33,326 results match your criteria Bone Marrow Failure


DAMPS and complement activation in platelet concentrates that induce adverse reactions in patients.

Transfusion 2022 Aug 11. Epub 2022 Aug 11.

Department Immunopathology, Sanquin Blood Supply, Amsterdam, The Netherlands.

Background: Patients with severe thrombocytopenia due to bone marrow failure and after chemotherapy are still treated with platelet transfusions. Platelet concentrates (PC) are associated with a high incidence of adverse reactions (AR). Platelet-derived damage-associated molecular patterns (DAMPS) and complement were proposed to play a role in the pathology of AR. Read More

View Article and Full-Text PDF

Pneumonia and seizures due to hypereosinophilic syndrome-organ damage and eosinophilia without synchronisation: A case report.

World J Clin Cases 2022 Jun;10(18):6325-6332

Department of Psychiatry, Shinyukai Nakae Hospital, Sapporo 001-0022, Japan.

Background: Hypereosinophilic syndrome (HES) is a condition characterized by increased eosinophil proliferation in the bone marrow, as well as tissue eosinophilia, often causing organ damage. The cause of the disease is unknown. Initial symptoms include fatigue, cough, shortness of breath, myalgia, angioedema, fever, and pneumonia. Read More

View Article and Full-Text PDF

A fatal SARS-coronavirus-2 induced bone marrow aplasia complicated with invasive fungal infection and severe neutropenic enterocolitis.

BMC Infect Dis 2022 Aug 9;22(1):682. Epub 2022 Aug 9.

Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Immunization against the coronavirus disease 2019 (COVID-19) began in January 2021 in Iran; nonetheless, due to a lack of vaccination among children under 12, this age group is still at risk of SARS-CoV-2 infection and its complications.

Case Presentation: SARS-CoV-2 infection was diagnosed in a 6-year-old girl who had previously been healthy but had developed a fever and pancytopenia. The bone marrow aspiration/biopsy demonstrated just hypocellular marrow without signs of leukemia. Read More

View Article and Full-Text PDF

Blood Cancer and the Heart: Light Chain Cardiomyopathy in Refractory Multiple Myeloma.

Case Rep Cardiol 2022 30;2022:7846846. Epub 2022 Jul 30.

Heart Center, Banner University Medical Center, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.

We report a case of a 57-year-old woman with a history of multiple myeloma (MM) and light chain (AL) amyloidosis who presented due to worsening dyspnea on exertion. Her MM has been refractory to multiple chemotherapy regimens and two autologous bone marrow transplantation. Diagnostic evaluations including serum kappa and lambda chains, echocardiogram, pyrophosphate cardiac scan, and cardiac magnetic resonance were indicative of a progression to AL cardiomyopathy. Read More

View Article and Full-Text PDF

Severe aplastic anaemia after serial vaccinations for SARS-CoV-2, pneumococcus and seasonal influenza.

EJHaem 2022 May 4. Epub 2022 May 4.

Division of Hematology and Oncology Department of Medicine Hospital of the University of Pennsylvania Philadelphia Pennsylvania USA.

We present a 67-year-old woman who developed progressive pancytopenia over 10 months, concomitant with administration of severe adult respiratory syndrome coronavirus-2 (SARS-CoV-2), pneumococcal and influenza vaccines. She developed mild leukopenia ∼2 weeks after the SARS-CoV-2 mRNA vaccine sequence, with progressive symptoms after subsequent vaccines, eventually developing severe aplastic anaemia (SAA). While there have been several reports of vaccine-related SAA, at time of submission, our case is the first reported to develop after the Moderna mRNA SARS-CoV-2 vaccine, as well as the first to document the gradual development of SAA over the course of many vaccine exposures. Read More

View Article and Full-Text PDF

GSDMD-dependent neutrophil extracellular traps promote macrophage-to-myofibroblast transition and renal fibrosis in obstructive nephropathy.

Cell Death Dis 2022 Aug 8;13(8):693. Epub 2022 Aug 8.

Department of Nephrology, Blood Purification Research Center, the First Affiliated Hospital, Fujian Medical University, Fuzhou, China.

Renal fibrosis is a common consequence of various progressive nephropathies, including obstructive nephropathy, and ultimately leads to kidney failure. Infiltration of inflammatory cells is a prominent feature of renal injury after draining blockages from the kidney, and correlates closely with the development of renal fibrosis. However, the underlying molecular mechanism behind the promotion of renal fibrosis by inflammatory cells remains unclear. Read More

View Article and Full-Text PDF

Editorial Commentary: Rotator Cuff Repairs Fail at an Alarmingly High Rate During Long-Term Follow-Up: Graft Augmentation and Biologics May Improve Future Outcomes.

Arthroscopy 2022 Aug;38(8):2413-2416

Wake Forest University School of Medicine (N.A.T.).

Rotator cuff repairs (RCR) frequently fail to heal, particularly those with advanced fatty infiltration, supraspinatus and infraspinatus atrophy, narrowed acromiohumeral distance, and large-to-massive tear size. Unfortunately, the longer the follow up, the more sobering the statistics, with some reported retear rates ranging up to 94%. Importantly, recent long-term radiographic assessments after primary RCR reveal direct correlations between failure and patient-reported outcomes, functional deterioration, and ultimately, progression of glenohumeral arthritis and/or frank cuff tear arthropathy. Read More

View Article and Full-Text PDF

The mouse homolog of the mutant WASp responsible for human X-linked neutropenia renders granulopoiesis ineffective.

Biochem Biophys Res Commun 2022 Sep 14;622:177-183. Epub 2022 Jul 14.

Division of Molecular Therapy, Advanced Clinical Research Center, The Institute of Medical Science, The University of Tokyo, Japan; Institute of Innovation Advancement, Tokyo Medical and Dental University, Japan.

Severe congenital neutropenia (SCN) is characterized by severe neutropenia and recurrent critical infections. X-linked neutropenia (XLN) is caused by a gain-of-function mutation in the Wiskott-Aldrich syndrome gene (WAS), the product of which (WASp) is expressed only in blood cells, especially during neutrophil maturation. To investigate the mechanism of neutropenia, we established a novel knock-in mouse line expressing WASp-I292T. Read More

View Article and Full-Text PDF
September 2022

Germline thymidylate synthase deficiency impacts nucleotide metabolism and causes dyskeratosis congenita.

Am J Hum Genet 2022 08;109(8):1472-1483

Genomics and Child Health, Blizard Institute, Queen Mary University of London, Newark Street, London E1 2AT, UK; Barts Health NHS Trust, London, UK.

Dyskeratosis congenita (DC) is an inherited bone-marrow-failure disorder characterized by a triad of mucocutaneous features that include abnormal skin pigmentation, nail dystrophy, and oral leucoplakia. Despite the identification of several genetic variants that cause DC, a significant proportion of probands remain without a molecular diagnosis. In a cohort of eight independent DC-affected families, we have identified a remarkable series of heterozygous germline variants in the gene encoding thymidylate synthase (TYMS). Read More

View Article and Full-Text PDF

The biology and management of dyskeratosis congenita and related disorders of telomeres.

Expert Rev Hematol 2022 Aug 8:1-12. Epub 2022 Aug 8.

Centre for Genomics and Child Health, Blizard Institute, Barts and The London Faculty of Medicine and Dentistry, Queen Mary University of London, London, UK.

Background: Dyskeratosis congenita (DC) is a multisystem syndrome characterized by mucocutaneous abnormalities, bone marrow failure, and predisposition to cancer. Studies over the last 25 years have led to the identification of 18 disease genes. These have a principal role in telomere maintenance, and patients usually have very short/abnormal telomeres. Read More

View Article and Full-Text PDF

Allogeneic Hematopoietic Stem Cell Transplantation After Prior Lung Transplantation for Hereditary Pulmonary Alveolar Proteinosis: A Case Report.

Front Immunol 2022 14;13:931153. Epub 2022 Jul 14.

Department of Department of Chronic Diseases and Metabolism (CHROMETA), Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), KU Leuven, Leuven, Belgium.

Pulmonary alveolar proteinosis (PAP) is a rare, diffuse lung disorder characterized by surfactant accumulation in the small airways due to defective clearance by alveolar macrophages, resulting in impaired gas exchange. Whole lung lavage is the current standard of care treatment for PAP. Lung transplantation is an accepted treatment option when whole lung lavage or other experimental treatment options are ineffective, or in case of extensive pulmonary fibrosis secondary to PAP. Read More

View Article and Full-Text PDF

Challenges in the interpretation of a germline TERT variant in a patient with juvenile myelomonocytic leukemia.

Pediatr Blood Cancer 2022 Aug 4:e29909. Epub 2022 Aug 4.

Department of Pediatrics, Oncology and Haematology, Medical University of Lodz, Lodz, Poland.

Dyskeratosis congenita (DC) is a bone marrow failure syndrome with extrahematopoietic abnormalities. DC is a paradigmatic telomere biology disorder (TBD) caused by germline mutations in genes responsible for telomere maintenance including TERT. Cryptic TBD is a bone marrow failure syndrome due to premature telomere shortening but without additional symptoms, frequently clinically indistinguishable from severe aplastic anemia (SAA) or hypoplastic myelodysplastic syndrome. Read More

View Article and Full-Text PDF

Efficacy of Sinopharm Vaccine among Stem Cell Transplant Recipient during two peaks of Delta and Omicron variants of COVID-19.

Curr Drug Saf 2022 Aug 1. Epub 2022 Aug 1.

Nurse of BMT, Mashhad University of Medical Sciences, Montaserieh Hospital, Mashhad, Iran.

Background: Considering the dearth of research on the complications of Sinopharm coronavirus disease 2019 (COVID-19) vaccine in the immunocompromised individuals and the lack of available data on COVID-19 vaccination from Iran.

Objective: This study aimed to investigate the efficacy of vaccine of Sinopharm COVID-19 vaccine and its complications in bone marrow transplant (BMT) recipients.

Methods: This was a retrospective cross-sectional study was conducted on 250 patients with BMT who were referred to Montaserieh Hospital, Mashhad, Iran. Read More

View Article and Full-Text PDF

Transplantation of mesenchymal stem cells for prevention of acute myocardial infarction induced heart failure: study protocol of a phase III randomized clinical trial (Prevent-TAHA8).

Trials 2022 Aug 4;23(1):632. Epub 2022 Aug 4.

Department of Pathology, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Results from recent clinical trials on bone marrow mononuclear cell (BM-MNC) transplantation show that this intervention can help reduce the incidence of heart failure (HF) after acute myocardial infarction (AMI). However, no study has evaluated the effect of the transplantation of mesenchymal stem cells (MSCs) on a clinical endpoint such as HF.

Methods: This single-blinded, randomized, multicenter trial aims to establish whether the intracoronary infusion of umbilical cord-derived Wharton's jelly MSCs (WJ-MSCs) helps prevent HF development after AMI. Read More

View Article and Full-Text PDF

Anti-COX-2 autoantibody is a novel biomarker of immune aplastic anemia.

Leukemia 2022 Aug 5. Epub 2022 Aug 5.

Hematology Research Unit Helsinki, University of Helsinki and Department of Hematology, Helsinki University Hospital Comprehensive Cancer Center, Helsinki, Finland.

In immune aplastic anemia (IAA), severe pancytopenia results from the immune-mediated destruction of hematopoietic stem cells. Several autoantibodies have been reported, but no clinically applicable autoantibody tests are available for IAA. We screened autoantibodies using a microarray containing >9000 proteins and validated the findings in a large international cohort of IAA patients (n = 405) and controls (n = 815). Read More

View Article and Full-Text PDF

Isolation of Swine Bone Marrow Lin-/CD45-/CD133 + Cells and Cardio-protective Effects of its Exosomes.

Stem Cell Rev Rep 2022 Aug 4. Epub 2022 Aug 4.

Medical College of Yangzhou University, Yangzhou, 225001, Jiangsu, China.

Background: The identification in murine bone marrow (BM) of CD133 + /Lin-/CD45- cells, possessing several features of pluripotent stem cells, encouraged us to investigate if similar population of cells could be also isolated from the swine BM. Heart failure is the terminal stage of many cardiovascular diseases, and its key pathological basis is cardiac fibrosis (CF). Research showed that stem cell derived exosomes may play a critical role in cardiac fibrosis. Read More

View Article and Full-Text PDF

[Drug-induced bone marrow changes].

Authors:
Hans H Kreipe

Pathologie (Heidelb) 2022 Jul 9;43(4):256-262. Epub 2022 Jun 9.

Institut für Pathologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.

Cytomorphological and histomorphological analysis of bone marrow cells in cases with abnormal production of blood cells must always take the unwanted side effects of drugs into account. Drugs can affect single lineages or the complete bone marrow. Besides quantitative phenomena consisting of hypoplasia or hyperplasia, maturation of blood cells may be disturbed. Read More

View Article and Full-Text PDF

Zebrafish models of inflammation in hematopoietic development and disease.

Front Cell Dev Biol 2022 18;10:955658. Epub 2022 Jul 18.

Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada.

Zebrafish offer an excellent tool for studying the vertebrate hematopoietic system thanks to a highly conserved and rapidly developing hematopoietic program, genetic amenability, optical transparency, and experimental accessibility. Zebrafish studies have contributed to our understanding of hematopoiesis, a complex process regulated by signaling cues, inflammation being crucial among them. Hematopoietic stem cells (HSCs) are multipotent cells producing all the functional blood cells, including immune cells. Read More

View Article and Full-Text PDF

Variable Clinical Features in a Large Family With Diamond Blackfan Anemia Caused by a Pathogenic Missense Mutation in .

Front Genet 2022 18;13:914141. Epub 2022 Jul 18.

Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD, United States.

Diamond Blackfan anemia (DBA) is an autosomal dominant ribosomopathy caused predominantly by pathogenic germline variants in ribosomal protein genes. It is characterized by failure of red blood cell production, and common features include congenital malformations and cancer predisposition. Mainstays of treatment are corticosteroids, red blood cell transfusions, and hematologic stem cell transplantation (HSCT). Read More

View Article and Full-Text PDF

A Case Report of Hemophagocytic Lymphohistiocytosis (HLH) - An Unusual Complication of Dengue Infection.

Cureus 2022 Jul 1;14(7):e26504. Epub 2022 Jul 1.

Department of Medicine, Datta Meghe Institute of Medical Science, Wardha, IND.

Hemophagocytic lymphohistiocytosis (HLH) is an autoimmune phenomenon characterized by reactive hyperactivity of cytotoxic T cells and histiocytes, leading to hypercytokinemic injury to cells and organ system, which leads to multiorgan dysfunction and ultimate failure. Epstein-Barr virus (EBV) is most commonly associated with secondary HLH with high mortality, but increasing evidence suggests the association of the dengue virus. When associated with dengue infection, it carries a grave prognosis and correlates with the disease severity. Read More

View Article and Full-Text PDF

[Cunninghamella bertholletiae-infective endocarditis complicated by tricuspid valve giant vegetation in a patient with aplastic anemia].

Rinsho Ketsueki 2022 ;63(7):740-745

Department of Hematology and Oncology, Japanese Red Cross Society Himeji Hospital.

A 62-year-old female was presented to the hospital of the current study for pancytopenia and was diagnosed with severe aplastic anemia. She was treated with a combination therapy of antithymocyte globulin, cyclosporine A, and eltrombopag. The patient also presented with febrile neutropenia after commencement of the treatment and did not respond to the various antibiotics and antifungal agents. Read More

View Article and Full-Text PDF

Mcm2 hypomorph leads to acute leukemia or hematopoietic stem cell failure, dependent on genetic context.

FASEB J 2022 Sep;36(9):e22430

Genetics Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.

Minichromosome maintenance proteins (Mcm2-7) form a hexameric complex that unwinds DNA ahead of a replicative fork. The deficiency of Mcm proteins leads to replicative stress and consequent genomic instability. Mice with a germline insertion of a Cre cassette into the 3'UTR of the Mcm2 gene (designated Mcm2 ) have decreased Mcm2 expression and invariably develop precursor T-cell lymphoblastic leukemia/lymphoma (pre-T LBL), due to 100-1000 kb deletions involving important tumor suppressor genes. Read More

View Article and Full-Text PDF
September 2022

Immunophenotypic changes of monocytes in myelodysplastic syndrome and clinical significance.

Clin Exp Med 2022 Aug 2. Epub 2022 Aug 2.

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

Background: Myelodysplastic syndrome (MDS) is a type of heterogeneous myeloid clonal disorder usually diagnosed based on a combination of multiple laboratory examinations, including analysis of peripheral blood cells, bone marrow cell morphology and cytogenetics. However, there is a certain difficulty in cases with no distinct changes in hematology and marrow cell morphology.

Methods: We adopt flow cytometry to quantitatively analyze the immunophenotypic changes of marrow monocytes according to the surface antigens and their combinations at different differentiation stages, so as to study the changes of monocytes during differentiation in patients with bone marrow failure. Read More

View Article and Full-Text PDF

Does immune destruction drive all forms of bone marrow failure?

J Clin Invest 2022 Aug;132(15)

Cell Therapy and Transplant Section, Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

Current paradigms of bone marrow failure (BMF) pathophysiology suggest that immune-mediated destruction of hematopoietic stem and progenitor cells (HSPCs) drives acquired aplastic anemia. In contrast, loss of HSPCs due to senescence and/or apoptosis causes BMF in inherited BMF syndromes. In this issue of the JCI, Casado and colleagues challenge this dichotomous conception by demonstrating that NK cell-dependent, immune-mediated hematopoietic suppression and HSPC clearance drive BMF in Fanconi anemia (FA). Read More

View Article and Full-Text PDF

Levamisole Suppresses CD4 T-Cell Proliferation and Antigen-Presenting Cell Activation in Aplastic Anemia by Regulating the JAK/STAT and TLR Signaling Pathways.

Front Immunol 2022 14;13:907808. Epub 2022 Jul 14.

State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, China.

Aplastic anemia (AA) is a life-threatening disease primarily caused by a metabolic disorder and an altered immune response in the bone marrow (BM) microenvironment, where cytotoxic immune cells attack resident cells and lead to hematopoietic failure. We previously reported an efficient strategy by applying cyclosporin (CSA) combined with levamisole (CSA+LMS-based regimen) in the treatment of AA, but the immunoregulatory mechanism of LMS was still unclear. Here, the therapeutic effects of LMS were examined using the BM failure murine model. Read More

View Article and Full-Text PDF

Allogeneic Hematopoietic Cell Transplantation for Patients With Deficiency of Adenosine Deaminase 2 (DADA2): Approaches, Obstacles and Special Considerations.

Front Immunol 2022 7;13:932385. Epub 2022 Jul 7.

Department of Pediatrics, Microbiology, Immunology, and Transplantation, The European Reference Network Rare Immunodeficiency Autoinflammatory and Autoimmune Diseases Network (ERN RITA) Core Center, University Hospitals Leuven, Katholieke Universiteit (KU) Leuven, Leuven, Belgium.

Deficiency of adenosine deaminase 2 (DADA2) is an inherited autosomal recessive disease characterized by autoinflammation (recurrent fever), vasculopathy (livedo racemosa, polyarteritis nodosa, lacunar ischemic strokes, and intracranial hemorrhages, end organ vasculitis), immunodeficiency, lymphoproliferation, immune cytopenias, and bone marrow failure. Allogeneic hematopoietic cell transplantation (HCT) is curative for DADA2 as it reverses the hematological, immune and vascular phenotype of DADA2. The primary goal of HCT in DADA2, like in other non-malignant diseases, is engraftment with the establishment of normal hematopoiesis and normal immune function. Read More

View Article and Full-Text PDF

Acute hepatitis-like presentation with cholestasis of CBFB-MYH11-positive acute myeloid leukemia in an adult male: a case report.

J Med Case Rep 2022 Jul 31;16(1):294. Epub 2022 Jul 31.

Department of Translational and Precision Medicine, Sapienza University, Viale dell'Università 37, 00185, Rome, Italy.

Background: Liver involvement in adults with acute myeloid leukemia is uncommon. Most of the case reports describe acute liver failure or obstructive jaundice, while acute hepatitis is rarely mentioned. We report a patient with acute myeloid leukemia who presented with clinical, biochemical, and radiological signs of acute hepatitis that totally regressed after chemotherapy. Read More

View Article and Full-Text PDF

Haploidentical bone marrow transplantation in patients with relapsed or refractory severe aplastic anaemia in the USA (BMT CTN 1502): a multicentre, single-arm, phase 2 trial.

Lancet Haematol 2022 Jul 27. Epub 2022 Jul 27.

Huntsman Cancer Institute, Salt Lake City, UT, USA.

Background: Relapsed severe aplastic anaemia is a marrow failure disorder with high morbidity and mortality. It is often treated with bone marrow transplantation at relapse post-immunosuppressive therapy, but under-represented minorities often cannot find a suitably matched donor. This study aimed to understand the 1-year overall survival in patients with relapsed or refractory severe aplastic anaemia after haploidentical bone marrow transplantation. Read More

View Article and Full-Text PDF

The efficacy and safety of allogeneic stem cell transplantation in Mevalonate Kinase Deficiency.

Pediatr Rheumatol Online J 2022 Jul 29;20(1):56. Epub 2022 Jul 29.

Department of Pediatrics, University Medical Center Utrecht, 3584, EA, Utrecht, the Netherlands.

Objectives: Mevalonate kinase deficiency (MKD) is a rare autoinflammatory syndrome. Several reports have described allogeneic hematopoietic stem cell transplantation in severely affected patients, sometimes with promising results. In view of the scarcity of data, this study aims to analyse the efficacy and safety of allogeneic hematopoietic stem cell transplantation (HSCT) to give a more complete overview of this treatment. Read More

View Article and Full-Text PDF