A blastic plasmacytoid dendritic cell neoplasm-like immunophenotype is negatively associated with CEBPA bZIP mutation and predicts unfavorable prognosis in acute myeloid leukemia.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive myeloid malignancy which characteristically expresses an atypical phenotype including CD123+, CD56+, and CD4+. We are aimed to investigate the clinical and prognostic characteristics of AML patients exhibiting BPDCN-like immunophenotype and provide additional insights for risk stratification of AML. A total of 241 newly diagnosed AML patients were enrolled in this retrospective study and categorized into BPDCN-like positive (n = 125)/negative (n = 116) groups, determined by the present with CD123+ along with either CD56+ or CD4+, or both.

Effective Tacrolimus Treatment for Patients with Non-Severe Aplastic Anemia That is Refractory/Intolerant to Cyclosporine A: A Retrospective Study.

Background: For symptomatic non-severe aplastic anemia (NSAA) patients who cannot afford anti-thymocyte globulin (ATG) or allogeneic hematopoietic stem cell transplantation (HSCT), tacrolimus (FK) may be an option if these patients do not respond or become tolerant to cyclosporine A (CsA).

Methods: We enrolled 101 NSAA patients who were refractory or intolerant to CsA with no chance of HSCT or ATG treatment and treated these patients with tacrolimus for at least 6 months, with follow-up for at least one year.

Results: The overall response rate (ORR) was 38.

Successful sirolimus treatment of patients with pure red cell aplasia complicated with renal insufficiency.

For patients with pure red cell aplasia (PRCA), cyclosporine (CsA) is the first line therapy. Occasionally, some patients who suffer from renal insufficiency cannot tolerate CsA. To explore the efficacy and tolerance of sirolimus treatment for those patients, twelve PRCA patients with renal insufficiency from May 2014 to May 2018 in Peking Union Medical College Hospital were enrolled, treated with sirolimus, and followed up at the median time of 16 (10-50) months.

Comparison of frontline treatment with intensive immunosuppression therapy and HLA-haploidentical hematopoietic stem cell transplantation for young patients with severe aplastic anemia - A meta analysis.

Aim: To compare the survivals and treatment related complications between immunosuppression therapy (IST) and haploidentical hematopoietic stem cell transplantation (haplo-HSCT) on children and young adults with severe aplastic anemia (SAA) in East Asia during the last 10 years.

Methods: After looking through Pubmed, Embase, Web of Science and Wanfang Data, a total of 491 patients from 7 retrospective studies conducted in East-Asia were included for meta-analysis based on Stata program. Publication bias was measured by Begger and Egger tests.

Correction to: prediction of thrombosis risk in patients with paroxysmal nocturnal hemoglobinuria.

The authors determined an error in the affiliation section; it was captured as Department of Hematology, Peking Union Hospital, CAMS & PUMC, Beijing 100,730, China. The correct affiliation should be Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China.

Prediction of thrombosis risk in patients with paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic disease with thrombosis as a major complication. The mechanism of thrombosis and related risk factors in PNH patients are still not well characterized. We retrospectively enrolled 99 patients with newly diagnosed PNH at our institute from 2011 to 2016.

Clinical Efficacy and Safety of High-Dose Dexamethasone Plus Low-Dose Rituximab as First-Line Therapy in Newly Diagnosed Primary Immune Thrombocytopenia.

Standard-dose prednisone as first-line therapy for primary immune thrombocytopenia (ITP) can not obtain high long-term responses. Results from high-dose dexamethasone course administered in adult newly diagnosed ITP were promising. The role of standard-dose rituximab in first-line treatment of newly diagnosed ITP were also investigated.

Multi-angles of smoking and mild cognitive impairment: is the association mediated by sleep duration?

Although the association between cigarette smoking and risk of mild cognitive impairment (MCI) is controversial, most recent studies have shown that this influence is negative. However, it is unknown how multiple factors of smoking affect MCI, and the mechanisms of different smoking factors are not yet clarified. This study will examine the impact of various angles of smoking on MCI and the potential mediating effects of sleep duration on smoking MCI association in the elderly.

[Clinical outcome of allogeneic hematopoietic stem cell transplantation in the treatment of 9 myeloid leukemia patients with granulocytic sarcoma].

To explore the efficacy and influencing factors of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with myeloid leukemia and granulocytic sarcoma (GS). Clinical outcome including hematopoietic reconstitution, transplant-related complications, survival and relapse were collected and retrospectively analyzed in 9 patients with myeloid leukemia and GS after allo-HSCT. Hematopoiesis reconstitution was achieved in all the 9 recipients.

The polymorphisms of tumor necrosis factor-induced protein 3 gene may contribute to the susceptibility of chronic primary immune thrombocytopenia in Chinese population.

Primary immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by decrease of the platelet count and increased risk of mucocutaneous bleeding. Multiple factors have been demonstrated in ITP pathogenesis, including the genetic variants. Tumor necrosis factor-induced protein 3 (TNFAIP3) gene encodes the ubiquitin-modifying enzyme A20 which limits inflammation by terminating NF-κB activation through several signaling pathways including TNF and Toll-like receptors and regulates immunostimulatory effects of dendritic cells and attenuates antigen presentation.

Reduced intensity conditioning and co-transplantation of unrelated peripheral stem cells combined with umbilical cord mesenchymal stem/stroma cells for young patients with refractory severe aplastic anemia.

Five young patients with a long history of severe aplastic anemia (SAA) who had failed initial CSA treatment and lacked a HLA-matched sibling donor, underwent co-transplantation of unrelated donor peripheral blood stem cells (UD-PBSCs) and umbilical cord mesenchymal/stroma stem cells (UC-MSCs). After FLU + CTX + ATG ± 2GY TBI conditioning, all patients received UD-PBSCs and UC-MSCs. There were no side effects attributable to the infused MSCs, and no severe complications or infections were observed in any patient after transplantation.