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Br J Haematol 2021 May 7. Epub 2021 May 7.

Department of Haematology, Post graduate Institute of Medical Education and Research Chandigarh, Chandigarh, India.

Eur J Haematol 2021 May 4. Epub 2021 May 4.

Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Objectives: Patients with acquired aplastic anemia (AA) without HLA-matched sibling donors or aged >40 years receive immunosuppressive therapy (IST) with anti-thymocyte globulin (ATG). We investigated the relationship between plasma rabbit ATG (r-ATG) concentration and IST response.

Methods: From May 2012 to October 2017, 81 patients with severe AA who required initial IST were included. Read More

Life Sci 2021 May 1:119556. Epub 2021 May 1.

Department of Biochemistry & Biophysics, University of Kalyani, Kalyani 741235, India. Electronic address:

Objective: Poly(ADP-ribose) polymerase-1 (PARP1) interacts and poly(ADP-ribosyl)ates telomere repeat binding factor 2 (TRF2), which acts as a platform to recruit a large number of proteins at the telomere. Since the discovery of TRF2-SLX4 interaction, SLX4 is becoming the key player in telomere length (TL) maintenance and repair by telomere sister chromatid exchange (T-SCE). Defective TL maintenance pathway results in a spectrum of diseases called telomeropathies like dyskeratosis congenita, aplastic anemia, fanconi anemia, cancer. Read More

Blood 2021 May 4. Epub 2021 May 4.

University of Perugia, Perugia, Italy.

The BCL6 co-repressor (BCOR) is a transcription factor involved in the control of embryogenesis, mesenchymal stem cells function, hematopoiesis and lymphoid development. Recurrent somatic clonal mutations of the BCOR gene and its homologue BCORL1 have been detected in several hematological malignancies and aplastic anemia. They are scattered across the whole gene length and mostly represent frameshifts (deletions, insertions), nonsense and missence mutations. Read More

Expert Rev Hematol 2021 May 4. Epub 2021 May 4.

Peking University People's Hospital, Peking University Institute of Hematology, National Clinical Research Center for Hematologic Disease, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing, China.

Introduction: : Allogeneic hematopoietic stem cell transplantation (allo-HSCT) has resulted in increased levels of disease-free survival in severe aplastic anemia (SAA). Haploidentical transplantation (haplo-SCT) was previously not recommended due to unacceptable incidences of graft-versus-host disease (GvHD) and graft failures. With the advent of intensive GvHD prophylaxis strategies, the outcomes obtained with haplo-SCT for SAA have gradually improved. Read More

Blood Res 2021 Apr;56(S1):S51-S64

Department of Laboratory Medicine, Seoul National University Hospital, Seoul, Korea.

Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematological neoplasms characterized by ineffective hematopoiesis, morphologic dysplasia, and cytopenia. MDS overlap syndromes include various disorders, such as myelodysplastic/myeloproliferative neoplasms and hypoplastic MDS with aplastic anemia characteristics. MDS overlap syndromes share the characteristics of other diseases, which make differential diagnoses challenging. Read More

Sci Rep 2021 Apr 30;11(1):9401. Epub 2021 Apr 30.

National Medical Research Center of Pediatric Hematology, Oncology and Immunology Named After Dmitry Rogachev, Russian Ministry of Healthcare, 1 Samory Mashela Str, Moscow, Russia, 117997.

Immune thrombocytopenia (ITP) is believed to be associated with platelet function defects. However, their mechanisms are poorly understood, in particular with regard to differences between ITP phases, patient age, and therapy. We investigated platelet function and bleeding in children with either persistent or chronic ITP, with or without romiplostim therapy. Read More

Br J Haematol 2021 Apr 29. Epub 2021 Apr 29.

Hematology Branch, National Heart, Lung and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, MD, USA.

Haematologica 2021 04 29. Epub 2021 Apr 29.

Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation, Leiden, Neitherland; Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern.

Not available. Read More

Haematologica 2021 Apr 29. Epub 2021 Apr 29.

Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health.

Pretreatment blood counts, particularly the absolute reticulocyte count (ARC) ≥25×109/L, correlate with response to immunosuppressive therapy (IST) in severe aplastic anemia (SAA). In recent trials, eltrombopag (EPAG) combined with standard IST yielded superior responses than did IST alone. Our single institution retrospective study aimed to elucidate whether historical response predictors associated with IST plus EPAG. Read More

J Pediatr Adolesc Gynecol 2021 Apr 25. Epub 2021 Apr 25.

Division of Pediatric and Adolescent Gynecology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, 200 1(st) St SW, Rochester MN 55905 USA. Electronic address:

Study Objectives: To describe the structure of a pediatric fertility preservation (FP) program and share safety and patient satisfaction data.

Design: The FP program operates under prospective research protocols approved by the Mayo Clinic Institutional Review Board (IRB).

Setting: The FP program is a multidisciplinary effort between pediatric gynecology, reproductive endocrinology, pediatric urology, pediatric surgery, and laboratory medicine. Read More

Br J Haematol 2021 Apr 26. Epub 2021 Apr 26.

Department of Pathology, University of Colombo Faculty of Medicine, Colombo, Sri Lanka.

Blood Adv 2021 Apr;5(8):2106-2114

Division of Pediatric Allergy, Immunology, and Bone Marrow Transplant.

Transplant-associated thrombotic microangiopathy (TA-TMA) is an endothelial injury syndrome that complicates hematopoietic stem cell transplant (HSCT). Morbidity and mortality from TA-TMA remain high, making prevention critical. We describe our retrospective single-center experience of TA-TMA after pediatric allogeneic HSCT and present a novel pre-HSCT risk-stratification system and prophylaxis regimen. Read More

Indian J Hematol Blood Transfus 2021 Apr 10;37(2):220-225. Epub 2020 Jun 10.

Department of Hematology, All India Institute of Medical Sciences, New Delhi, 110049 India.

PNH is a rare disease with wide spectrum of intra-vascular hemolysis and thrombosis to sub-clinical PNH clones. We aimed to study the clinico-hematological profile and clone size on granulocytes and monocytes of PNH patients classified as per International PNH Interest Group recommendations. A retrospective analysis of clinico-hematological profile of 112 PNH clone positive patients by FLAER based flow cytometry between January and September 2017 done and classified into classical PNH, PNH with aplastic anemia or myelodysplastic syndrome (PNH-AA/MDS) and sub-clinical PNH clones (PNH-sc). Read More

Indian J Hematol Blood Transfus 2021 Apr 26;37(2):210-219. Epub 2020 Apr 26.

Department of Pediatrics, King George's Medical University, Chowk, Lucknow, 226003 India.

Aplastic anemia (AA) is an immune-mediated disorder in which hematopoietic stem and progenitor cells are targeted by a number of cellular and molecular pathways. This case control study aims to investigate the association of interleukin-1beta () gene polymorphisms, ( and ) and their plasma levels with acquired AA. Genotyping was done by Restricted Fragment Length Polymorphism (PCR-RFLP) method and plasma levels were evaluated in peripheral blood using ELISA. Read More

Indian J Hematol Blood Transfus 2021 Apr 10;37(2):197-203. Epub 2019 Jul 10.

Department of Hematology, NRS Medical College, Kolkata, 700014 India.

The current study was conducted to assess response to immunosuppressive therapy (IST) in acquired aplastic anaemia (AA). It was a retrospective and prospective observational study. Patients were diagnosed as per standard international guidelines and IST was started as per standard protocol. Read More

Transplant Cell Ther 2021 Apr 14. Epub 2021 Apr 14.

Division of Pediatric Oncology, Dana Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA, USA; Department of Pediatrics, Harvard University Medical School, Boston, MA.

Background: Long-term survival following hematopoietic stem cell transplant (HSCT) in childhood continues to improve and patients are thus increasingly faced with the late effects of treatment. Infertility is very common for both males and females following HSCT and is one of the most distressing sequelae. Adoption and surrogate egg or sperm donation are possibilities for some patients but post-HSCT reversal of gonadal failure is not possible. Read More

Pediatr Blood Cancer 2021 Apr 15:e29066. Epub 2021 Apr 15.

Department of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.

Background: Immunosuppressive therapy with horse antithymocyte globulin and cyclosporine currently remains the standard therapy for children with severe aplastic anemia (SAA) who lack human leukocyte antigen (HLA)-identical sibling. The thrombopoietin receptor agonist eltrombopag has been recently approved for SAA patients 2 years and older. However, there are limited data on its safety and efficacy in pediatric cohorts. Read More

Br J Haematol 2021 Apr 14. Epub 2021 Apr 14.

Division of Haematology, Hospital A Beneficência Portuguesa, São Paulo, Brazil.

The progress in aplastic anaemia (AA) management is one of success. Once an obscure entity resulting in death in most affected can now be successfully treated with either haematopoietic stem cell transplantation (HSCT) or immunosuppressive therapy (IST). The mechanisms that underly the diminution of haematopoietic stem cells (HSCs) are now better elucidated, and include genetics and immunological alterations. Read More

Transplant Cell Ther 2021 Apr 11;27(4):301-307. Epub 2020 Dec 11.

Department of Hematology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences. Electronic address:

In the era of eculizumab, the number of patients with paroxysmal nocturnal hemoglobinuria (PNH) who undergo hematopoietic stem cell transplantation (HSCT) has decreased significantly. However, owing to the possibility of severe aplastic anemia (AA) or a suboptimal response to eculizumab, HSCT still plays an important role in the treatment of patients with PNH combined with AA or recurrent hemolysis-related symptoms despite its high level of risk. Here we review studies involving patients with PNH who underwent HSCT over the past 15 years and conclude that patients with refractory AA/PNH and patients with severe classical PNH are candidates for HSCT in countries where eculizumab is unavailable. Read More

Cancer Immunol Immunother 2021 Apr 10. Epub 2021 Apr 10.

Immunology and Oncology, Centro Nacional de Biotecnología-CSIC, Madrid, Spain.

Background: Antibody-based therapies blocking the programmed cell death-1/ligand-1 (PD-1/PD-L1) axis have provided unprecedent clinical success in cancer treatment. Acquired resistance, however, frequently occurs, commonly associated with the upregulation of additional inhibitory molecules. Diacylglycerol kinase (DGK) α limits the extent of Ras activation in response to antigen recognition, and its upregulation facilitates hypofunctional, exhausted T cell states. Read More

Chin J Integr Med 2021 Apr 10. Epub 2021 Apr 10.

Institute of Hematology Research, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, 310006, China.

Objective: To evaluate the efficacy and safety of Pai-Neng-Da Capsule (, panaxadiol saponins component, PNDC) in combination with the cyclosporine and androgen for patients with chronic aplastic anemia (CAA).

Methods: A total of 79 CAA patients was randomly divided into 2 groups by a random number table, including PCA group [43 cases, orally PNDC 320 mg/d plus cyclosporine 5 mg/(kg·d) plus andriol 80 mg/d] and CA group [36 cases, orally cyclosporine 5 mg/(kg·d) plus andriol 160 mg/d]. All patients were treated and followed-up for 6 treatment courses over 24 weeks. Read More

Transplant Cell Ther 2021 Apr 24;27(4):331.e1-331.e7. Epub 2021 Jan 24.

Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China; Institute of Organ Transplantation, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China. Electronic address:

Aplastic anemia (AA) is a life-threatening hematological disorder that can be cured by hematopoietic stem cell transplantation. Haploidentical transplantation becomes an alternative choice for patients in the absence of a matched sibling donor. We used a retrospective study aimed to confirm the feasibility of busulfan-based modified post-transplantation cyclophosphamide (PTCY) strategy in haploidentical hematopoietic stem cell transplantation for AA patients. Read More

Transplant Cell Ther 2021 Apr 16;27(4):313.e1-313.e8. Epub 2021 Jan 16.

Clinical Genetics Branch, Division of Cancer Epidemiology & Genetics, National Cancer Institute, National Institutes of Health, Bethesda, Maryland. Electronic address:

Cellular aging in hematopoietic cell transplantation (HCT) is important in the context of immune reconstitution and age-related complications. Recently, several DNA-methylation (DNAm)-based biomarkers of aging known as "epigenetic clocks" have been introduced as novel tools to predict cellular age. Here, we used Cox proportional hazards models to assess the possible associations of donor pre-HCT DNAm age, and its post-HCT changes, using the recently published lifespan-associated epigenetic clock known as "DNAm-GrimAge," with outcomes among patients with severe aplastic anemia (SAA). Read More

Transplant Cell Ther 2021 Apr;27(4):286-291

Blood and Marrow Transplant Program, University of Minnesota, Minneapolis, Minnesota.

For cord blood transplantation (CBT), appropriate patient and conditioning regimen selection is necessary to achieve long-term disease-free survival. This review aims to provide comprehensive guidelines on these issues using evidence from the literature and experience at dedicated CBT centers. Topics include patient and disease characteristics that make CBT a good or poor choice and a review of outcomes in commonly used conditioning regimens in CBT. Read More

Transplant Cell Ther 2021 Feb 14;27(2):190-191. Epub 2020 Dec 14.

Department of Haematology, St Vincent's Hospital, Darlinghurst, New South Wales, Australia; Faculty of Medicine, University of New South Wales, Sydney, Australia.

Leukemia 2021 Apr 6. Epub 2021 Apr 6.

Department of Hematology, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan.

To characterize glycosylphosphatidylinositol-anchored protein-deficient (GPI[-]) and HLA-class I allele-lacking (HLA[-]) hematopoietic stem progenitor cells (HSPCs) in acquired aplastic anemia (AA), we studied the peripheral blood (PB) of 56 AA patients in remission who possessed both (n = 13, Group A) or either GPI(-) (n = 34, Group B) and HLA(-) (n = 9, Group C) cell populations. Seventy-seven percent (10/13) of Group A had HLA(-) cells in all lineages of PB cells, including platelets, while only 23% (3/13) had GPI(-) cells in all lineages, and the median percentage of HLA(-) granulocytes in the total granulocytes (21.2%) was significantly higher than that of GPI(-) granulocytes (0. Read More

Nefrologia 2021 Mar 31. Epub 2021 Mar 31.

Unidad de Trasplante Renal, Instituto Mexicano del Seguro Social Centro Médico Nacional La Raza, Hospital de Especialidades «Dr. Antonio Fraga Mouret», Centro Médico Nacional La Raza, Ciudad de México, México.

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2021 Apr;29(2):557-566

State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020,

AbstractObjective: To establish an acquired aplastic anemia animal model for investigating the function of T lymphocyte and the pathogenesis and treatment of aplastic anemia(AA).

Methods: To establish the acquired aplastic anemia mouse model through the X-ray irradiation in combination with lymphocytes injection. AA Group: the purified Pan T lymphocytes from the spleen of C57BL/6J mice were enriched and injected to the mice through tail vein(5×10), the CB6F1 mice were exposed to 3,4 and 5 Gy X-ray irradiation; TBI Group: the CB6F1 mice were exposed to 3,4 and 5 Gy X-ray irradiation, and were injected with the same volume of PBS buffer; Control group: the CB6F1 mice were only injected with the same volume of PBS buffer. Read More

Cancers (Basel) 2021 Mar 26;13(7). Epub 2021 Mar 26.

Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Autoimmune cytopenias (AICy) and autoimmune diseases (AID) can complicate both lymphoid and myeloid neoplasms, and often represent a diagnostic and therapeutic challenge. While autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are well known, other rarer AICy (autoimmune neutropenia, aplastic anemia, and pure red cell aplasia) and AID (systemic lupus erythematosus, rheumatoid arthritis, vasculitis, thyroiditis, and others) are poorly recognized. This review analyses the available literature of the last 30 years regarding the occurrence of AICy/AID in different onco-hematologic conditions. Read More