21,778 results match your criteria Bone Marrow Failure


Binding of FANCI-FANCD2 Complex to RNA and R-Loops Stimulates Robust FANCD2 Monoubiquitination.

Cell Rep 2019 Jan;26(3):564-572.e5

Department of Pediatrics, Yale Medical School, New Haven, CT 06520, USA; Department of Pathology, Yale Medical School, New Haven, CT 06520, USA. Electronic address:

Fanconi anemia (FA) is characterized by developmental abnormalities, bone marrow failure, and cancer predisposition. FA cells are hypersensitive to DNA replicative stress and accumulate co-transcriptional R-loops. Here, we use the Damage At RNA Transcription assay to reveal colocalization of FANCD2 with R-loops in a highly transcribed genomic locus upon DNA damage. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.celrep.2018.12.084DOI Listing
January 2019

Rare Isolated Renal Involvement with Marginal Zone B Cell Lymphoma: A Case Report with Literature Review of Contemporary Management Strategies.

Cureus 2018 Nov 8;10(11):e3560. Epub 2018 Nov 8.

Hematology and Oncology, The University of Arizona, Tucson, USA.

Marginal zone B cell lymphomas are divided into nodal, extranodal and splenic types. Renal involvement by extranodal B cell lymphoma is extremely rare with an incidence of about 0.1%. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.7759/cureus.3560DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6325030PMC
November 2018

A Case of Adalimumab-associated Pancytopenia and Reversible Lymphadenopathy.

Cureus 2018 Oct 22;10(10):e3477. Epub 2018 Oct 22.

Internal Medicine, Penn State Health Milton S. Hershey Medical Center, Hershey, USA.

Adalimumab is a recombinant monoclonal antibody to tumor necrosis factor alpha (TNFα) used in the treatment of inflammatory and autoimmune conditions, including ulcerative colitis (UC). Adverse side effects include infection and injection-site cutaneous reactions; however, rare adverse events such as pancytopenia have been recorded. Here we describe a case of pancytopenia and reversible lymphadenopathy related to adalimumab administration in a patient with UC. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.7759/cureus.3477DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6318094PMC
October 2018

Autoimmune myelofibrosis: a rare haematological involvement in systemic lupus erythematosus.

BMJ Case Rep 2019 Jan 14;12(1). Epub 2019 Jan 14.

Internal Medicine and Infectious Disease Department, Centre Hospitalier Sud Ile de France, Melun, France.

Autoimmune myelofibrosis is a distinct clinicopathological entity that occurs with autoimmune disorders. We report the case of a 44-year-old woman admitted with pancytopenia and clinical features of systemic lupus erythematosus, Sjogren's syndrome and antiphospholipid antibodies syndrome. Bone marrow biopsy showed decreased global cells and an increase of reticulin fibres on argentic coloration, consistent with myelofibrosis. Read More

View Article

Download full-text PDF

Source
http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22752
Publisher Site
http://dx.doi.org/10.1136/bcr-2018-227520DOI Listing
January 2019
1 Read

Exclusive dependence of IL-10Rα signalling on intestinal microbiota homeostasis and control of whipworm infection.

PLoS Pathog 2019 Jan 14;15(1):e1007265. Epub 2019 Jan 14.

Wellcome Sanger Institute, Wellcome Genome Campus, Hinxton, United Kingdom.

The whipworm Trichuris trichiura is a soil-transmitted helminth that dwells in the epithelium of the caecum and proximal colon of their hosts causing the human disease, trichuriasis. Trichuriasis is characterized by colitis attributed to the inflammatory response elicited by the parasite while tunnelling through intestinal epithelial cells (IECs). The IL-10 family of receptors, comprising combinations of subunits IL-10Rα, IL-10Rβ, IL-22Rα and IL-28Rα, modulates intestinal inflammatory responses. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1371/journal.ppat.1007265DOI Listing
January 2019

Determination of the effects of bone marrow derived mesenchymal stem cells and ovarian stromal stem cells on follicular maturation in cyclophosphamide induced ovarian failure in rats.

Taiwan J Obstet Gynecol 2019 Jan;58(1):53-59

Department of Medical Biochemistry, Faculty of Medicine, Gazi University, Ankara, Turkey.

Objective: Chemotherapy causes depletion of primordial follicles that leads to premature ovarian failure in female cancer survivals. We investigated the effect of bone marrow derived mesenchymal (BMMSCs) and ovarian stromal stem cells (OSSCs) on follicle maturation in chemotherapy induced ovarian failure.

Material And Methods: Thirty six Wistar Albino female rats were divided into three groups. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S10284559183028
Publisher Site
http://dx.doi.org/10.1016/j.tjog.2018.11.010DOI Listing
January 2019
1 Read

Bone Marrow Transplantation for Adolescents and Young Adults with Sickle Cell Disease: Results of a Prospective Multicenter Pilot Study.

Am J Hematol 2019 Jan 13. Epub 2019 Jan 13.

Hematology/Oncology, Children's Hosp Oakland, United States.

We conducted a multicenter pilot investigation of the safety and feasibility of bone marrow transplantation (BMT) in adults with severe sickle cell disease (SCD) (NCT 01565616) using a reduced toxicity preparative regimen of Busulfan (13.2 mg/kg), Fludarabine (175 mg/m2) and Thymoglobulin (6 mg/kg) and cyclosporine or tacrolimus and methotrexate for graft-versus-host disease (GVHD) prophylaxis. Twenty-two patients (median age 22 years; range 17-36) were enrolled at 8 centers. Read More

View Article

Download full-text PDF

Source
http://doi.wiley.com/10.1002/ajh.25401
Publisher Site
http://dx.doi.org/10.1002/ajh.25401DOI Listing
January 2019
3 Reads

The Progress of Traditional Chinese Medicine in the Treatment of Aplastic Anemia.

J Transl Int Med 2018 Dec 31;6(4):159-164. Epub 2018 Dec 31.

Department of Hematology, First Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China.

Aplastic anemia (AA) is a common hematologic disease that is characterized by hematopoietic failure of the bone marrow and pancytopenia of the peripheral blood, which can be divided into severe and non-severe aplastic ane-mia, or acute and chronic aplastic anemia according to the severity of the disease and the progress of the clinical course. During the past years, the advantages of Traditional Chinese Medicine (TCM) on the treatment of AA have been well clarified and its theory system has been improved as well. This review is mainly on representing the pathogenesis, therapeutic principle and method, research progression, and advantages of TCM on AA. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2478/jtim-2018-0031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326026PMC
December 2018

Chronic active EBV infection associated with NK cell lymphoma and hemophagocytic lymphohistiocytosis in a 27-year-old woman: A case report.

Medicine (Baltimore) 2019 Jan;98(2):e14032

Department of Infectious Diseases, The Third Affiliated Hospital of Hebei Medical University, Shijiazhuang.

Rationale: Chronic active Epstein-Barr virus infection (CAEBV) is a common infectious disease that often affects multiple organs or systems. However, it is liable to be neglected and misdiagnosed owing to its insidious onset, lack of specific findings in the early phase, and a general lack of awareness among clinicians. PATIENT CONCERNS:: a 27-year-old woman case has been described who was initially misdiagnosed as drug-induced liver injury due to onset presentation of mild splenomegaly, recurrent liver dysfunction, and disputable pathological evidence of liver biopsy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MD.0000000000014032DOI Listing
January 2019
2 Reads
5.723 Impact Factor

What are the most promising new agents in myelodysplastic syndromes?

Curr Opin Hematol 2019 Jan 9. Epub 2019 Jan 9.

Smilow Cancer Center at Yale New Haven Hospital, New Haven, Connecticut, USA.

Purpose Of Review: Myelodysplastic syndromes (MDS) are a diverse group of clonal disorders of hematopoietic stem or progenitor cells that represent the most common class of acquired bone marrow failure syndromes in adults. Despite significant improvement in the pathologic insight into this group of disorders, therapeutic options remain limited and allogeneic hematopoietic stem-cell transplantation is the only treatment that can induce long-term remission in patients with MDS. The goals of therapy for MDS are based on disease prognostication, with a focus of minimizing transfusion dependence and preserving quality of life in low-risk groups and preventing progression of disease to acute myeloid leukemia in high-risk groups. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MOH.0000000000000483DOI Listing
January 2019
1 Read

A rare BCR-ABL1 transcript in Philadelphia-positive acute myeloid leukemia: case report and literature review.

BMC Cancer 2019 Jan 10;19(1):50. Epub 2019 Jan 10.

Department of Clinical and Molecular Medicine, Hematology Sant'Andrea University Hospital, Sapienza University of Rome, Rome, Italy.

Background: Philadelphia (Ph) chromosome results from the reciprocal translocation t(9;22)(q34.1;q11.2) and is diagnostic for chronic myeloid leukemia (CML). Read More

View Article

Download full-text PDF

Source
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-
Publisher Site
http://dx.doi.org/10.1186/s12885-019-5265-5DOI Listing
January 2019
2 Reads

The complexity of an overlap type resistant cryoglobulinemia: a case report and review of the literature.

Clin Rheumatol 2019 Jan 10. Epub 2019 Jan 10.

Department of Internal Medicine C, Wolfson Medical Center, 61 Halochamim Street, 58100, Holon, Israel.

Type I cryoglobulinemia is associated with B cell proliferative diseases, whereas essential mixed cryoglobulinemia is classically associated with infections, malignancy, and autoimmune diseases, but may be idiopathic. Prognosis in patients with grave manifestations and renal involvement is often poor. We report a case of a 40-year-old woman, 2 weeks post-partum for pre-eclampsia who was hospitalized with nephritic syndrome and acute renal failure. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10067-018-04423-yDOI Listing
January 2019

Hyperthyroidism with Biventricular Heart Failure and Cirrhotic Transformation of the Liver.

Case Rep Cardiol 2018 9;2018:3861340. Epub 2018 Dec 9.

Reading Hospital, Tower Health System, West Reading, PA, USA.

Cardiovascular symptoms remain the most common presenting features and leading causes of death in hyperthyroidism. We report a young female with reported thyroid disease and medication noncompliance presenting with atrial fibrillation, severe atrioventricular regurgitation, severely dilated right heart with reduced function, and moderate pulmonary hypertension (PH), which was further complicated by congestive liver injury with ascites and pancytopenia. Thyroid work-up revealed suppressed TSH, elevated free T4 and T3 along with elevated anti-thyroglobulin antibodies, thyroid peroxidase antibodies, and thyroid-stimulating immunoglobulin, suggesting Graves' thyrotoxicosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2018/3861340DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305034PMC
December 2018

The role of p53 in developmental syndromes.

J Mol Cell Biol 2019 Jan 8. Epub 2019 Jan 8.

Division of Radiation and Cancer Biology in the Department of Radiation Oncology, Stanford University School of Medicine, Stanford, CA, USA.

While it is well appreciated that loss of the p53 tumor suppressor protein promotes cancer, growing evidence indicates that increased p53 activity underlies the developmental defects in a wide range of genetic syndromes. The inherited or de novo mutations that cause these syndromes affect diverse cellular processes, such as ribosome biogenesis, DNA repair, and centriole duplication, and analysis of human patient samples and mouse models demonstrates that disrupting these cellular processes can activate the p53 pathway. Importantly, many of the developmental defects in mouse models of these syndromes can be rescued by loss of p53, indicating that inappropriate p53 activation directly contributes to their pathogenesis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/jmcb/mjy087DOI Listing
January 2019

Persistence of in the Bone Marrow of Infected Mice.

J Immunol Res 2018 3;2018:5370414. Epub 2018 Dec 3.

Programa de Investigación en Enfermedades Tropicales, Escuela de Medicina Veterinaria, Universidad Nacional, Heredia, Costa Rica.

Brucellosis is a zoonotic bacterial infection that may persist for long periods causing relapses in antibiotic-treated patients. The ability of to develop chronic infections is linked to their capacity to invade and replicate within the mononuclear phagocyte system, including the bone marrow (BM). Persistence of in the BM has been associated with hematological complications such as neutropenia, thrombocytopenia, anemia, and pancytopenia in human patients. Read More

View Article

Download full-text PDF

Source
https://www.hindawi.com/journals/jir/2018/5370414/
Publisher Site
http://dx.doi.org/10.1155/2018/5370414DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6304906PMC
December 2018
1 Read

Single-nucleotide human disease mutation inactivates a blood-regenerative GATA2 enhancer.

J Clin Invest 2019 Jan 8. Epub 2019 Jan 8.

The development and function of stem and progenitor cells that produce blood cells are vital in physiology. GATA2 mutations cause GATA-2-deficiency syndrome involving immunodeficiency, myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). GATA-2 physiological activities necessitate that it be strictly regulated, and cell type-specific enhancers fulfill this role. Read More

View Article

Download full-text PDF

Source
http://www.jci.org/articles/view/122694
Publisher Site
http://dx.doi.org/10.1172/JCI122694DOI Listing
January 2019
3 Reads

Chronic progressive disseminated histoplasmosis in a renal transplant recipient.

Med Mycol Case Rep 2019 Mar 21;23:53-54. Epub 2018 Dec 21.

Division of Infectious Disease, Department of Internal Medicine, St. John Hospital and Medical Center, 19251 Mack Avenue, Suite 340, Detroit 48236, MI, United States of America.

Hematopoietic and solid organ transplant recipients are at increased risk of opportunistic infections and infections usually are severe due to impaired cell mediated immunity. We report an unusual case of disseminated histoplasmosis in a renal transplant recipient manifesting with a chronic progressive course over several years. After starting treatment with itraconazole, the patient showed marked improvement in his symptoms and had clinical resolution. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.mmcr.2018.12.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6313815PMC

Molecular Patterns of Resistance Among Strains in South-Western Poland.

Front Microbiol 2018 18;9:3154. Epub 2018 Dec 18.

Department of Microbiology, Wrocław Medical University, Wrocław, Poland.

Treatment failure of infection is caused mainly by progressive antibiotic resistance among strains. In Poland, the prevalence of strains resistant to metronidazole is higher than in other developed countries, reaching almost 50%, and resistance to clarithromycin is as high as 30% and is still increasing, contributing to the failure of first-line therapy in approximately 70% of patients. Moreover, the introduction of levofloxacin to eradication therapy of infection quickly led to the emergence of resistant strains. Read More

View Article

Download full-text PDF

Source
https://www.frontiersin.org/article/10.3389/fmicb.2018.03154
Publisher Site
http://dx.doi.org/10.3389/fmicb.2018.03154DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305312PMC
December 2018
1 Read

Human iPSC-based model of severe congenital neutropenia reveals elevated UPR and DNA damage in CD34 cells preceding leukemic transformation.

Exp Hematol 2019 Jan 4. Epub 2019 Jan 4.

Department of Oncology, Hematology, Immunology, Rheumatology, and Pulmonology, University Hospital Tuebingen, Tuebingen, Germany. Electronic address:

We describe the establishment of an embryoid-body-based protocol for hematopoietic/myeloid differentiation of human induced pluripotent stem cells that allows the generation of CD34 cells or mature myeloid cells in vitro. Using this model, we were able to recapitulate the defective granulocytic differentiation in patients with severe congenital neutropenia (CN), an inherited preleukemia bone marrow failure syndrome. Importantly, in vitro maturation arrest of granulopoiesis was associated with an elevated unfolded protein response (UPR) and enhanced expression of the cell cycle inhibitor p21. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.exphem.2018.12.006DOI Listing
January 2019

Association of bone mineral density and fat fraction with magnetic susceptibility in inflamed trabecular bone.

Magn Reson Med 2019 Jan 7. Epub 2019 Jan 7.

Department of Medical Physics and Biomedical Engineering, University College London, United Kingdom.

Purpose: To evaluate the relationship between bone mineral density (BMD) and magnetic susceptibility, and between proton density fat fraction and susceptibility, in inflamed trabecular bone.

Methods: Two different phantoms modeling the fat fraction (FF) and BMD values of healthy bone marrow and disease states were scanned using a multiecho gradient echo acquisition at 3T. After correction for fat-water chemical shift, susceptibility mapping was performed, and susceptibility measurements were compared with BMD and FF values using linear regression. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/mrm.27634DOI Listing
January 2019

Resistant type II cryoglobulinaemic vasculitis successfully treated with bortezomib in a patient with SLE.

BMJ Case Rep 2019 Jan 4;12(1). Epub 2019 Jan 4.

Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.

We report an interesting case of hepatitis C virus-negative type II cryoglobulinaemic vasculitis (CV) in a patient with a background history of systemic lupus erythematosus. The type II CV became less responsive to traditional treatments over time and culminated in an intensive care unit admission with critical multiorgan failure. A detailed flow cytometric evaluation of the bone marrow proved to be helpful in treatment. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2018-226083DOI Listing
January 2019

A Young Woman With an Erythematous Rash, Fever, Tachycardia, and Pancytopenia: Answer.

Am J Dermatopathol 2019 Jan;41(1):73-74

Department of Dermatology, Dell Medical School, The University of Texas, Austin, TX.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/DAD.0000000000000933DOI Listing
January 2019

Myelodysplastic syndrome from theoretical review to clinical application view.

Oncol Rev 2018 Jul 7;12(2):397. Epub 2018 Dec 7.

Medical Oncology, Department of Medical Oncology, Faculty of Medicine, Zagazig University, Egypt.

Myelodysplastic syndromes (MDS), called ineffective hematopoiesis is indicated by bone marrow failure and tendency to acute myeloid leukemia transformation. Since the disease is more common in elderly with non- hematology co-morbidities, the research for less toxic and curative novel agents is essential. More than 12 years without new Food and Drug Administration approved drugs in MDS management through the whole course, only 5 drugs. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4081/oncol.2018.397DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6291758PMC
July 2018
1 Read

Pancytopenia of Unknown Cause in Adult Patients Admitted to a Tertiary Hospital in Ethiopia: Case series.

Ethiop J Health Sci 2018 Jul;28(4):375-382

Department of Internal Medicine, Jimma University, Jimma.

Background: Over the past few years, we have witnessed a dramatic increase in the number of patients presenting with severe pancytopenia to Jimma University Hospital. We now present sociodemographic and clinical characteristics of adult patients admitted with pancytopenia of unknown cause to Jimma University Hospital during the period of March 2015 to June 2016. Complete blood count and other diagnostic tests were done for all patients to uncover underlying causes. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4314/ejhs.v28i4.3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308738PMC
July 2018
1 Read

Peripheral blood immunophenotyping in a large cohort of patients with Shwachman-Diamond syndrome.

Pediatr Blood Cancer 2019 Jan 2:e27597. Epub 2019 Jan 2.

Cystic Fibrosis Center, Azienda Ospedaliero Universitaria Ospedali Riuniti, Ancona, Italy.

Shwachman-Diamond syndrome (SDS) is one of the more common inherited bone marrow failure syndromes, characterized by neutropenia, occasional thrombocytopenia, and anemia. Bone marrow evaluation reveals an increased number of monocytes and mature B cells along with decreased granulocytes. However, little is known about the subpopulations of peripheral blood cells, and few previous publications have been based on a small number of patients. Read More

View Article

Download full-text PDF

Source
http://doi.wiley.com/10.1002/pbc.27597
Publisher Site
http://dx.doi.org/10.1002/pbc.27597DOI Listing
January 2019
4 Reads

Posterior reversible encephalopathy syndrome (PRES) and infection: a systematic review of the literature.

Neurol Sci 2019 Jan 2. Epub 2019 Jan 2.

Department of Biomedical Sciences and Morphological and Functional Imaging, University of Messina, Policlinico "G. Martino" Via Consolare Valeria 1, 98100, Messina, Italy.

Posterior reversible encephalopathy syndrome (PRES) is an encephalopathy characterized by a rapid onset of symptoms including headache, seizures, confusion, blurred vision, and nausea associated with a typical magnetic resonance imaging appearance of reversible subcortical vasogenic edema prominent and not exclusive of parieto-occipital lobes. Vasogenic edema is caused by a blood-brain barrier leak induced by endothelial damage or a severe arterial hypertension exceeding the limits of cerebral blood flow autoregulation. Although the exact pathophysiological mechanism is still unclear, frequent conditions that may induce PRES include severe hypertension, eclampsia/pre-eclampsia, acute kidney diseases and failure, immunosuppressive therapy, solid organ, or bone marrow transplantation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10072-018-3651-4DOI Listing
January 2019
1 Read

Clinical features of dyskeratosis congenita in mainland China: case reports and literature review.

Int J Hematol 2019 Jan 3. Epub 2019 Jan 3.

Department of Pediatrics, Tongji Hospital, Tongji University School of Medicine, Xincun Road 389, Shanghai, 200065, People's Republic of China.

Dyskeratosis congenita (DC) is a rare-inherited bone marrow failure syndrome associated with multi-system disorder. To summarize the clinical features, epidemiology, and treatment of DC in mainland China, we retrospectively reviewed the medical records of two patients diagnosed with DC at our hospital and published reports on other DC patients in mainland China. The clinical features of 82 DC patients were summarized. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12185-018-02582-xDOI Listing
January 2019
1.679 Impact Factor

Successful rituximab treatment of granulomatous/lymphocytic interstitial lung disease in common variable immunodeficiency.

Epidemiol Mikrobiol Imunol 2018 ;67(3):142-148

Successful rituximab treatment of granulomatous/lymphocytic interstitial lung disease in common variable immunodeficiency Common variable immunodeficiency, a heterogeneous group of diseases, represents a clinically relevant form of antibody immunodeficiency. Granulomatous/lymphocytic interstitial lung disease is among the most serious complications. A case report is presented of a young women with granulomatous/lymphocytic interstitial lung disease and splenomegaly accompanied by pancytopenia. Read More

View Article

Download full-text PDF

Source
January 2018
1 Read

The clinical features and mortality risk factors of cytomegalovirus infection in patients with systemic lupus erythematosus.

J Microbiol Immunol Infect 2018 Dec 16. Epub 2018 Dec 16.

Division of Allergy, Immunology, & Rheumatology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan; National Yang-Ming University Faculty of Medicine, Taipei, Taiwan. Electronic address:

Background: The clinical features and outcomes of cytomegalovirus (CMV) diseases in patients with systemic lupus erythematosus (SLE) are unknown. We analyzed such data from a medical center in Taiwan.

Methods: We retrospectively reviewed the medical records of patients with SLE who were diagnosed with CMV diseases between 2006 and 2016 in Taipei Veterans General Hospital Taiwan. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jmii.2018.12.002DOI Listing
December 2018
1 Read

Intensive care unit complications and outcomes of adult patients with hemophagocytic lymphohistiocytosis: A retrospective study of 16 cases.

World J Crit Care Med 2018 Nov 30;7(6):73-83. Epub 2018 Nov 30.

Department of Pulmonary, Critical Care and Sleep, Baylor College of Medicine, Houston, TX 77030, United States.

Aim: To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis (HLH) in the intensive care unit (ICU).

Methods: We performed a retrospective observational study of adult patients with the diagnosis of "HLH" admitted to the two academic medical ICUs of Baylor College of Medicine between 01/01/2013 to 06/30/2017. HLH was diagnosed using the HLH-2004 criteria proposed by the Histiocyte Society. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.5492/wjccm.v7.i6.73DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305525PMC
November 2018
2 Reads

Arthroscopic repair of the meniscus: Surgical management and clinical outcomes.

EFORT Open Rev 2018 Nov 8;3(11):584-594. Epub 2018 Nov 8.

Department of Orthopaedic Surgery, 'La Paz' University Hospital, Spain.

From the biomechanical and biological points of view, an arthroscopic meniscal repair (AMR) should always be considered as an option. However, AMR has a higher reoperation rate compared with arthroscopic partial meniscectomy, so it should be carefully indicated.Compared with meniscectomy, AMR outcomes are better and the incidence of osteoarthritis is lower when it is well indicated. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1302/2058-5241.3.170059DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6275851PMC
November 2018

Immunosuppressive TNI -based reconditioning regimens after graft rejection or graft failure in pediatric patients treated with myeloablative allogeneic hematopoietic cell transplantation.

Int J Radiat Oncol Biol Phys 2018 Dec 26. Epub 2018 Dec 26.

Department of Radiation Oncology, University of Tuebingen, Tuebingen, Germany.

Purpose: This retrospective analysis aims to address the efficacy of total nodal irradiation (TNI)-based reconditioning regimens in pediatric patients with graft failure/rejection after allogeneic HCT.

Methods And Materials: 33 pediatric patients with malignant (n=25) and non-malignant diseases (n=8) were treated by a TNI-based reconditioning regimen. All patients received 7 Gray single dose combined with anti-T lymphocyte antibody OKT3 (n=16), antithymocyte globulin (n=24), fludarabine (n=31) and/or thiotepa (n=28) followed by an infusion of peripheral blood stem cells (n=31) or bone marrow transplant (n=2). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijrobp.2018.12.031DOI Listing
December 2018

Clinical and molecular characterization of early T-cell precursor acute lymphoblastic leukemia: Two cases report and literature review.

Medicine (Baltimore) 2018 Dec;97(52):e13856

Department of Hematology, The First Hospital, China Medical University, Shenyang, China.

Rationale: Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a small subtype of T-cell acute lymphoblastic leukemia with a typical immune-phenotype: lack of T-lineage cell surface markers CD1a and CD8 expression, weak or absent CD5 expression, at least one of the myeloid or hematopoietic stem cell markers. It is characterized by high rate of induction failure and the effective unified treatment strategies are still indeterminate. We present 2 ETP-ALL cases. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MD.0000000000013856DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314700PMC
December 2018
1 Read

Acute renal failure in a patient with PR3-ANCA and monoclonal immunoglobulin deposition disease: Case report.

Medicine (Baltimore) 2018 Dec;97(52):e13799

Institute of Health Policy, Management, and Evaluation, University of Toronto, Canada.

Rationale: Acute renal failure developing over a short period of time with evidence of glomerular disease by urine sediment microscopy characterizes the clinical syndrome of rapidly progressive glomerulonephritis (RPGN), of which the most common causes are ANCA-associated glomerulonephritis (GN), immune-complex mediated GN and anti-GBM disease.

Patient Concerns: This was a middle-aged gentleman who presented with acute renal failure and a positive PR3-ANCA.

Diagnosis: Renal biopsy showed an unusual combination of PR3-ANCA GN with focal crescents, monoclonal immunoglobulin deposition disease (MIDD) and mesangial IgA deposition on renal biopsy. Read More

View Article

Download full-text PDF

Source
http://Insights.ovid.com/crossref?an=00005792-201812280-0004
Publisher Site
http://dx.doi.org/10.1097/MD.0000000000013799DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314724PMC
December 2018
3 Reads

Case 28-2018: A Man with Epistaxis, Pain and Erythema of the Forearm, and Pancytopenia.

N Engl J Med 2018 12;379(24):2381-2382

Ohio State University, Columbus, OH

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1056/NEJMc1813964DOI Listing
December 2018

Fate alteration of bone marrow-derived macrophages ameliorates kidney fibrosis in murine model of unilateral ureteral obstruction.

Nephrol Dial Transplant 2018 Dec 24. Epub 2018 Dec 24.

Shanxi Medical University, Taiyuan, Shanxi, People's Republic of China.

Background: Renal fibrosis is a key pathological feature and final common pathway leading to end-stage kidney failure in many chronic kidney diseases. Myofibroblast is the master player in renal fibrosis. However, myofibroblasts are heterogeneous. Read More

View Article

Download full-text PDF

Source
https://academic.oup.com/ndt/advance-article/doi/10.1093/ndt
Publisher Site
http://dx.doi.org/10.1093/ndt/gfy381DOI Listing
December 2018
4 Reads

[A Case of Suspected Therapy-Related Leukemia after Chemotherapy for Breast Cancer].

Gan To Kagaku Ryoho 2018 Dec;45(12):1775-1777

Dept. of Surgery, Nagato General Hospital.

Therapy-related leukemia(TRL)is a distinctive clinical syndrome that occurs after exposure to chemotherapy or radiotherapy. We report a case of suspected TRLafter chemotherapy in a patient with breast cancer. A 61-year-old woman underwent total mastectomy and sentinel lymph node biopsy(negative)for her breast cancer. Read More

View Article

Download full-text PDF

Source
December 2018
5 Reads

Squamous cell carcinoma of the tongue in a patient with dyskeratosis congenita: a rare entity.

Br J Oral Maxillofac Surg 2018 Dec 23. Epub 2018 Dec 23.

Tata Memorial Hospital, Parel Mumbai, 400012.

Dyskeratosis congenita is a rare genetic disorder that results from progressive failure of the bone marrow. It presents with a classic triad of reticular pigmentations of the skin, dystrophic nails, and oral leukoplakia; patients rarely develop cancers. We report a patient with dyskeratosis congenita who presented with squamous cell carcinoma of the oral tongue. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.bjoms.2018.12.002DOI Listing
December 2018

Hemophagocytic Lymphohistiocytosis Due to Primary HHV-8 Infection in a Liver Transplant Recipient.

Transplant Direct 2018 Dec 27;4(12):e411. Epub 2018 Nov 27.

Division of Infectious Diseases, Icahn School of Medicine at Mount Sinai, New York, NY.

Human herpesvirus-8 (HHV-8) remains best known as an oncogenic virus, but nonneoplastic disease manifestations, such as bone marrow failure or hemophagocytic lymphohistiocytosis (HLH) have gained greater recognition in recent years. In organ transplantation, HHV-8 infection commonly occurs with reactivation of latent virus among recipients from endemic regions of the world or due to transmission from the organ donor. We describe a case of HHV-8-associated HLH in a liver transplant recipient at increased risk for primary infection. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/TXD.0000000000000850DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6283084PMC
December 2018
2 Reads

Pediatric Outcomes in Transplant: PersOnaliSing Immunosuppression To ImproVe Efficacy (POSITIVE Study): The Collaboration and Design of a National Transplant Precision Medicine Program.

Transplant Direct 2018 Dec 27;4(12):e410. Epub 2018 Nov 27.

Division of Cardiology, Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Background: Despite age-related differences in biology, physiology, and behavior, transplant immunosuppression is not tailored by age. This likely contributes to high graft failure and posttransplant complications. We present the aims, design, and methods of the Pediatric Outcomes in Transplant: PersOnaliSing Immunosuppression To ImproVe Efficacy Study aimed at personalizing posttransplant immunosuppression in children and young adults. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/TXD.0000000000000842DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6283088PMC
December 2018

The Incidence Rate, Risk Factors and Clinical Outcome of Acute Kidney Injury in Critical Patients.

Iran J Public Health 2018 Nov;47(11):1717-1724

Dept. of Internal Medicine, School of Medicine, Ardabil University of Medical Sciences, Ardabil, Iran.

Background: Acute kidney injury (AKI) is the most common cause of organ dysfunction in intensive care unit (ICU) patients. There is no consensus definition of AKI in ICU patients. Therefore, we aimed to evaluate the incidence rate, risk factors and clinical outcome of AKI using the RIFLE (Risk, Injury, Failure, Loss of kidney function, and End-stage kidney disease) classification in ICU patients. Read More

View Article

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6294852PMC
November 2018

Severe Metabolic Acidemia in a Patient with Aleukemic Leukemia.

Case Rep Nephrol 2018 18;2018:1019034. Epub 2018 Nov 18.

Program Director of Hematology Oncology Fellowship at St. Joseph's University Medical Center, 703 Main St, Paterson, NJ 07503, USA.

Malignancy associated lactic acidosis is a rare metabolic complication that may accompany various types of malignancies. To date, most cases that have been reported are associated with hematologic malignancies (lymphoma and leukemia). Many theories have been proposed to explain the pathophysiology of lactic acidosis in malignancies. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2018/1019034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276439PMC
November 2018
1 Read

Apoptosis of Platelets Inhibited By Herba Sarcandrae Extract through the Mitochondria Pathway.

Evid Based Complement Alternat Med 2018 18;2018:1956902. Epub 2018 Nov 18.

Department of Hematology, Shanghai Baoshan Hospital of Integrated Traditional Chinese and Western Medicine (Baoshan Branch of Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine), Shanghai 201999, China.

The purpose of the present study is to decode the underlying mechanism of Herba Sarcandrae that indicated antipurpuric effect and to unveil one of its primary components, flavonoids, which play an important role. An immune mediated bone marrow failure (BMF) model in mouse was established by infusion thymus suspension cells after radiation Platelets isolated were prepared from normal mice and BMF mice, respectively. The expressions of PS, P-selectin, PAC-1, Bax, Bad, Bid, and caspase-9 were examined by flow cytometry, and alteration of morphology of platelets under different conditions was observed. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2018/1956902DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276404PMC
November 2018

Lingual liability: macroglossia and dyspnoea as the harbinger of systemic AL (light-chain) cardiac amyloidosis.

BMJ Case Rep 2018 Dec 22;11(1). Epub 2018 Dec 22.

Division of Cardiology, Tripler Army Medical Center, Tripler, Hawaii, USA.

A 58-year-old man presented with a chief complaint of tongue indentations and discomfort. Otolaryngology treated him for oral thrush with counselling to avoid tongue biting. In addition, the patient reported dyspnoea described as a decrease in tolerance of his physical activities. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2018-225923DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307669PMC
December 2018
2 Reads

Contribution of bone marrow-derived cells to in situ engineered tissue capsules in a rat model of chronic kidney disease.

Biomaterials 2018 Dec 15;194:47-56. Epub 2018 Dec 15.

Department of Internal Medicine, Leiden University Medical Center, the Netherlands; Einthoven Laboratory for Vascular and Regenerative Medicine, Leiden University Medical Center, the Netherlands. Electronic address:

Tissue engineered blood vessels (TEBVs) hold great promise for clinical use in patients with end stage renal disease (ESRD) requiring vascular access for hemodialysis. A promising way to make TEBVs is to exploit foreign body response (FBR) of polymeric rods used as templates. However, since the FBR predominantly involves bone-marrow (BM) derived cells and ESRD coincides with impaired function of BM, it is important to assess the generation of TEBVs in conditions of renal failure. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.biomaterials.2018.12.014DOI Listing
December 2018
1 Read

MDS-associated mutations in germline GATA2 mutated patients with hematologic manifestations.

Leuk Res 2019 Jan 4;76:70-75. Epub 2018 Dec 4.

Laboratory of Myeloid Malignancies, Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.

Germline mutation in GATA2 can lead to GATA2 deficiency characterized by a complex multi-system disorder that can present with many manifestations including variable cytopenias, bone marrow failure, myelodysplastic syndrome/acute myeloid leukemia (MDS/AML), and severe immunodeficiency. Penetrance and expressivity within families is often variable. There is a spectrum of bone marrow disease in symptomatic cytopenic patients ranging from hypocellular marrows without overt dysplasia to those with definitive MDS, AML, or chronic myelomonocytic leukemia. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S01452126183048
Publisher Site
http://dx.doi.org/10.1016/j.leukres.2018.11.013DOI Listing
January 2019
5 Reads

Late Mortality after Allogeneic Bone Marrow Transplantation in Childhood for Bone Marrow Failure Syndromes and Severe Aplastic Anemia.

Biol Blood Marrow Transplant 2018 Dec 20. Epub 2018 Dec 20.

Institute for Cancer Outcomes and Survivorship, University of Alabama at Birmingham, Birmingham, Alabama; Division of Hematology and Oncology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama. Electronic address:

Children with bone marrow failure syndromes and severe aplastic anemia (SAA) are treated with allogeneic blood or marrow transplantation (BMT). However, there is a paucity of studies examining late mortality risk after allogeneic BMT performed in childhood for bone marrow failure syndromes and SAA and evaluating how this risk differs between these diseases. We investigated cause-specific late mortality in 2-year survivors of allogeneic BMT for bone marrow failure syndromes and SAA performed before age 22 years between 1974 and 2010 at 2 US transplantation centers. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.bbmt.2018.12.063DOI Listing
December 2018
1 Read

A proof-of-concept clinical trial using mesenchymal stem cells for the treatment of corneal epithelial stem cell deficiency.

Transl Res 2018 Nov 22. Epub 2018 Nov 22.

IOBA (Institute of Applied Ophthalmobiology), University of Valladolid, Valladolid, Spain; CIBER-BBN (Biomedical Research Networking Centre in Bioengineering, Biomaterials, and Nanomedicine), Carlos III National Institute of Health, Valladolid, Spain. Electronic address:

Ocular stem cell transplantation derived from either autologous or allogeneic donor corneoscleral junction is a functional cell therapy to manage extensive and/or severe limbal stem cell deficiencies that lead to corneal epithelial failure. Mesenchymal stem cells have been properly tested in animal models of this ophthalmic pathology, but never in human eyes despite their potential advantages. We conducted a 6- to 12-month proof-of-concept, randomized, and double-masked pilot trial to test whether allogeneic bone marrow-derived mesenchymal stem cell transplantation (MSCT], n = 17) was as safe and as equally efficient as allogeneic cultivated limbal epithelial transplantation (CLET), (n = 11) to improve corneal epithelial damage due to limbal stem cell deficiency. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.trsl.2018.11.003DOI Listing
November 2018