Impact of blood pressure early after allogeneic hematopoietic cell transplantation on clinical outcomes
In this study, we analyzed the relationship between blood pressure control early after allo-HCT and survival outcomes. All patients who survived longer than 28  days after allo-HCT at our center between June 2007 and June 2018 (n = 353) were included, and the average systolic blood pressure (asBP) from 1 to 28 days after allo-HCT was calculated. According to the results of a ROC curve analysis, an asBP of 131 mmHg was defined as a cut-off value between high and low asBP groups. Non-relapse mortality (NRM) and OS were significantly inferior in the high asBP group (2-year-NRM 28.0% vs 11.1%,P&thi...
Source: Annals of Hematology - May 19, 2020 Category: Hematology Source Type: research

Management of myelofibrosis after ruxolitinib failure
AbstractMyelofibrosis is a BCR-ABL1 –negative myeloproliferative neoplasm characterized by anemia, progressive splenomegaly, extramedullary hematopoiesis, bone marrow fibrosis, constitutional symptoms, leukemic progression, and shortened survival. Constitutive activation of the Janus kinase/signal transducers and activators of trans cription (JAK-STAT) pathway, and other cellular pathways downstream, leads to myeloproliferation, proinflammatory cytokine expression, and bone marrow remodeling. Transplant is the only curative option for myelofibrosis, but high rates of morbidity and mortality limit eligibility. Several...
Source: Annals of Hematology - May 19, 2020 Category: Hematology Source Type: research

Outcome of stem cell transplantation for Waldenstr öm’s macroglobulinemia: analysis of the Japan Society for Hematopoietic Cell Transplantation (JSHCT) Lymphoma Working Group
AbstractThe role of stem cell transplantation (SCT) for patients with Waldenstr öm’s macroglobulinemia (WM) remains undetermined. Therefore, we retrospectively evaluated the outcome of autologous and allogeneic SCT for patients with WM using the registry database of the Japan Society for Hematopoietic Cell Transplantation. Forty-six patients receiving autologous and 31 recei ving allogeneic SCT were analyzed. The allogeneic SCT group included more patients with advanced disease status at transplant and received more lines of chemotherapy. The cumulative incidences of non-relapse mortality (NRM) at 1 year were 30...
Source: Annals of Hematology - May 18, 2020 Category: Hematology Source Type: research

Modern management of splenomegaly in patients with myelofibrosis
AbstractMyelofibrosis (MF) is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly secondary to extramedullary hematopoiesis. Patients frequently exhibit debilitating symptoms including pain and early satiety, in addition to cellular sequestration causing severe cytopenias. JAK 1/2 inhibitors, such as ruxolitinib and fedratinib, are the mainstay of therapy and produce significant and durable reductions in spleen volume. However, many patients are not eligible for JAK 2 inhibitor therapy or become refractory to treatment over time. Novel therapies are in development that can reduce the degree of sple...
Source: Annals of Hematology - May 17, 2020 Category: Hematology Source Type: research

Nationwide prevalence and trends in acute cardiovascular events and in-hospital mortality among adult African Americans with sickle cell trait
(Source: Annals of Hematology)
Source: Annals of Hematology - May 17, 2020 Category: Hematology Source Type: research

Progression of disease within 2  years (POD24) is a clinically relevant endpoint to identify high-risk follicular lymphoma patients in real life
AbstractFollicular lymphoma (FL) is an indolent non-Hodgkin ’s lymphoma with heterogeneous outcomes. Progression or relapse of FL within 2 years (so-called POD24) after diagnosis is associated with a poor outcome for patients treated with R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, prednisone) in clinical trials. POD24 needs further validation before it can be used as a relevant endpoint to assess treatment efficacy. In the present retrospective monocentric study, we investigated the predictive value of POD24 in a cohort of grade 1, 2, or 3a FL patients treated in our institution (Nantes M...
Source: Annals of Hematology - May 16, 2020 Category: Hematology Source Type: research

Primary pulmonary diffuse large B cell lymphoma presenting as bulky bilateral lung tumors
(Source: Annals of Hematology)
Source: Annals of Hematology - May 16, 2020 Category: Hematology Source Type: research

Daratumumab for refractory warm autoimmune hemolytic anemia
(Source: Annals of Hematology)
Source: Annals of Hematology - May 14, 2020 Category: Hematology Source Type: research

Could ruxolitinib be effective in patients with COVID-19 infection at risk of acute respiratory distress syndrome (ARDS)?
(Source: Annals of Hematology)
Source: Annals of Hematology - May 14, 2020 Category: Hematology Source Type: research

Combined gemcitabine, cisplatin, dexamethasone, methotrexate, and pegaspargase (GDP-ML) for patients with newly diagnosed extranodal natural killer/T cell lymphoma, nasal type: a single arm, single center, prospective phase 2 study
AbstractExtranodal natural killer/T cell lymphoma, nasal type (ENKL) is a highly aggressive tumor with relatively poor prognosis. In this prospective study, we investigated the efficacy and toxicity of a novel GDP-ML regimen (combined gemcitabine, cisplatin, dexamethasone, methotrexate, and pegaspargase) as front-line treatment in newly diagnosed ENKL. Eligible newly diagnosed stage I/II ENKL patients received sandwich chemoradiation therapy. Patients with stage III/IV disease received an initial 4 cycles of GDP-ML regimen. After 4 cycles, responding patients continued to receive either autologous transplantation or additi...
Source: Annals of Hematology - May 13, 2020 Category: Hematology Source Type: research

Necrotizing fasciitis in haematological patients: a different scenario
AbstractTo describe and compare the characteristics of necrotizing fasciitis (NF) in patients with and without haematological malignancy. All adult patients diagnosed with NF and treated at our hospital were included (January 2010 –March 2019). Diagnosis was based on intraoperative findings or consistent clinical/radiological characteristics, and patients were classified as group A (with haematological malignancy) or group B (without haematological malignancy). Student’st (quantitative), Fisher ’s exact (qualitative), and Kaplan-Meyer tests were used for the statistical analysis. The study included 29 pat...
Source: Annals of Hematology - May 12, 2020 Category: Hematology Source Type: research

Upfront autologous hematopoietic stem cell transplantation for high-risk patients with double-expressor diffuse large B cell lymphoma
In conclusion, MYC and BCL2 co-expression did not have a poor prognostic impact among high-risk patients with DLBCL treated with upfront ASCT regardless of molecular class ification. This preliminary study suggested that the role of consolidative ASCT is needed to be evaluated in a prospective randomized clinical trial. (Source: Annals of Hematology)
Source: Annals of Hematology - May 10, 2020 Category: Hematology Source Type: research

Baseline clinical characteristics and disease burden in patients with paroxysmal nocturnal hemoglobinuria (PNH): updated analysis from the International PNH Registry
AbstractThe International Paroxysmal Nocturnal Hemoglobinuria (PNH) Registry (NCT01374360) was initiated to optimize patient management by collecting data regarding disease burden, progression, and clinical outcomes. Herein, we report updated baseline demographics, clinical characteristics, disease burden data, and observed trends regarding clone size in the largest cohort of Registry patients. Patients with available data as of July 2017 were stratified by glycosylphosphatidylinositol (GPI)-deficient granulocyte clone size (
Source: Annals of Hematology - May 10, 2020 Category: Hematology Source Type: research

A novel Twist1/hsa-miR138-5p/caspase-3 pathway regulates cell proliferation and apoptosis of human multiple myeloma
AbstractThe nuclear transcription factor twist-related protein 1 (Twist1) is associated with tumor malignant transformation and metastasis in various types of carcinomas. We found that Twist1 was highly expressed in clinical multiple myeloma (MM) cells, and explored its roles in proliferation and apoptosis in human MM cell lines U266 and RPMI-8226. In these cells, Twist1 transcriptionally regulated the miRNA hsa-miR138-5p, which targeted caspase-3 to control apoptosis. Silencing of Twist1 significantly suppressed cell proliferation and increased apoptosis, which was reversed by overexpression of hsa-miR138-5p or simultaneo...
Source: Annals of Hematology - May 9, 2020 Category: Hematology Source Type: research

Chimeric antigen receptor therapy in hematological malignancies: antigenic targets and their clinical research progress
ABSTRACTChimeric antigen receptor (CAR)-based immunotherapy has achieved dramatic success in the treatment of B cell malignancies, based on the summary of current research data, and has shown good potential in early phase cancer clinical trials. Modified constructs are being optimized to recognize and destroy tumor cells more effectively. By targeting the proper B-lineage-specific antigens such as CD19 and CD20, adoptive immunotherapy has demonstrated promising clinical results and already plays a role in the treatment of several lymphoid malignancies, which highlights the importance of target selection for other CAR thera...
Source: Annals of Hematology - May 9, 2020 Category: Hematology Source Type: research

A patient with cerebral venous sinus thrombosis induced by facial hyaluronic acid injection
(Source: Annals of Hematology)
Source: Annals of Hematology - May 9, 2020 Category: Hematology Source Type: research

Pegfilgrastim improves the outcomes of mobilization and engraftment in autologous hematopoietic stem cell transplantation for the treatment of multiple myeloma
AbstractAutologous stem cell transplantation (ASCT) is the only curable therapy for multiple myeloma (MM), while its success primarily relies on mobilization to obtain sufficient hematopoietic stem/progenitor cells (HPC). Although the role of Pegfilgrastim (PEG), a novel PEGylated form of the recombinant G-CSF filgrastim (FIL), in mobilization has been demonstrated, it remains unclear whether this approach is cost-effective in MM treatment. Here, we performed a real-world analysis to evaluate the efficacy and cost of PEG for mobilization in a cohort of MM patients, of which 53% carried high-risk cytogenetic abnormalities. ...
Source: Annals of Hematology - May 8, 2020 Category: Hematology Source Type: research

Signals of T h 2 immune response from COVID-19 patients requiring intensive care
(Source: Annals of Hematology)
Source: Annals of Hematology - May 8, 2020 Category: Hematology Source Type: research

Post-transplant cyclophosphamide combined with anti-thymocyte globulin for graft-vs-host disease prophylaxis improves survival and lowers non-relapse mortality in older patients undergoing allogeneic hematopoietic cell transplantation
AbstractGraft-versus-host disease (GVHD) represents a major contributor to morbidity and mortality in recipients of allogeneic hematopoietic cell transplants (HCT). Several strategies exist for GVHD prophylaxis and include post-transplant cyclophosphamide (PTCY) and anti-thymocyte globulin (ATG). While several groups have described the use of PTCY in younger patients, there is a paucity of data about the efficacy of PTCY in older individuals, particularly when combined with ATG. We investigated the effect of PTCY and ATG combination on transplant outcomes in older patients at Princess Margaret Cancer Centre, Toronto, Canad...
Source: Annals of Hematology - May 7, 2020 Category: Hematology Source Type: research

Long PFS of more than 7  years is achieved in 9% of myeloma patients in the era of conventional chemotherapy and of first-generation novel anti-myeloma agents: a single-center experience over 20-year period
AbstractAdvances in the management of multiple myeloma (MM) have led to a significant prolongation of both progression-free (PFS) and overall survival (OS). Herein, we evaluated the characteristics of patients who achieved a PFS of at least 7  years following frontline therapy, as compared with all other patients who were treated in a single center during the same time period. Thirty-six (8.8%) patients achieved a PFS of at least 7 years (long PFS group) after frontline treatment. Long PFS patients were younger (p 
Source: Annals of Hematology - May 7, 2020 Category: Hematology Source Type: research

Immunodeficiency risk score for prediction of mortality by parainfluenza virus infection in patients with hematologic malignancy
AbstractParainfluenza virus (PIV) infection is a significant cause of morbidity and mortality, especially in hematologic malignancy patients including hematopoietic stem cell transplantation (HCT) recipients. However, limited information is available for risk stratification in PIV-infected patients with hematologic malignancy with or without HCT. Patients with hematologic malignancy diagnosed with PIV from January 2009 to December 2018 were retrospectively included in a tertiary care hospital in Seoul, South Korea. Upper respiratory tract infection (URTI) was defined as the detection of PIV in a nasopharyngeal sample with ...
Source: Annals of Hematology - May 7, 2020 Category: Hematology Source Type: research

Risk of kidney toxicity with carfilzomib in multiple myeloma: a meta-analysis of randomized controlled trials
AbstractThe incidence and relative risk of kidney toxicity with carfilzomib in multiple myeloma (MM) has been incompletely characterized. We performed a systematic review and meta-analysis of randomized controlled trials (RCTs) comparing carfilzomib-based with non-carfilzomib-based regimens in MM to investigate the risk of kidney toxicity. Point estimates were pooled in the form of risk ratios (RR) with 95% confidence intervals (CI) using the random-effects model. We identified four RCTs with 2954 patients. The median duration of treatment ranged from 16.3 to 88  weeks in carfilzomib arms. The cumulative rate of kidne...
Source: Annals of Hematology - May 7, 2020 Category: Hematology Source Type: research

Real-world risk assessment and treatment initiation among patients with myelofibrosis at community oncology practices in the United States
This study was conducted to evaluate how physicians risk-stratify patients at the time of MF diagnosis, the accuracy of the risk stratification, and its effect on treatment selection. Medical charts were reviewed at US community hematology/oncology practices in the Cardinal Health Oncology Provider Extended Network; patient clinical characteristics, risk stratification, and treatment data were collected. Physician-assigned risk categorizations were compared with data-derived risk categorizations based on the International Prognostic Scoring System, the system recommended at diagnosis. A total of 491 patients diagnosed with...
Source: Annals of Hematology - May 7, 2020 Category: Hematology Source Type: research

A case of idiopathic multicentric Castleman disease presenting with diffuse lung cysts: how to evaluate treatment response?
(Source: Annals of Hematology)
Source: Annals of Hematology - May 7, 2020 Category: Hematology Source Type: research

The concomitance of lymphoma and breast carcinoma in the bone
(Source: Annals of Hematology)
Source: Annals of Hematology - May 7, 2020 Category: Hematology Source Type: research

Rosette-like formation in multiple myeloma
(Source: Annals of Hematology)
Source: Annals of Hematology - May 7, 2020 Category: Hematology Source Type: research

A study of the geographic distribution and associated risk factors of leg ulcers within an international cohort of sickle cell disease patients: the CASiRe group analysis
AbstractVasculopathy is a hallmark of sickle cell disease ultimately resulting in chronic end organ damage. Leg ulcer is one of its sequelae, occurring in ~  5–10% of adult sickle cell patients. The majority of leg ulcer publications to date have emanated from single center cohort studies. As such, there are limited studies on the geographic distribution of leg ulcers and associated risk factors worldwide. The Consortium for the Advancement of Sickl e Cell Research (CASiRe) was formed to improve the understanding of the different phenotypes of sickle cell disease patients living in different geographic locatio...
Source: Annals of Hematology - May 6, 2020 Category: Hematology Source Type: research

Hypomethylating agents super-responders: challenging the dogma of long-term remission for acute myeloid leukemia
(Source: Annals of Hematology)
Source: Annals of Hematology - May 6, 2020 Category: Hematology Source Type: research

Strongyloides stercoralis hyperinfection syndrome in mantle cell lymphoma in post-transplant setting
(Source: Annals of Hematology)
Source: Annals of Hematology - May 6, 2020 Category: Hematology Source Type: research

A case of transplantation-associated thrombotic microangiopathy with autopsy-proven fatal myocardial ischemia after allogeneic hematopoietic stem cell transplantation
(Source: Annals of Hematology)
Source: Annals of Hematology - May 6, 2020 Category: Hematology Source Type: research

Long-term follow-up of a prospective phase 2 clinical trial of extended treatment with rituximab in patients with B cell post-transplant lymphoproliferative disease and validation in real world patients
In conclusion, PTLD patients in CR after rituximab have an excellent long-term outcome. These results not o nly apply in the clinical trial setting but are also reproducible in the real world. However, those patients who do not respond represent an unmet clinical need and should be included in prospective clinical trials. (Source: Annals of Hematology)
Source: Annals of Hematology - May 4, 2020 Category: Hematology Source Type: research

Outcomes related to intravenous fluid administration in sickle cell patients during vaso-occlusive crisis
AbstractWhile fluid replacement therapy is a primary treatment modality used in vaso-occlusive crises for sickle cell disease, data is limited on its safety, efficacy, and variability. We performed a retrospective analysis on 157 unique patient encounters from 49 sickle cell patients hospitalized with a vaso-occlusive episode at our institution from 2013 to 2017. The median length of hospital stay was 4  days (IQR 2–7). The mean total amount of intravenous fluid administered during the hospitalization was 7.4 L (Std 9.6). The mean total amount of fluid intake including intravenous fluids, blood transfusions...
Source: Annals of Hematology - May 4, 2020 Category: Hematology Source Type: research

Uterine myeloid sarcoma
(Source: Annals of Hematology)
Source: Annals of Hematology - May 4, 2020 Category: Hematology Source Type: research

Acquired amegakaryocytic thrombocytopenic purpura possibly induced by anti-PD-1 antibody
(Source: Annals of Hematology)
Source: Annals of Hematology - May 4, 2020 Category: Hematology Source Type: research

Red blood cell consumption in a large cohort of patients with thalassaemia: a retrospective analysis of main predictors
In this study, we explored the role and the contribution of several factors potentially involved in determining mean ATV in a cohort of TDT patients which have been followed since long time. We collected data on one-hundred and twenty-seven patients with transfusion-dependent β-thalassaemia followed at Rare Blood Cell Disease Unit, AORN Cardarelli Hospital. Age at first transfusion, genotype, spleen status (splenectomy or not), and mean soluble transferrin receptor (sTfR) were the parameters included in the analysis. At stepwise regression analysis which included all th e parameters, only splenectomy and mean sTfR sig...
Source: Annals of Hematology - May 4, 2020 Category: Hematology Source Type: research

Allogeneic hematopoietic cell transplantation for adults with acute myeloid leukemia conducted in Japan during the past quarter century
This study aimed to address the implementation status of allogeneic HCT for adults with AML in Japan and to provide a comprehensive overview of post-transplant outcomes. For this purpose, we analyzed data of 15,186 patients undergoing allogeneic HCT between 1992 and 2016 who were consecutively reported to the Japanese nationwide transplantation registry. The constant increase in the annual number of transplantations was clearly attributable to the growth of unrelated transplantation, and umbilical cord blood transplantation currently accounts for one-third of all allogeneic HCTs. The proportion of older patients has increa...
Source: Annals of Hematology - May 4, 2020 Category: Hematology Source Type: research

Sickle cell anemia in the state of Maranh ão: a haplotype study
AbstractSickle cell anemia (SCA) is the most severe form of sickle cell disease caused by homozygosity of the βS-gene (S/S or βSβS) and has worldwide distribution. Six polymorphic sites in the β-globin gene cluster were analyzed from a sample of 56 chromosomes of patients with SCA from the state of Maranhão, northeastern Brazil. PCR-RFLP showed that the CAR haplotype was predominant with a frequency of 64.28%, followed by the BEN haplotype (28.57%). Atypical haplotypes were identified a t a frequency of 7.15%. Genotypes CAR/CAR, BEN/BEN, and CAR/BEN were present in 46.43%, 10.71%, and 35.71% of p...
Source: Annals of Hematology - May 4, 2020 Category: Hematology Source Type: research

IgM-MGUS and associated membranoproliferative glomerulonephritis during IVIG administration
(Source: Annals of Hematology)
Source: Annals of Hematology - May 4, 2020 Category: Hematology Source Type: research

Targeted next-generation sequencing identified novel mutations associated with hereditary anemias in Brazil
AbstractHereditary anemias are a group of heterogeneous disorders including hemolytic anemias and hyporegenerative anemias, as congenital dyserythropoietic anemia (CDA). Causative mutations occur in a wide range of genes leading to deficiencies in red cell production, structure, or function. The genetic screening of the main genes is important for timely diagnosis, since routine laboratory tests fail in a percentage of the cases, appropriate treatment decisions, and genetic counseling purposes. A conventional gene-by-gene sequencing approach is expensive and highly time-consuming, due to the genetic complexity of these dis...
Source: Annals of Hematology - May 1, 2020 Category: Hematology Source Type: research

Influence of HLA 1 –3-locus mismatch and antithymocyte globulin administration in unrelated bone marrow transplantation
AbstractFor patients without an HLA-matched donor, an HLA-mismatched unrelated donor (MMUD) has been considered as an alternative donor in allogeneic hematopoietic cell transplantation (allo-HCT). We conducted a nationwide retrospective study to compare the transplant outcomes among 1-, 2-, and 3-locus (allele/antigen) mismatched unrelated donors (1MMUDn = 2044, 2MMUDn = 492, and 3MMUDn = 73) in allo-HCT and to assess the impact of antithymocyte globulin (ATG) in allo-HCT from 1-3MMUD. 2MMUD and 3MMUD were independent significant adverse factors for grade III–IV acute graft-v...
Source: Annals of Hematology - May 1, 2020 Category: Hematology Source Type: research

Residual disease by flow cytometry in patients with nucleophosmin-mutated acute myeloblastic leukemia
(Source: Annals of Hematology)
Source: Annals of Hematology - May 1, 2020 Category: Hematology Source Type: research

A 19-year-old patient with atypical chronic myeloid leukemia
(Source: Annals of Hematology)
Source: Annals of Hematology - May 1, 2020 Category: Hematology Source Type: research

Successful treatment of intracranial Rosai-Dorfman disease with cytarabine and dexamethasone: case report and review of literature
(Source: Annals of Hematology)
Source: Annals of Hematology - May 1, 2020 Category: Hematology Source Type: research

Current IST-free/relapse-free survival as a new endpoint after allogeneic hematopoietic stem cell transplantation
(Source: Annals of Hematology)
Source: Annals of Hematology - May 1, 2020 Category: Hematology Source Type: research

Successful use of venetoclax for treatment of eosinophilic dermatosis of myeloproliferative disease in a patient with chronic lymphocytic leukemia
(Source: Annals of Hematology)
Source: Annals of Hematology - May 1, 2020 Category: Hematology Source Type: research

Rectal involvement in pre-early T acute lymphoblastic leukemia
(Source: Annals of Hematology)
Source: Annals of Hematology - May 1, 2020 Category: Hematology Source Type: research

The management of gynecological complications in long-term survivors after allogeneic hematopoietic cell transplantation —a single-center real-life experience
AbstractIn everyday gynecological practice, there is an unmet need to manage survivors after allogeneic hematopoietic cell transplantation (allo-HCT). The major gynecological complications include premature ovarian insufficiency (POI), chronic graft-versus-host disease (cGVHD) of the anogenital zone (cGVHDgyn), and secondary neoplasms. Aiming to assess a real-life scale of problems associated with HCT, we performed a detailed analysis of a consecutive series of females after allo-HCT who were referred for a routine gynecological evaluation. The study includes 38 females after allo-HCT in whom gynecological examination with...
Source: Annals of Hematology - April 28, 2020 Category: Hematology Source Type: research

Absence of influence of peripheral blood CD34+ and CD3+ graft cell counts on outcomes after reduced-intensity conditioning transplantation using post-transplant cyclophosphamide
In conclusion, our data support the fact that there is no need to manipulate the graft before infusion in the particular context of PBSC/PTCY Baltim ore-based allotransplant. (Source: Annals of Hematology)
Source: Annals of Hematology - April 28, 2020 Category: Hematology Source Type: research

Long-term treatment response to PD-1 blockade therapy in a patient with DLBCL relapsed after anti-CD19 chimeric antigen receptor T cell treatment
(Source: Annals of Hematology)
Source: Annals of Hematology - April 25, 2020 Category: Hematology Source Type: research

Pre-transplant FLT3/ITD status predicts outcome in FLT3-mutated acute myeloid leukemia following allogeneic stem cell transplantation
AbstractAcute myeloid leukemia (AML) with fetal liver tyrosine kinase 3 (FLT3) internal tandem duplication (ITD) is associated with poor prognosis, and allogeneic stem cell transplantation (Allo-SCT) seems to be the preferred therapeutic approach. However, the predictors of post-transplant outcomes were not well-defined. The aim of the study was to evaluate the significance ofFLT3/ITD mutation by polymerase chain reaction as minimal residual disease (MRD) marker of outcomes after transplantation. We identified 43 patients (28  females and 15 males) with FLT3-mutated AML at the median age of 45 years who were allo...
Source: Annals of Hematology - April 24, 2020 Category: Hematology Source Type: research