Retrospectively analysis of the difference of bleeding frequency and hemophilic arthropathy between hemophilia A and hemophilia B patients / 中华血液学杂志

Objective@#To analyze the difference of bleeding frequency, plain radiographic (X-ray) , risk factors in hemophilic arthropathy progression and the Arnold-Hilgartner classification.@*Methods@#A retrospective study was conducted in 211 hemophilia patients hospitalized in our medical center between January 2007 and December 2010, some patients with hemarthrosis were followed up for 5 years.@*Results@#All patients were male, including 150 hemophilia A (HA) and 61 hemophilia B (HB) . The HA patients bled more frequently than HB patients with annualized total bleeding rate 20.5 (0-48) vs 13 (1-40) ; annualized joint bleeding rate 13.5 (0-38) vs 8 (0-33) , especially in moderate hemophilia [26 (1-48) vs 12 (1-36) , P<0.001; 18 (0-36) vs 7.5 (0-26) , P=0.001], but severe hemophilia had no difference in bleeding frequency [33 (1-41) vs 26 (1-40) , P=0.702; 22 (0-36) vs 18 (0-33) , P=0.429]. The condition of the affected joints of 108 HA and 54 HB was evaluated on roentgenography. In HA patients, the Arnold-Hilgartner classification increased with the severity ratings (r=0.063, P=0.004) . However, similar associations were not found in HB patients (r=0.045, P=0.082) . Five years later, 36 HA and 19 HB patients received the same joint X-ray, there were no significant differences in joints radiographic progression between the total HA and HB groups (z=1.941, P=0.052) . However, significant difference between moderate HA and HB was observed (z=0.076, P=0.002) . Multivariate unconditioned Logistic analysis showed that annualized joint bleeding rate [P<0.001, OR=1.166 (95%CI 1.097-1.239) ] and articular structural injuries [P=0.018, OR=2.842 (95% CI 1.196-6.755) ] were independent risk factors for the joints radiographic progression.@*Conclusion@#The study suggests that there was a difference in bleeding phenotype between HA and HB, especially in moderate hemophilia. HB patients showed mild but progressive development over time, compared with HA patients. Annualized joint bleeding rate and articular structural injuries were independent risk factors for the joints radiographic progression.

Predictors of fatigue among individuals with primary immune thrombocytopenia in China / 中华血液学杂志

Objective@#To study the fatigue symptoms of adult patients with primary immune thrombocytopenia (ITP) and to analyze the possible factors that affect the severity of fatigue.@*Methods@#Eligible adult patients with ITP who admitted to Institute of Hematology & Blood Diseases Hospital were enrolled in this study and the questionnaires including a Chinese version of the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue) , the Pittsburgh sleep quality index (PSQI) , the Hospital Anxiety and Depression scale (HADS) and demographic information were completed. The predictors of fatigue were determined with multiple linear regression analyses.@*Results@#A total of 207 patients with ITP were enrolled, including 70 males (33.8%) and 137 females (66.2%) , the median age was 42 (18-72) years old. The FACIT-F score in ITP patients was (37.50±9.05) . The FACIT-F severity of ITP patients was positively correlated with the platelet count (r=0.307, P<0.001) . The FACIT-F severity was negatively correlated with bleeding severity (r=-0.276, P<0.001) , sleep quality (r=-0.654, P<0.001) , depression (r=-0.598, P<0.001) and anxiety (r=-0.616, P<0.001) . A multiple linear regression analysis revealed that the severity of ITP-related fatigue was significantly correlated with platelet count (P<0.001) , bleeding severity (P=0.004) , sleep quality (P<0.001) and depression (P<0.001) .@*Conclusion@#Fatigue was determined by complicated factors in adult ITP patients. Interventions addressing depressive symptoms, sleep quality, bleeding symptoms and platelet count could be potential avenues for treatment of fatigue in patients with ITP.

Lupus anticoagulant: two cases report and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To deepen the understanding of clinical manifestations and treatment of patients with positive lupus anticoagulant (LAC).</p><p><b>METHODS</b>The clinical data of 2 patients were analyzed and related literature were reviewed.</p><p><b>RESULTS</b>Case 1, a 31-year-old female, diagnosed as lupus anticoagulant positive, secondary to undifferentiated connective tissue disease, was presented with menorrhagia and thrombocytopenia. Anti-nuclear antibody (ANA) was positive 1:1000 (homogeneous type) with anti-double stranded DNA positive, and dRVVT LA1/LA2 was 3.4. Coagulation function was alleviated after treatment with glucocorticoid and total glucosides of paeony. Case 2, a 59-year-old female was presented with gingival bleeding, hematuria with the level of F II:C 13%. dRVVT LA1/LA2 was 2.0. Anti-nuclear antibody (ANA) was positive 1:1000 (type of cytoplasmic granule), anti-double stranded DNA was positive. The patient was diagnosed as hypoprothrombinemia-lupus anticoagulant syndrome (LAHS) and acquired coagulation factor deficiency. The signs of hemorrhage were alleviated after treatment with methylprednisolone 40 mg/day and cyclophosphamide, while the level of F II:C was below normal.</p><p><b>CONCLUSION</b>Symptoms of patients with positive LAC are variable. The diagnosis relies on history of disease and laboratory test. Currently, there is no standardized treatment. Cases of LAHS should be thoroughly investigated for any known causes and related disorder.</p>

Surgical resection of huge non-hepatic tumors in the right upper quadrant of abdomen / 中华肝胆外科杂志

Objective To study the pathology and treatment of huge nonhepatic tumors in the right upper quadrant of abdomen.Methods The clinical data of 9 patients with huge nonhepatic tumor in the right upper quadrant of abdomen treated surgically at our hospital from May 2004 to December 2009 were retrospectively analyzed.Results Preoperative imaging failed to define the tumors as nonhepatic in original in 7 patients and operation failed to recognize the origin of the tumors in 2 patients.All the tumors were successfully resected,with combined hemigastectomy in 1 patient,partial resection of the lateral wall of the infrahepatic vena cava in 2,complete resection of adipose capsule of the right kidney in 2,pancreatoduodenectomy plus transverse colectomy in 1,and transection of pancreatic duct of the body and tail of the pancreas and pancreaticojejunostomy in 1.The median operation time was 390 min (318-660 min).The median intraoperative blood loss was 2560 ml (400-6000 ml).The median intraoperative blood transfusion was 2450 ml (0 -5250 ml).The average diameter of the resected tumor was 14.5 cm (11-30 cm),and the average tumor weight was 2465 g (960-5100 g).Postoperative pathological diagnoses showed that 8 patients had malignant tumors and 1 had a potentially malignant and undifferentiated tumor (solid pseudopapillary tumor of pancreas).Perioperative pancreatic anastomotic leak occurred in 1 patient,and there were no severe postoperative complications and operative death in this series.Tumor recurrence was detected 5 months following operation in 1 patient.The 1,2-,3-year survival rates were 100％,56％,33％,respectively.One patient survived for more than 5 years.Conclusions Huge non-hepatic tumors in the right upper quadrant of abdomen could easily be misdiagnosed as hepatic neoplasms.The surgical resection rate was high.The prognosis for patients who received resectional treatment was satisfactory.