinal symptoms,limb numbness,bleeding symptoms,headache,rash,blurred vision.2.All three subtypes of MPN have fatigue,poor mobility,abdominal discomfort,pruritus,bone pain,weight loss,poor concentration,night sweats,early satiety,and fever.Including the highest incidence of fatigue and load scores.The MPN-10 scale can reflect the patient’s symptom burden and help select the appropriate treatment.The DIPSS system can be used to stratify the risk of PMF patients,with the most in the intermediate-risk group,with no low-risk patients.3.Age>60 years old,JAK2 gene mutation,history of previous thrombosis are risk factors leading to thrombotic events.Hypertension history,increased blood viscosity are also risk factors for the event.In PV patients,fatigue and thrombotic events are significantly related. |
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