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Intracranial hemorrhage (ICH) is a rare and life-threatening hemorrhagic event in patients with immune thrombocytopenia (ITP). However, its mortality and related risk factors remain unclear. Herein, we conducted a nationwide multicenter real-world study of ICH in adult ITP patients. According to data from 27 centers in China from 2005 to 2020, the mortality rate from ICH was 33.80% (48/142) in ITP adults. We identified risk factors by logistic univariate and multivariate logistic regression for 30-day mortality in a training cohort of 107 patients as follows: intraparenchymal hemorrhage (IPH), platelet count ≤10 × 109/L at ICH, a combination of serious infections, grade of preceding bleeding events, and Glasgow coma scale (GCS) level on admission. Accordingly, a prognostic model of 30-day mortality was developed based on the regression equation. Then, we evaluated the performance of the prognostic model through a bootstrap procedure for internal validation. Furthermore, an external validation with data from a test cohort with 35 patients from 11 other centers was conducted. The areas under the receiver operating characteristic (ROC) curves for the internal and external validation were 0.954 (95% confidence interval [CI], 0.910-0.998) and 0.942 (95% CI, 0.871-1.014), respectively. Both calibration plots illustrated a high degree of consistency in the estimated and observed risk. In addition, the decision curve analysis showed a considerable net benefit for patients. Thus, an application (47.94.162.105:8080/ich/) was established for users to predict 30-day mortality when ICH occurred in adult patients with ITP.

Idarubicin 12 mg/m

Primary immune thrombocytopenia (ITP) is a bleeding disorder commonly encountered in clinical practice. The International Working Group (IWG) on ITP has published several landmark papers on terminology, definitions, outcome criteria, bleeding assessment, diagnosis, and management of ITP. The Chinese consensus reports for diagnosis and management of adult ITP have been updated to the 4th edition. Based on current consensus positions and new emerging clinical evidence, the thrombosis and hemostasis group of the Chinese Society of Hematology issued Chinese guidelines for management of adult ITP, which aim to provide evidence-based recommendations for clinical decision making.

Hemophilia is an X-linked recessive inherited bleeding disorder. Despite the improved treatment in recent years with the advent of replacement therapies, the progression of atherosclerosis is not slowed down after the reduction of clotting factors in hemophilia. As life expectancy increases, more hemophilia patients will suffer from age-related cardiovascular diseases. Since cardiac surgery needs heparinization and cardiopulmonary bypass (CPB), it is extremely challenging to balance hemostasis and coagulation in patients with hemophilia. Here we report three cases of hemophilia patients who underwent cardiac surgery successfully.

Background: We reviewed patients with fever of unknown origin (FUO) and splenomegaly and assessed the diagnostic value of splenectomy and measured risk factors suggestive of an underlying lymphoma. Methods: FUO patients (n = 83) who had splenomegaly and underwent splenectomy were enrolled into this retrospective single-center study. Clinical presentations were documented and risk factors suggestive of an underlying lymphoma were tested. Results: Seventy-four patients (89.2%) had a diagnosis of lymphoma or not after splenectomy and follow-up. Of those (55.4%) diagnosed with lymphoma, 29 had B-cell non-Hodgkin lymphoma and 12 had T-cell non-Hodgkin lymphoma. The remaining 33 (44.6%) had diseases other than lymphoma. Using multivariate logistic analysis, the following 3 independent risk factors were found to be related to a final diagnosis of lymphoma: age (continuous) (HR 1.086; 95% CI 1.033-1.141; p = 0.001), massively enlarged spleen (HR 7.797; 95% CI 1.267-47.959; p = 0.027), and enlarged intra-abdominal lymph nodes (HR 63.925; 95% CI 7.962-513.219; p < 0.001). The calibration of the model was satisfactory (p = 0.248 using the Hosmer-Lemeshow test), and the discrimination power was good (area under the receiver operating characteristic curve 0.925; 95% CI 0.863-0.987). Conclusions: Splenectomy is an effective diagnostic procedure for patients with FUO and splenomegaly and lymphoma is a common cause. Older age, a massively enlarged spleen, and enlarged intra-abdominal lymph nodes are risk factors suggesting an underlying lymphoma, and surgery for high-risk patients should be considered.

CD200 and its receptor CD200R are both type-1 membrane glycoproteins, which are members of the immunoglobulin superfamily (IgSF). Besides the inhibitory effect on macrophages, CD200/CD200R also play an important role in regulating the regulatory T cells, allergicreaction, autoimmune diseases, allograft, neurological diseases and other autoimmune-related diseases, etc.To investigate the role of CD200 and its receptor in the graft versus host disease (GVHD).Experimental samples were divided into aGVHD group, non-aGVHD group, cGVHD group and non-cGVHD group, the healthy persons were used as normal controls. Firstly, the expression levels of CD200 and CD200R on CD19+ cell, CD3+ cell and dendritic cell (CD19- CD14- CD1c+) surfaces in each group were detected by using flow cytometry, so as to determine whether there were expression differences among each groups. Then, the mRNA levels of each groups were tested by using real-time quantitative polymerase chain reaction for finding the differences of mRNA expression level among each group. Finally, the peripheral blood mononuclear cells of the patients and healthy controls were co-cultured with anti-CD200R1 antibody for 48 hours, and the interleukin-10 level in the co-culture system was tested by using enzyme linked immunosorbent assay for verifying the function of CD200/CD200R.The CD200 expression level on CD19+ cell surface in the aGVHD group and non-aGVHD group was both lower than that in healthy control group; that in the non-aGVHD group was higher than that in the aGVHD group. The CD200 expression level on CD19+ CD200+ cells in the non-cGVHD group were higher than that in the cGVHD group and healthy control group. There were no significant differences of CD200 and CD200R expression levels on CD3 cells and dendritic cells among all groups. The CD200 mRNA expression levels in the aGVHD group and cGVHD group were both lower than the healthy control group. The CD200 mRNA expression level was lower in the aGVHD group than in the non-aGVHD group, and was lower in the cGVHD group than in the non-cGVHD group. There was no significant difference of the CD200R mRNA expression level among all groups. After the peripheral blood mononuclear cells of the patients and healthy controls were co-cultured with anti-CD200R1 antibody for 48 hours, the interleukin-10 concentration decreased with the increasing of anti-CD200R1 antibody concentrations in the co-culture system.The CD200/CD200R may play a role in the pathogenesis of GVHD after allo-HSCT.

To investigate the clinical features and outcome of patients with acute leukemia and Aeromonas sobria infection.The clinical data of two patients from Peking Union Medical College Hospital from 1994 to 2014 as well as patients reported in English literature (from PubMed and Medline databases) and Chinese literature (China Hospital Knowledge Database) during the same period were retrospectively analysed. Data were extracted and analyzed using Chi-square tests.Totally 15 patients worldwide were available for analysis with a male-to-female ratio of 12:3 and an acute myeloid leukemia to acute lymphoblastic leukemia ratio of 10:5. The median age was 49.5 years (range:27-80 years). Most patients were neutropenic at the time of onset of fever,and the most common manifestation was lower limb infection (66.7%). Despite empirical antibiotics treatment,the mortality was still as high as 80%. Chi-square tests revealed that among the dead patients,the rates of lower limb infection (P0.01) and septic shock (P=0.02) were significantly higher.Aeromonas sobria infection is an uncommon but highly fatal condition that requires special attention in clinical settings.

To investigate the incidence, pathogens, and clinical features of infection in consecutive cases from 2010 to 2012 in Peking Union Medical College Hospital.The incidence, pathogen, treatment, and outcomes of patients with hematological diseases who had positive findings of bacterium in their samples from 2010 to 2012 were retrospectively analyzed.There were 449 positive samples (5.8%) from 4 890 patients during this period, among which 388 were proved to be with pathogenic bacteria. Samples separated from patients with community-aquired infections accounted for 8.4% of all positive samples. Most community-aquired infections were caused by Gram-negative bacteria (75%), although no multidrug-resistant bacteria was observed. Samples separated from patients with nosocomial infections accounted for 91.6% of all positive samples. Respiratory tract (49.4%) and peripheral blood (32.6%) were the most common samples with positive results. Skin soft tissues (10.4%), and urine (3.7%) were less common samples. Most of the pathogenic bacteria of the nosocomial infections were Gram-negative (66.9%). The most common Gram-negative bacteria included Escherichia coli (13.8%), Pseudomonas aeruginosa (12.1%), and Klebsiella pneumonia (12.1%), while Staphylococcus aureus (10.4%), Enterococcus faecium (7.0%), and Staphylococcus epidermidis (5.1%) were the most common Gram-positive bacteria. Gram-negative bacteria consisted of most of sputum samples and peripheral blood samples. Samples from the surface of skin wound and anal swab were composed largely by Gram-positive bacteria (63.8%). The detection rates of extended-spectrum beta-lactamase-producing Klebsiella pneumonia/Klebsiella oxytoca, Escherichia coli, and Proteus mirabilis were 24.0%, 87.9% and 38.4%, respectively. The resistance to Acinetobacter baumannii was serious. Multidrug-resistant, extensive drug resistant and pan drug resistant A. baumannii acountted for 74% of all A. Baumannii infections. Stenotrophomonas maltophilia showed low resistance to sulfamethoxazole/trimethoprim, levofloxacin and minocycline. Also, 22 methicillin-resistant Staphylococcus aureus and 9 methicillin-resistant Staphylococcus Epidermidis were detected, which were only sensitive to vancomycin, teicoplanin, and linezolid. All patients were treated in the haematology wards and most of them were under agranulocytosis or immunosuppression. Finally, 22 patients reached clinical recovery through anti-infective therapy, whereas 49 patients died. Among those deaths, 42 patients attributed to severe infections and infection-associated complications. Fourteen of all the deaths might be infected with drug-resistance bacteria. There were 61 samples proved to be bacteria colonization. Nonfermenters such as Acinetobacter baumannii and Stenotrophomonas maltophilia made up for a large amount of bacteria colonization.The pathogens of nosocomial infections in the hematology ward are mainly Gram-negative bacteria. The incidences and pathogens vary from different infection sites. Nosocomial infection still has a higher mortality rate. Once nonfermenters are detected positive, the pathogenic or colonial bacteria should be distinguished.

To evaluate the efficacy and safety of prophylactic ceftazidime on early bacterial infection in APBSCT recipients during neutropenia.APBSCT recipients were prospectively randomly assigned to intravenous ceftazidime treatment group and control group (no prophylaxis of antibiotics). The treatment started from the first day until resolution of neutropenia or the appearance of early bacterial infection.From March 2010 to January 2013, 70 APBSCT recipients were enrolled in the study with 36 in treatment and 34 in control group. Overall, 29(41.4%) patients developed early bacterial infection, among which, 9(25.0%) in the treatment group and 20(58.8%) in the control group (P = 0.004). The median infection free survival (IFS) was not reached in the treatment group and was 8 days in the control group (P = 0.005). Despite whether patients received single high dose melphalan or other conditioning regimes, the early bacterial infection rate was lower in the treatment group than in the control group, and the median IFS was longer in the treatment group than that in the control group. The mean courses of antibiotic administration were (8.08 ± 2.03) days and (3.68 ± 3.56) days respectively in the treatment and control groups (P0.001). However, the duration of empirical carbapenems were (1.67 ± 3.03) days and (3.68 ± 3.56) days respectively (P = 0.013). There was no significant difference of antibiotics cost per patient between the two groups. Four patients in the treatment group had a transient elevated serum creatinine. Overall, no infection related mortality was observed in either group.Prophylaxis of intravenous ceftazidime for APBSCT recipients is effective in preventing early bacterial infection with an acceptable toxicity and cost profile. However, it doesn't have effect on infection related mortality. Therefore, our results do not support the use of antibiotic prophylaxis for patients undergoing APBSCT.

To analyze the clinical features, diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) in patients with systemic lupus erythematosus (SLE).Clinical manifestations, laboratory findings, diagnosis,treatment and prognosis of 14 SLE patients with TTP were retrospectively analyzed.Of the 14 patients diagnosed with SLE and TTP, 4 were men and 10 were women. The median age at diagnosis was 23 (17-69) years old. In five patients, the onset of SLE preceded TTP, and in nine patients SLE and TTP occurred simultaneously. All the 14 patients had thrombocytopenia and hemolytic anemia, 12 had fever, 11 had neurologic abnormalities, and 11 had renal dysfunction. Eight patients presented with the classic pentad of symptoms. Six patients were given steroids (alone or in combination with intravenous immunoglobulin and cyclophosphamide), and eight patients were treated with steroids in combination with plasmapheresis, with response rates of 2/6 and 6/8, respectively. Six patients died, with overall mortality rate of 6/14. No patients relapsed during the follow-up period.SLE and TTP share some similar clinical symptoms. As a result, repeated examinations of peripheral blood smears are very important for early diagnosis. The renal damage in patients of co-existing diseases is more serious than those with TTP alone or SLE alone. Early diagnosis and prompt treatment with plasma exchange and steroids may improve the prognosis in SLE patients with TTP.

To analyze efficacy of radiotherapy for adult patients with Langerhans cell histiocytosis (LCH).Clinical features and efficacy of radiotherapy for biopsy-proven adult patient with LCH from January 2000 to October 2012 in our hospital were retrospectively analyzed.Seventeen (11 male and 6 female) adult LCH patients with a mean age of 31 (18-56) years old were treated by irradiation, all patients presented as single-system disease. The mean duration from diagnosis to irradiation was 8.3 (0-108) months. Although 12 of 17 patients (70.6%) had short-term response to radiotherapy, all patients but one (94.1%) progressed during long-term follow-up, the mean progression-free survival (PFS) was 14 (0-131) months. Of the progressed patients, one relapsed in situ, the remaining 15 patients progressed outside the irradiated region. Thirteen patients (76.5%) eventually progressed to multisystem disease.Though radiotherapy for LCH in adults produced a high short-term response up to 70.6%, most of patients eventually progressed in situ or outside the irradiation region during long-term follow-up.

To evaluate the efficacy and safety of rituximab in treating patients with refractory and/or relapsing thrombotic thrombocytopenic purpura (TTP).Totally three patients received rituximab as salvage therapy in our hospital. Rituximab was administered at a weekly dose of 375 mg/m(2) for 2 or 4 consecutive weeks. After clinical remission, patients were followed up every 3 months.All three patients achieved complete remission. The median time to platelet count recovery was 7 days (4-12 days) after the first rituximab infusion. During the follow-up (median: 12 months; range: 9-18 months), no patients experienced relapse. No side effect was noted during treatment and follow-up period.Therapy with rituximab is effective and well tolerated for patients with refractory or relapsing TTP.

To explore the distribution and influence factors of protein C (PC), protein S (PS) and antithrombin (AT) activities and to determine the prevalence of their deficiencies in the Chinese Han healthy population.Healthy volunteers including blood donors and individuals for routine check-up were recruited from 4 Chinese medical centers. The plasma levels of PC, PS and AT activities were measured. The plasma levels of activities were measured by chromogenic substrate assay (AT and PC) and clotting assay (PS).A total of 3493 healthy Chinese adults had been recruited in this study. Males had higher PS and PC activities than females, especially for PS (P0.01). PC activities increased with age in both sexes but decreased in men after 50 years old. There was no significant change with age were of PS in 50 years old, while there was a decline in males and a rise in females above 50 years old. AT tended to increase with age in women but decreased with age in men after 50 years old. Based on the age and gender, the general prevalence of PC, PS and AT deficiencies in the general Chinese Han population were 1.15%, 1.49% and 2.29%, respectively.PC, PS and AT activities have correlation with age and gender in Chinese Han population. Reference range should be laid down and deficiencies should be identified

To evaluate the efficacy of porcine anti-human lymphocyte globulin (P-ALG) plus cyclosporine A (CsA) therapy for severe aplastic anemia (SAA).Forty-eight SAA patients (31 males, 17 females) including 17 very severe aplastic anemias (vSAA) were treated with ALG plus CsA between 1999 to 2009 in our hospital and the outcomes were analyzed retrospectively for early mortality, response rate and quality, survival rate, toxicity and complications.The median age was 28 (13 - 64) years. The interval from diagnosis to treatment was 45 days. The median neutrophil count at diagnosis was 0.178 × 10(9)/L. Overall response was 83.3% (54.2% complete, 29.2% partial) with a median time of 90 (23 - 380) days. 10.4% died of infection within 30 days mainly of fungi infection. Only 1 patient relapsed 2 years after treatment. No clonal disease was found. The 1.5-year survival rate was 87.5%. vSAAs had less response, higher early mortality and less survival (64.7%, 29.4% and 51.8%, respectively) compared to that of SAA (93.5%, 0, 100%, respectively, P0.05). Grouped patients with different age, gender, intervals between diagnosis and treatment and pre-existing infections had similar response. The main side effects were fever and skin rash (52.1%), serum sickness (16.7%), impaired liver function (60.4%) and hemorrhage (2.1%). No treatment-related mortality was found.P-ALG plus CsA is an ideal and well tolerated treatment for SAA but not for vSAA.

To evaluate the efficacy and safety of autologous peripheral blood stem cell transplantation (auto-PBSCT) after high dose melphalan in patients with POEMS syndrome.Nine patients including 6 males and 3 females received 10 auto-PBSCT after high dose melphalan in our hospital from June 2005 to October 2009. The median age at transplantation was 44 (39 - 48) years. The median time from onset of disease to transplantation was 12 (5 - 60) months. Peripheral stem cells were mobilized by G-CSF alone in one patient and 8 patients by G-CSF plus chemotherapy. Two patients were conditioned by melphalan 140 mg/m(2) and 7 by melphalan 200 mg/m(2). The median number of MNC was 3.75 (1.05 - 8.33) × 10(8)/kg, and that of CD34(+) cell was 5.37 (1.32 - 10.90) × 10(6)/kg.One patient received tandem auto-PBSCT and others received single one. Stem cell engrafted in all but 1 patient who died of severe infection and acute renal failure on day 9 after transplantation. Eight patients were evaluable for response. The median time to ANC ≥ 0.5 × 10(9)/L and platelet ≥ 20 × 10(9)/L was 10 (9 - 11) and 11.5 (9 - 14) days respectively. Two patient reached negative immunofixation electrophoresis (IFE) after stem cell mobilization and transplantation respectively, and the other 6 remained IFE postive after auto-PBSCT. Skin changes and edema of lower extremities were improved in 5 of 6 patients, lymphadenopathy relieved in 1 and papilledema improved in 2 of 3 patients. All but 1 patient achieved gradual neurologic improvement after transplantation.PBSCT is an effective and safe therapy for POEMS syndrome patients with low treatment related mortality.

To compare the efficacy and adverse effects between arsenic trioxide (ATO) and all-trans retinoic acid (ATRA) in patients with acute promyelocytic leukemia (APL).The clinical data of 71 patients with newly diagnosed APL were retrospectively analyzed. Two groups were classified according to the induction regimens, namely ATO group (n = 41) and ATRA group (n = 30). The complete remission (CR) rate and the time to CR were compared between these two groups.The CR rate was 97.5% in ATO group and 93.3% in ATRA group (P0.05). The median time to CR was 29 days (21-45 days) in ATO group, which was significantly shorter than 38.5 days (24-63 days) in ATRA group (P0.001). Retinoic acid syndrome occurred in 52.9% of patients treated with ATRA, which affected the further use of ATRA.Both ATO and ATRA have high response rates for newly diagnosed patients with APL. Compared with ATRA, ATO induction therapy has shorter time to achieve CR and less adverse effects, and therefore may be the first-line therapy for APL.

To explore the frequency, clinical features and risk factors of venous thromboembolism (VTE) in hospitalized patients.The frequency, demographic features, and acquired and inherited factors of in-patient cases of VTE in Peking union medical college hospital from 1994 to 2004 were analyzed retrospectively.Six hundred and seventy-two patients were enrolled. Among them, male to female ratio was 1.2 and the median age was 53 (14 - 92). Five hundred and eighty (86.3%) patients were at their first diagnosis with the peak ages between 40 and 50 for men and 50 and 60 for women. More common acquired risk factors were antiphospholipid antibody syndrome (APS) (32.0%), trauma / surgery (31.1%) and malignancies (17.1%). 35.7% of the patients had multiple acquired risk factors. Before the initiation of anticoagulation therapy, the activities of protein C (PC), protein S (PS) and antithrombin (AT) were measured in 94 patients. The deficiency of these three natural anticoagulants was 44.7%. Among the anticoagulant deficiencies, PC deficiency was the commonest one (13.8%). Combined deficiency of PC and AT accounted for 10.6%. 31.6% of the 94 patients had inherited plus acquired risk factors.Age for the first event of VTE in the men was about 10 years ahead of that in the women. The major acquired risk factors were APS, trauma/surgery and malignancies, and inherited risk factors were PC deficiency and PC + AT combined deficiencies. It seems that the coexistence of multiple risk factors plays an important role in triggering VTE.

To investigate the incidence of infection and pathogens in hematology ward.The data of incidence, pathogen, and outcome of infection of 2388 hospitalized patients in an open hematology ward of Peking Union Medical College Hospital from 1993 to 2004 were analyzed retrospectively.The overall incidence of infection was 34.3% according to the person-times of hospitalization, 24.4% for nosocomial infection and 9.9% for community-acquired infection. Most of the pathogenic bacteria of the nosocomial infection were Gram negative. The most common bacteria in the sputum samples included Enterobacter (27%), Pseudomonas aeruginosa (16%) and coagulase negative Staphylococcus (MRSCoN, 12%), the most common bacteria in the blood samples included Escherichia coli (43%), Enterobacter cloacae (11%), and Klebsiella (11%). Whereas in the community-acquired infection the most common bacteria in the sputum samples were Haemophilus parainfluenzae (15%), MRSCoN (28%), and Staphylococcus epidermidis (10%), and the most common bacteria in the blood samples were MRSCoN (28%), E. coli (28%), and Klebsiella (9.4%). Fungi were more often found in nosocomial infection, especially in the sputum samples. 12-year follow up showed that in nosocomial infection Pseudomonas aeruginosa remained the most common bacteria in the sputum samples, whereas E. coli and Enterobacter cloacae became the major bacteria in the blood samples. In community-acquired infection, the proportion of Staphylococcus aureus, that of Klebsiella in blood samples, and that of E. coli in throat swab samples increased in recent years. The incidence of fungi infection had increased in both nosocomial infection and community acquired infection. The mortality of nosocomial infection was 6.1%, higher than that of the community-acquired infection, however, not statistically significant (P = 0.17). There was a trend of decrease in the mortality of community-acquired infection but did not in the nosocomial infection.The patients in hematology ward are susceptible to infection, especially nosocomial infection that has a higher mortality rate in comparison with the community-acquired infection, however, not statistically significant. The pathogens of nosocomial infection are most likely G- bacteria, fungi and other bacteria resistant to most antibiotics. The mortality rate of nosocomial infection remains almost unchanged in the 12-year follow up.

To investigate the distribution frequencies of angiotensin-converting enzyme (ACE), angiotensinogen (AGT), angiotensin II I type receptor (AT1R) genotypes in Chinese, to find the relationships between polymorphisms of ACE, AGT and AT1R gene, and coronary artery thrombosis disease (CATD) and to study the interactions of themselves, PCR and PCR-RFLP techniques were performed to determine the genotypes of ACE, AGT and AT1R gene in CATD group (192 cases) and control group (110 cases). The results showed that (1) genotype frequencies of the three polymorphisms in the control group were 12.2% (DD), 43.9% (ID), and 43.9% (II) for the ACE I/D polymorphism; 8.2% (MM), 36.7% (MT), and 55.1% (TT) for AGT M235T polymorphism; 91.8% (AA), 8.2% (AC) for AT1R A1166C polymorphism respectively; (2) there were no significant differences between patients in either the control group, the non-MI group, or the MI group in any genotype frequency of all these three genes (P0.05). (3) the odds ratio for CATD in subjects carrying both AT1R-AC and AGT-TT genotype was 3.517 (95% CI 0.988 - 12.527), compared with those carrying AT1R-AA and AGT-TT genotype and was 15.000 (95% CI 1.940-115.963), compared with those carrying AT1R-AC and AGT-MM/MT genotype. In subjects with AT1R-AC genotype, there was also a great difference of ACE D allele frequency between control group and CATD group (P=0.017). It is concluded that genotype frequencies of ACE I/D polymorphism, AGT M235T polymorphism, and AT1R A1166C polymorphism were obviously different from those in western countries. Although these three polymorphisms were not independent risk factors for CATD or myocardial infarction (MI) in Chinese, AT1R-AC genotype has a significant synergistic effect with AGT-TT genotype. There is also a obvious interaction between AT1R-AC genotype and ACE D allele.