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Eur J Haematol 2022 May 20. Epub 2022 May 20.

Department of Hematology and Stem Cell Transplantation, West German Cancer Center, University Hospital Essen, Germany.

COVID-19 is a potential life-threatening viral disease caused by SARS-CoV-2 and was declared a pandemic by the WHO in March 2020. mRNA-based SARS-CoV-2 vaccines are routinely recommended in immune-compromised patients, including patients with AA, as these patients are at increased risk of contracting COVID-19 and developing a more severe course of disease. Between March 2021 and November 2021 relapse of AA occurred in four (age (median): 53 years, range 30 - 84 years) out of 135 patients currently registered at our department and two de novo cases of AA in temporal context to vaccination against SARS-CoV-2, were documented. Read More

Med (N Y) 2022 Jan;3(1):42-57.e5

Department of Medicine, Massachusetts General Hospital, Boston, MA 02114, USA; Center for Regenerative Medicine, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA; Harvard Stem Cell Institute, Cambridge, MA, USA; Massachusetts General Hospital Cancer Center, Boston, MA, USA. Electronic address:

Background: Idiopathic aplastic anemia is a potentially lethal disease, characterized by T cell-mediated autoimmune attack of bone marrow hematopoietic stem cells. Standard of care therapies (stem cell transplantation or immunosuppression) are effective but associated with a risk of serious toxicities.

Methods: An 18-year-old man presented with aplastic anemia in the context of a germline gain-of-function variant in STAT1. Read More

Expert Rev Hematol 2022 May 18. Epub 2022 May 18.

AJA Cancer Epidemiology Research and Treatment Center (AJA- CERTC), AJA University of Medical Sciences, Tehran, Iran.

Introduction: COVID-19 crisis continues around the world. Some patients developed complications after the disease, which have been reported in limited studies. The aim of this study is to comprehensively assess the post-COVID hematologic complications in patients. Read More

J Cell Mol Med 2022 May 17. Epub 2022 May 17.

Department of Hematology, Tianjin Medical University General Hospital, Tianjin, China.

Cofilin-1 interacts with actin to regulate cell movement. The importance of cofilin-1 in immunity has been established, and its involvement in a number of autoimmune diseases has been confirmed. However, its role in severe aplastic anaemia (SAA) remains elusive. Read More

Front Immunol 2022 27;13:883826. Epub 2022 Apr 27.

Department of Pediatric Hematology and Stem Cell Transplantation, Willem-Alexander Children's Hospital, Leiden University Medical Center, Leiden, Netherlands.

Background: Severe multilineage cytopenia in childhood caused by bone marrow failure (BMF) often represents a serious condition requiring specific management. Patients are at risk for invasive infections and bleeding complications. Previous studies report low rates of identifiable causes of pediatric BMF, rendering most patients with a descriptive diagnosis such as aplastic anemia (AA). Read More

Dis Markers 2022 6;2022:3700691. Epub 2022 May 6.

Department of Hematology, Tianjin Medical University General Hospital, 154 Anshan Street, Heping District, Tianjin 300052, China.

Severe aplastic anemia (SAA) is an immune-mediated bone marrow failure characterized by pancytopenia. This study was aimed at uncovering proteins of plasma that were differentially expressed in SAA patients. 8 SAA patients and 8 health controls were enrolled and detected by data independent acquisition (DIA) technology. Read More

Clin Exp Immunol 2022 May;207(3):272-278

Department of Transplant Immunology and Immunogenetics, All India Institute of Medical Sciences, New Delhi, India.

Acquired aplastic anemia (aAA) is an autoimmune disease, characterized by infiltration of T lymphocytes in the bone marrow with destruction of hematopoietic stem cells by the effector cells. Interferon-gamma (IFN-γ) and perforin are important mediators of cell destruction. In this flow cytometry-based study, we have investigated the percentage of intracellular IFN-γ+ and perforin+ CD5+ T cells in peripheral blood of newly diagnosed aAA patients before and after immunosuppressive therapy (IST). Read More

Sci Rep 2022 May 11;12(1):7743. Epub 2022 May 11.

Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Francesco Sforza 35, 20100, Milan, Italy.

Data concerning the efficacy of SARS-CoV-2 vaccines in patients with non-oncological hematologic conditions are lacking. These include autoimmune cytopenias (autoimmune hemolytic anemia AIHA, immune thrombocytopenia ITP, and autoimmune neutropenia), and bone marrow failure syndromes (aplastic anemia, low risk myelodysplastic syndromes, and paroxysmal nocturnal hemoglobinuria). These conditions may relapse/reactivate after COVID-19 infection and vaccine. Read More

Blood Adv 2022 May 6. Epub 2022 May 6.

Shinshu Unversity Shool of Medicine, Japan.

Acquired pure red cell aplasia (PRCA) is a rare syndrome characterized by anemia with reticulocytopenia and a marked reduction in erythroid precursors. Given its rarity, the true incidence is largely unknown, and epidemiological data representing the general population, with a description of the full spectrum of etiologies, are scarce. An epidemiological study on PRCA in Japan conducted 30 years ago estimated the annual incidence as 0. Read More

Drug Des Devel Ther 2022 28;16:1231-1254. Epub 2022 Apr 28.

Hunan Academy of Chinese Medicine, Hunan University of Chinese Medicine, Changsha, Hunan, 410013, People's Republic of China.

Purpose: This study aimed to reveal the multicomponent synergy mechanisms of SWP based on network pharmacology and metabolomics for exploring the relationships of active ingredients, biological targets, and crucial metabolic pathways.

Materials: Network pharmacology, including TRRUST, GO, and KEGG, enrichment was used to discover the active ingredients and potential regulation mechanisms of SWP. LC-MS and multivariate data analysis method were further applied to analyze serum metabolomics profiling for discovering the potential metabolic mechanisms of SWP on AA induced by Cyclophosphamide (CTX) and 1-Acetyl-2-phenylhydrazine (APH). Read More

Transplant Cell Ther 2022 May 2. Epub 2022 May 2.

Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei,430030, China; Institute of Organ Transplantation, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei,430030, China. Electronic address:

Background: Haploidentical transplantation has become an alternative treatment option for aplastic anemia patients without matched sibling donors or matched unrelated donors. Recently, the post-transplantation cyclophosphamide (PTCY) regimen and granulocyte colony-stimulating factor (G-CSF)/antithymocyte globulin (ATG) regimen have become the most common protocols used worldwide.

Objective: We designed this retrospective study to compare the outcomes of patients receiving a modified post-transplantation cyclophosphamide (mPTCY) regimen versus the G-CSF/ATG regimen. Read More

Bone Marrow Transplant 2022 May 4. Epub 2022 May 4.

Peking University People's Hospital, Peking University Institute of Hematology, Beijing, China.

Pediatr Hematol Oncol 2022 May 3:1-7. Epub 2022 May 3.

Department of Blood and Marrow Transplantation, Bai Jerbai Wadia Hospital for Children, Mumbai, India.

Indian J Hematol Blood Transfus 2022 Apr 26;38(2):319-326. Epub 2021 May 26.

Department of Internal Medicine, Division of Hematology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Thrombosis is a leading cause of morbidity and mortality in paroxysmal nocturnal hemoglobinuria (PNH). Multiple factors are responsible for the thrombotic tendency in these patients. Endothelial progenitorcells (EPCs) originate from primitive hematopoietic stem cells. Read More

Front Oncol 2022 14;12:844978. Epub 2022 Apr 14.

Department of Hematology, The Second Hospital of Shanxi Medical University, Taiyuan, China.

Aim: Bone marrow biopsy is essential and necessary for the diagnosis of patients with aplastic anemia (AA), myelodysplastic syndromes (MDS), and acute myeloid leukemia (AML). However, the convolutional neural networks (CNN) model that automatically distinguished AA, MDS, and AML based on bone marrow smears has not been reported.

Methods: Image-net pretrained model of CNN was used to construct the recognition model. Read More

Semin Hematol 2022 Jan 31;59(1):54-55. Epub 2021 Dec 31.

Translational Hematology and Oncology Research Department, Taussig Cancer Center, Cleveland Clinic, Cleveland, OH; Leukemia Program, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH. Electronic address:

In the last 30 years, the field of aplastic anemia (AA), and more generally bone marrow failure syndromes, has undergone a multitude of new discoveries. The application of modern and sophisticated sequencing techniques unveiled a variety of genes associated with these disorders and contributed to a better understanding of the disease pathobiology. This advancement was paralleled by the discovery, clinical testing and subsequent approval of new drugs for the treatment of AA and associated disorders. Read More

Semin Hematol 2022 Jan 5;59(1):47-53. Epub 2022 Jan 5.

Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH; Leukemia Program, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH. Electronic address:

Advances in molecular technologies accelerated investigations of the hematopoietic stem cell (HSC) compartment in aplastic anemia (AA). Initially, stem cell biology approaches indicated a profound depletion of HSC pool, while studies of paroxysmal nocturnal hemoglobinuria (PNH), X-chromosome inactivation and cytogenetics provided the first evidence for the presence of clonality in the context of a contracted HSC compartment. More recently, the introduction of deep NGS allowed a more precise assessment of clonal expansions in AA. Read More

Semin Hematol 2022 Jan 11;59(1):38-46. Epub 2022 Jan 11.

Severe Aplastic Anemia Working Party (SAAWP) of the European Group for Bone Marrow Transplantation (EBMT), Leiden, Neitherlands; AORN San Giuseppe Moscati Avellino, Italy; Federico II University, Naples.

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by intravascular hemolysis, thrombosis and bone marrow failure. Prior to the availability of specific therapy, PNH led to the death of around half of affected individuals, mainly through thrombotic complications, with a particular grim prognosis for patients presenting with classic PNH. The anti-C5 monoclonal antibody eculizumab has revolutionized treatment, controlling intravascular hemolysis and thrombosis occurrence, with improved long-term survival. Read More

Semin Hematol 2022 Jan 31;59(1):30-37. Epub 2022 Jan 31.

Division of Hematology, Maine Medical Center, Portland, ME.

Fanconi anemia, telomeropathies and ribosomopathies are members of the inherited bone marrow failure syndromes, rare genetic disorders that lead to failure of hematopoiesis, developmental abnormalities, and cancer predisposition. While each disorder is caused by different genetic defects in seemingly disparate processes of DNA repair, telomere maintenance, or ribosome biogenesis, they appear to lead to a common pathway characterized by premature senescence of hematopoietic stem cells. Here we review the experimental data on senescence and inflammation underlying marrow failure and malignant transformation. Read More

Semin Hematol 2022 Jan 19;59(1):21-29. Epub 2022 Jan 19.

Division of Hematology, Hospital A Beneficiência Portuguesa, São Paulo, Brazil.

Severe aplastic anemia, a disease characterized by pancytopenia and a hypocellular marrow, is treatable by either immunosuppressive therapy (IST) or hematopoietic stem cell transplant. Much is understood about the immune-mediated pathophysiology of AA now, but the inciting factor remains elusive. Many groups around the globe contributed to understanding the disease pathophysiology and optimizing the IST regimen. Read More

Semin Hematol 2022 Jan 5;59(1):13-20. Epub 2022 Jan 5.

Hematology and Transplant Center, University Hospital "San Giovanni di Dio e Ruggi d'Aragona", Salerno, Italy; Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Baronissi, Salerno, Italy.

Bone marrow failure (BMF) syndromes are a heterogeneous group of benign hematological conditions characterized by uni- or multi-lineage marrow and/or peripheral blood cytopenia(s), and can be classified in constitutional or acquired syndromes based on pathophysiology. In inherited diseases, germline mutations occur in the hematopoietic stem and progenitor cell (HSPC) compartment causing a progressive loss of normal hematopoiesis, while in acquired syndromes, HPSC compartment disruption can be caused by an extrinsic direct damage by external cytotoxic agents on the stem cell pool or by an autoimmune attack against HSPCs. Aplastic anemia is an acquired immune-mediated BMF syndrome where marrow disruption is driven by a cytotoxic T cell-mediated autoimmune attack against HSPCs sustained by type I interferons that polarize the immune system toward T helper 1 responses in early phases and then toward T helper 17 and effector memory CD8 T cell in late stage and severe disease. Read More

Semin Hematol 2022 Jan 15;59(1):1-3. Epub 2022 Mar 15.

Editor Seminars in Hematology, Bethesda, MD. Electronic address:

Zhonghua Er Ke Za Zhi 2022 May;60(5):490-493

Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatric Hematology, National Key Discipline of Pediatrics (Capital Medical University),Key Laboratory of Major Disease in Children, Ministry of Education, Beijing 100045, China.

Cell Death Dis 2022 Apr 28;13(4):412. Epub 2022 Apr 28.

Department of Medical Imaging, Hematology, and Oncology, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.

In acquired immune aplastic anemia (AA), pathogenic cytotoxic Th1 cells are activated and expanded, driving an immune response against the hematopoietic stem and progenitor cells (HSPCs) that provokes cell depletion and causes bone marrow failure. However, additional HSPC defects may contribute to hematopoietic failure, reflecting on disease outcomes and response to immunosuppression. Here we derived induced pluripotent stem cells (iPSCs) from peripheral blood (PB) erythroblasts obtained from patients diagnosed with immune AA using non-integrating plasmids to model the disease. Read More

Blood Adv 2022 Apr 27. Epub 2022 Apr 27.

UMass Chan Medical School, Worcester, Massachusetts, United States.

Severe chronic neutropenia (SCN), defined as blood neutrophils < 0.5 x 109/L for more than 3 months, is an uncommon hematological condition associated with recurrent and severe bacterial infections. After short-term clinical trials showed the benefits of granulocyte colony-stimulating factor (G-CSF) treatment for SCN, the Severe Chronic Neutropenia International Registry (SCNIR) opened to determine the long-term benefits and safety of this treatment. Read More

Leukemia 2022 Apr 26. Epub 2022 Apr 26.

Department of Hematology, Faculty of Medicine, Institute of Medical Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.

To determine whether antigen presentation by HLA-DR on hematopoietic stem progenitor cells (HSPCs) is involved in the development of acquired aplastic anemia (AA), we studied the HLA-DR expression on CD45CD34CD38 cells in the peripheral blood of 61 AA patients including 23 patients possessing HLA-class I allele-lacking (HLA-class I[-]) leukocytes. HLA-DR-lacking (DR[-]) cells accounted for 13.0-57. Read More

Evid Based Complement Alternat Med 2022 14;2022:9033297. Epub 2022 Apr 14.

The First School of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China.

Formula (BSJPQYF), an experienced formula, has been used to treat aplastic anemia (AA) more than three decades. To determinate the effect of BSJPQYF on AA, we constructed an immune-mediated AA mouse model. All mice were divided into four groups: control, model, low dose (0. Read More

Pharmaceuticals (Basel) 2022 Mar 30;15(4). Epub 2022 Mar 30.

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

Thrombopoietin receptor agonists (TPO-RA) are currently indicated for the treatment of chronic immune thrombocytopenia and relapsed refractory aplastic anemia. However, the off-label use of these drugs is more and more frequent, including in the setting of aplasia secondary to chemotherapy and hemopoietic stem cell transplant (SCT). Growing evidence suggests that mechanisms of action of TPO-RA go beyond the TPO-receptor stimulation and point at the immunomodulating properties of these drugs. Read More

Blood Adv 2022 Apr 22. Epub 2022 Apr 22.

UT Southwestern Medical Center at Dallas, Dallas, Texas, United States.

Front Immunol 2022 1;13:810620. Epub 2022 Apr 1.

Department of Hematology, Fujian Institute of Hematology, Fujian Provincial Key Laboratory of Hematology, Fujian Medical University Union Hospital, Fuzhou, China.

Thrombocytopenia is a multifactorial condition that frequently involves concomitant defects in platelet production and clearance. The physiopathology of low platelet count in thrombocytopenia remains unclear. Sialylation on platelet membrane glycoprotein and follicular helper T cells (TFHs) are thought to be the novel platelet clearance pathways. Read More