Background Philadelphia (Ph) chromosome-negative myeloproliferative neoplasms (MPNs) are a heterogeneous group of hematopoietic stem cell clonal diseases. Most patients with MPN are asymptomatic at diagnosis although some of them suffer from constitutional symptoms. Thrombosis and bleeding can also be one of the initial manifestations although the reported prevalence varied considerably across the studies. This systematic review and meta-analysis was conducted with the aims to better understand the prevalence and characteristics of thrombosis and bleeding among patients with newly-diagnosed MPN. Methods Using a search strategy that included the terms for myeloproliferative neoplasms, thrombosis, and bleeding, two investigators independently searched for published articles indexed in the MEDLINE and EMBASE databases from inception to August 2018. The pooled prevalence was calculated using the DerSimonian–Laird random-effects model with a double arcsine transformation. Results A total of 29 cohort studies (8 prospective and 21 retrospective) with 13,436 patients with MPN were included into this meta-analysis. At diagnosis, the pooled prevalence of overall thrombosis among patients with MPN was 20.0% (95% CI, 16.6–23.8%; I 2 96%), with the pooled prevalence of arterial thrombosis of 16.2% (95% CI, 13.0–20.0%; I 2 95%) and the pooled prevalence of venous thrombosis of 6.2% (95% CI, 4.9–7.8%; I 2 89%). Common thrombotic events included cerebrovascular disease/transient ischemic attack, coronary heart disease, and deep venous thrombosis. The pooled prevalence of hemorrhagic complications among patients who were newly diagnosed with MPN patients was 6.2% (95% CI, 5.0–7.8%; I 2 85%). Common sites of bleeding included gastrointestinal, mucosal, and cutaneous bleeding. Conclusions Thrombosis and bleeding are common initial manifestations of MPN. Investigations for MPN should be considered for patients who present with unexplained thrombosis or abnormal bleeding. Electronic supplementary material The online version of this article (10.1186/s12885-019-5387-9) contains supplementary material, which is available to authorized users.

The current systematic review and meta-analysis aimed to summarize the results of all available studies to compare the efficacies of rituximab and conventional treatment for acquired thrombotic thrombocytopenic purpura (TTP). Three investigators independently searched studies in the MEDLINE and EMBASE databases published before December 11, 2018. To be included in the meta-analysis, studies needed to be randomized-controlled or cohort studies comparing the efficacies of rituximab and conventional therapy for TTP treatment. The effect estimates and 95% confidence intervals (CIs) from each study were collected, and Mantel-Haenszel methods were used to pool the data. A total of 570 patients from 9 eligible studies were included in the meta-analysis (280 patients in the rituximab arm and 290 in the conventional treatment arm). Patients receiving rituximab in an acute phase to induce disease remission had a significantly lower relapse rate than those given conventional treatment (odds ratio [OR]: 0.40, 95% CI: 0.19-0.85, P = .02, I 2 = 43%). Similarly, the relapse rate in the rituximab group for preemptive therapy to prevent clinical relapse was also significantly lower than in the control group (OR: 0.09, 95% CI: 0.04-0.24, P < .00001, I 2 = 11%). Furthermore, the conventional treatment group had a significantly higher mortality rate than the rituximab group during the follow-up (OR: 0.41, 95% CI: 0.18-0.91, P = .03, I 2 = 0%). Rituximab offered high efficacy for the prevention of relapses and lower mortality rate in cases of acquired TTP.

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BackgroundOutpatient autologous stem cell transplantations (ASCTs) in multiple myeloma and lymphoma patients have been shown to reduce the overall costs and improve the quality of life relative to inpatient ASCTs. This systematic review and meta-analysis was performed with the aim of comprehensively comparing the risk of febrile neutropenia developing in ASCT outpatients and inpatients who have multiple myeloma or lymphoma.MethodsTo be eligible for the meta-analysis, studies needed to be either randomized, controlled studies or cohort studies. They also need to have two groups of patients with multiple myeloma or lymphoma who underwent ASCT, with the treatment being provided to one group in an outpatient setting and to the other on an inpatient basis. The studies had to report our primary outcome of interest, the rate of febrile neutropenia after stem cell infusion, for both groups. The Mantel–Haenszel method was used to pool the effect estimates and 95% confidence intervals of each study.ResultsFrom 9 eligible studies, a total of 1940 patients were included in the meta-analysis. Contrary to conventional concerns, the patients who underwent the outpatient ASCT had a significantly lower risk of developing febrile neutropenia than those admitted for ASCT, with a pooled odds ratio (OR) of 0.44 (95% confidence interval [CI]: 0.29–0.65; p < 0.0001; I2 = 52%). The risk of septicemia was also significantly lower for the outpatients than the inpatients, with a pooled OR of 0.40 (95% CI: 0.16–0.97; p = 0.04; I2 = 23%). Additional analyses found that the odds of having grade 2–3 mucositis and transplant-related mortality were numerically lower for the outpatient group, although the pooled result was not statistically significant. The odds of surviving at 2–3 years was also numerically higher for the ASCT outpatients, but the difference did not reach statistical significance.ConclusionsThis study found a significantly lower odds of developing febrile neutropenia and septicemia among patients with multiple myeloma and lymphoma who received an outpatient ASCT than among those who had an inpatient ASCT.Electronic supplementary materialThe online version of this article (10.1186/s12885-018-5054-6) contains supplementary material, which is available to authorized users.

BackgroundImmune thrombocytopenia is an acquired autoimmune disease. Recently, there has been evidence of thrombotic risk in patients with immune thrombocytopenia, but the mechanism is still inconclusive. Intravenous immunoglobulin infusion therapy is considered an efficient treatment; however, it still is associated with adverse events of fever, chills, and hypotension, as well as serious complications such as thrombosis. We report a case a patient with relapsed immune thrombocytopenia who developed ischemic stroke after an intravenous immunoglobulin infusion.Case presentationA 49-year-old Thai woman with relapsed/refractory immune thrombocytopenia came to our hospital with a large hematoma at the right buttock, and her platelet was decreased to 3 × 109/L. She was admitted to our hospital for intravenous immunoglobulin administration. One hour after completion of intravenous immunoglobulin infusion, the patient’s sister complained that the patient was unconscious and could not move both legs and arms. Emergency computed tomography of the brain showed no abnormal findings, such as brain edema, intracranial hemorrhage, or infarction. One day later, repeat computed tomography of the brain displayed extensive acute ischemic changes and loss of gray-white differentiation of bilateral cerebral hemispheres.ConclusionsWe performed an extensive literature review to determine the possible causes of serious thrombotic events in immune thrombocytopenia between the predictive factors of the disease and intravenous immunoglobulin. Although intravenous immunoglobulin is an effective treatment, thrombotic complications can occur. We emphasize that in patients with atherosclerosis risk factors or thrombophilia, the appropriateness of administering an intravenous immunoglobulin infusion should be carefully evaluated.Electronic supplementary materialThe online version of this article (10.1186/s13256-018-1955-x) contains supplementary material, which is available to authorized users.

The complete response rate after the first course of induction therapy was significantly greater among adult patients with AML who had received IDA as part of induction therapy compared with those who had received HDD.

Acute myeloid leukemia (AML) with mutated RUNX1 (RUNX1mut) is considered to have an unfavorable prognosis. However, recent studies have reported comparable survival outcomes with wild-type RUNX1 (RUNX1wt). To assess the clinical outcomes of AML with and without RUNX1mut, we performed a prospective cohort study and systematic review and meta-analysis. The study enrolled 135 patients (27 with RUNX1mut; 108 with RUNX1wt). There were no significant differences in the median OS and RFS of the RUNX1mut and RUNX1wt groups (9.1 vs. 12.2 months; p = 0.268 and 7.8 vs. 14.6 months; p = 0.481, respectively). A subgroup analysis of de novo AML patients with intermediate-risk cytogenetics showed similar outcomes. Our meta-analysis pooled data from 23 studies and our study. The complete remission rate was significantly lower in the RUNX1mut group (pooled odds ratio: 0.42). The OS, RFS, and event-free survival rates also favored the RUNX1wt group (pooled risk ratios: 1.36, 1.37, and 1.37, respectively). A subgroup analysis of de novo AML patients with intermediate-risk cytogenetics demonstrated nearly identical OS and RFS outcomes. This study confirms that patients with AML and RUNX1mut had poor prognoses. Nonetheless, in de novo AML with intermediate-risk cytogenetics, the survival outcomes of both groups were comparable.

Philadelphia (Ph)-like acute lymphoblastic leukemia (ALL) has disease phenotypes similar to Ph D ALL. This meta-analysis aimed to include cohort study of patients with ALL who reported the prevalence of Ph-like ALL. Across the 15 included studies, the most common gene subgroup was CRLF2 gene rearrangement, and the peak prevalence of Ph-like ALL occurred in adolescents and young adults. Background: The presence of Philadelphia (Ph)-like ALL among patients with acute lymphoblastic leukemia (ALL) may indicate a poor prognosis similar to Ph þ ALL, although the data are still inconclusive and the prevalence of Ph-like ALL varied considerably across studies. Patients and Methods: We performed a systematic review and meta-analysis in order to identify all cohort studies of patients with ALL that reported the prevalence of Ph-like ALL and to summarize their results together. The pooled prevalence and rate were calculated by the DerSimonian-Laird random-effect model with double arcsine transformation. Results: Across the 15 included studies describing 11,040 ALL patients, the peak prevalence of the presence of Ph-like ALL among patients with ALL was between ages 11 and 40 years, where the pooled prevalence was 25.8% to 26.2%. The pooled 5-year overall survival rate of Ph-like ALL was 42.8% (95% confidence interval, 23.9-64.1; I 2 93%). Comparative analysis with Bother ALL patients was conducted by the Mantel-Haenszel method; it found that Ph-like ALL patients had a significantly lower chance of being alive at 5 years (pooled odds ratio, 0.35; 95% confidence interval, 0.25-0.50; P < .00001, I 2 ¼ 40%). The chance of Ph-like ALL patients surviving at 5 years was similar to Ph-positive ALL patients (pooled odds ratio, 0.72; 95% confidence interval, 0.26-2.02; P ¼ .53, I 2 ¼ 77%). Conclusion: Ph-like ALL is not uncommon among ALL patients, and its presence is associated with an unfavorable outcome. More investigations are needed for better therapeutic options.