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Expert Rev Hematol 2019 Mar 18. Epub 2019 Mar 18.

d Case Western Reserve University , Case Comprehensive Cancer Center , Cleveland , OH , USA .

Introduction: Molecular and biologic heterogeneity in diffuse large B-cell lymphoma (DLBCL) has resulted in a broad range of clinical outcomes. While standard frontline chemoimmunotherapy cures majority of patients with DLBCL, treatment failure in certain DLBCL subsets remains high. Prognosis in these patients is dismal. Read More

Annu Rev Cancer Biol 2019 Mar 3;3:457-478. Epub 2018 Dec 3.

Department of Radiation Oncology and Center for DNA Damage and Repair, Dana-Farber Cancer Institute, Boston, Massachusetts 02215, USA;

Fanconi anemia (FA) is a complex genetic disorder characterized by bone marrow failure (BMF), congenital defects, inability to repair DNA interstrand cross-links (ICLs), and cancer predisposition. FA presents two seemingly opposite characteristics: () massive cell death of the hematopoietic stem and progenitor cell (HSPC) compartment due to extensive genomic instability, leading to BMF, and () uncontrolled cell proliferation leading to FA-associated malignancies. The canonical function of the FA proteins is to collaborate with several other DNA repair proteins to eliminate clastogenic (chromosome-breaking) effects of DNA ICLs. Read More

EJIFCC 2019 Mar 1;30(1):67-81. Epub 2019 Mar 1.

Department of Hematology & Immunohematology, School of Biomedical and Laboratory Sciences, College of Medicine and Health Sciences, University of Gondar, Ethiopia.

Diamond Blackfan Anaemia (DBA) is a sporadic inherited anemia with broad spectrum of anomalies that are presented soon after delivery. It is inherited mainly in autosomal dominant inheritance manner and caused by mutations and deletions in either large or small ribosomal protein genes that results in an imbalance between the biosynthesis of rRNA and ribosomal proteins, eventually the activation and stabilization of p53. Diagnosing DBA is usually problematic due to a partial phenotype and its wide inconsistency in its clinical expression; however, molecular studies have identified a heterozygous mutated gene in up to 50% of the DBA cases and corticosteroid drugs are the backbone treatment options of DBA. Read More

Lancet Haematol 2019 Mar 13. Epub 2019 Mar 13.

Hematologic Malignancies and Bone Marrow Transplantation Program, Department of Oncology, Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Johns Hopkins University School of Medicine, Baltimore, MD, USA; Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Background: Although severe haemoglobinopathies can be cured with allogeneic blood or bone marrow transplantation, availability of matched donors and toxic effects can be problematic. We previously found that non-myeloablative haploidentical related bone marrow transplantation with post-transplantation cyclophosphamide expanded the donor pool while limiting graft-versus-host disease (GVHD). However, graft failure-albeit with full host haemopoietic recovery-occurred in 50% of patients. Read More

Clin Med (Lond) 2019 Mar;19(2):129-130

Imperial College Healthcare NHS Trust, London, UK.

An 18-year-old female patient presented to the emergency department with non-specific neurological and gastrointestinal symptoms and was found to be pancytopenic. Her vitamin B level was low with a normal mean corpuscular volume and her full blood count 2 months previously had been within normal range. She reported heavy use of nitrous oxide over the previous 2 weeks and other investigations revealed no cause for her pancytopenia. Read More

J Pediatr Hematol Oncol 2019 Mar 12. Epub 2019 Mar 12.

Department of Anatomy and Cell Biology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan.

A 2-year-old Asian girl presented to our facility for the evaluation of thrombocytopenia. She was treated with intravenous immunoglobulin under the impression of immune thrombocytopenia. However, her body temperature spiked and progressive pancytopenia, hepatosplenomegaly, abnormal liver function, coagulopathy, and pulmonary infiltration developed. Read More

Ann Hematol 2019 Mar 13. Epub 2019 Mar 13.

CEINGE Biotecnologie Avanzate, Naples, Italy.

In this study, we aimed at disclosing the main features of paroxysmal nocturnal hemoglobinuria (PNH) clones, their association with presentation syndromes, and their changes during follow-up. A large-scale, cooperative collection (583 clones from 529 patients) of flow cytometric and clinical data was entered into a national repository. Reason for testing guidelines were provided to the 41 participating laboratories, which followed the 2010 technical recommendations for PNH testing by Borowitz. Read More

Case Rep Hematol 2019 5;2019:8928623. Epub 2019 Feb 5.

Department of Clinical Hematology, Troyes General Hospital, 10000 Troyes, France.

Paroxysmal nocturnal hemoglobinurea (PNH) is a rare disorder of complement regulation due to somatic mutation of PIGA (phosphatidylinositol glycan anchor) gene. We herewith report a case who developed a symptomatic PNH long after an allogenic marrow transplant. Some reasonable arguments concerning the origin of PNH clone have been discussed. Read More

Curr Opin Pulm Med 2019 Mar 6. Epub 2019 Mar 6.

SC Pneumologia - Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

Purpose Of Review: Pulmonary alveolar proteinosis (PAP) is characterized by the massive accumulation of lipoproteinaceous material within alveoli, which results in progressive respiratory failure. The abnormalities in surfactant clearance are caused by defective pulmonary macrophages, whose terminal differentiation is GM-CSF-dependent. In hereditary PAP, the rupture of GM-CSF signaling is because of mutations in the GM-CSF receptor genes. Read More

Curr Pharm Des 2019 Mar 12. Epub 2019 Mar 12.

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

Abnormal activation of the immune system plays an important role in the pathogenesis of aplastic anemia (AA). Various immune cells and cytokines constitute a complex immune network, leading to bone marrow failure. The known pathogenesis is an increase of the myeloid dendritic cell (mDC)/ plasmacytoid dendritic cell (pDC) ratio, which causes the ratio of T helper (Th)1/Th2 to be skewed in favor of Th1 and eventually leads to an abnormal activation of cytotoxic T lymphocyte (CTL). Read More

Curr Opin Obstet Gynecol 2019 Feb 21. Epub 2019 Feb 21.

Fundación IVI.

Purpose Of Review: We review the techniques recently tested in both animal models and humans to provide a state-of-the-art on adult stem cell ovarian transplant to achieve ovarian rejuvenation in patients with diminished ovarian reserves.

Recent Findings: As the firsts reports of spontaneous pregnancies achieved after bone marrow transplantation in oncologic women with primary ovarian insufficiency, increasing evidence supports the regenerative effects of stem cell-based therapies in the ovarian niche. Adult stem cells from several origins promote follicular development, increase ovarian local vascularization, increase follicle and stromal cell proliferation and reduce cell apoptosis and follicular atresia, although they do not modify embryo quality. Read More

SAGE Open Med Case Rep 2019 1;7:2050313X19834425. Epub 2019 Mar 1.

Section of Infectious Diseases, Department of Medicine, School of Medicine, Boston University, Boston, MA, USA.

Fournier's gangrene is classically associated with diabetes mellitus and alcohol use disorder. While it is associated with chemotherapy, there are few case reports of Fournier's gangrene as the initial presentation of acute myelogenous leukemia. A 38-year-old male presented with progressive scrotal swelling and hematochezia. Read More

BMJ Case Rep 2019 Mar 7;12(3). Epub 2019 Mar 7.

Cardiovascular Research Unit, Sheffield Teaching Hospitals and University of Sheffield, Sheffield, UK.

We present a case of constrictive pericarditis with concomitant blood and bone marrow appearances of chronic myelomonocytic leukaemia (CMML). Despite surgical treatment with pericardiectomy, the patient deteriorated into multiorgan failure. Pericardial histology disclosed a typical inflammatory picture with no evidence of monocytic or malignant infiltrate. Read More

Cell Stem Cell 2019 Mar;24(3):405-418.e7

Laboratory of Regenerative Hematopoiesis, Swiss Institute for Experimental Cancer Research (ISREC) & Institute of Bioengineering (IBI), School of Life Sciences, Ecole Polytechnique Fédérale de Lausanne (EPFL), Lausanne, Switzerland; Service and Central Laboratory of Hematology, Departments of Oncology and of Laboratories, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland. Electronic address:

It has been recently shown that increased oxidative phosphorylation, as reflected by increased mitochondrial activity, together with impairment of the mitochondrial stress response, can severely compromise hematopoietic stem cell (HSC) regeneration. Here we show that the NAD-boosting agent nicotinamide riboside (NR) reduces mitochondrial activity within HSCs through increased mitochondrial clearance, leading to increased asymmetric HSC divisions. NR dietary supplementation results in a significantly enlarged pool of progenitors, without concurrent HSC exhaustion, improves survival by 80%, and accelerates blood recovery after murine lethal irradiation and limiting-HSC transplantation. Read More

J Surg Res 2019 Mar 4;239:191-200. Epub 2019 Mar 4.

Department of Surgery, Campus Charité Mitte and Campus Virchow-Klinikum, Charité - Universitaetsmedizin, Berlin, Germany.

Background: Mesenchymal stem cells (MSCs) have been suggested to augment liver regeneration after surgically and pharmacologically induced liver failure. To further investigate this we processed human bone marrow-derived MSC according to good manufacturing practice (GMP) and tested those cells for their modulatory capacities of metabolic alterations and liver regeneration after partial hepatectomy in BALB/c nude mice.

Methods: Human MSCs were obtained by bone marrow aspiration of healthy donors as in a previously described GMP process. Read More

Clin Transplant 2019 Mar 7:e13518. Epub 2019 Mar 7.

Department of Infectious Diseases, The University of Queensland School of Medicine, Oschsner Clinical School, Oschner Medical Center, 1514 Jefferson Highway, New Orleans, LA, 70121.

These updated guidelines from the Infectious Diseases Community of Practice of the American Society of Transplantation review the diagnosis, prevention and management of HHV-6A, HHV-6B, HHV-7, and HHV-8 in the pre- and post-transplant period. The majority of HHV-6 (A and B) and HHV-7 infections in transplant recipients are asymptomatic; symptomatic disease is reported infrequently across organs. Routine screening for HHV-6 and 7 DNAemia is not recommended in asymptomatic patients, nor are prophylaxis or preemptive therapy. Read More

Br J Dermatol 2019 Mar 6. Epub 2019 Mar 6.

Division of Blood and Marrow Transplantation, Department of Pediatrics, Medical School, University of Minnesota, Minneapolis, MN, USA.

Background: Recessive dystrophic epidermolysis bullosa (RDEB) is a severe systemic genodermatosis lacking therapies beyond supportive care for its extensive, life-limiting manifestations.

Objectives: We report the safety and preliminary responses of 10 RDEB patients to bone marrow transplant with post-transplant cyclophosphamide (PTCy BMT) following reduced-intensity conditioning with subsequent infusions of immunomodulatory donor-derived mesenchymal stromal cells (median follow-up of 16 months).

Methods: BMT toxicities, donor blood and skin engraftment, skin biopsies, medical photography, and dynamic assessments of RDEB disease activity by providers and parents were obtained at intervals from pre- to 1 year post-BMT. Read More

Front Psychol 2019 20;10:329. Epub 2019 Feb 20.

Department of Health Sciences, San Paolo Hospital, University of Milan, Milan, Italy.

The main objective of this study is to gain further insights on how chronic myeloid leukemia (CML) patients involved in an interventional clinical trial with the purpose of reaching treatment free remission (TFR) phase, perceived and experienced TFR failure. TFR failure was defined for the individual patient as either not being eligible for drug discontinuation or as having relapse in the TFR phase with reintroduction of nilotinib treatment. Using a qualitative approach, out of 25 patients with CML who experienced TFR failure 14 were interviewed. Read More

Nat Commun 2019 Mar 6;10(1):1072. Epub 2019 Mar 6.

Department of Signal Transduction, Research Institute for Microbial Diseases, Osaka University, 3-1 Yamada-oka, Osaka, Suita,, 565-0871, Japan.

The balance between self-renewal and differentiation of hematopoietic stem and progenitor cells (HSPCs) maintains hematopoietic homeostasis, failure of which can lead to hematopoietic disorder. HSPC fate is controlled by signals from the bone marrow niche resulting in alteration of the stem cell transcription network. Regnase-1, a member of the CCCH zinc finger protein family possessing RNAse activity, mediates post-transcriptional regulatory activity through degradation of target mRNAs. Read More

Rinsho Ketsueki 2019 ;60(2):124-129

Department of Hematology/Oncology, Wakayama Medical University.

A 63-year-old woman was admitted to our hospital to receive a fourth course of modified rituximab-ESHAP chemotherapy for relapsed primary breast diffuse large B-cell lymphoma. She developed hemophagocytic lymphohistiocytosis (HLH) 20 days after admission. Polymerase chain reaction (PCR) detected cytomegalovirus (CMV) DNA in her peripheral blood; therefore, she was diagnosed with CMV-associated HLH and consequently treated with foscarnet (FCN). Read More

Rinsho Ketsueki 2019 ;60(2):106-111

Department of Hematology, Kyoto City Hospital.

A 64-year-old man presented to the emergency department with sudden-onset upper abdominal pain and pain in the left chest area. His platelet count was 121.7×10/µl. Read More

Ann Pharmacother 2019 Mar 6:1060028019836775. Epub 2019 Mar 6.

1 Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Objective: To review the literature for the treatment of classical and variant hairy cell leukemia (HCL, HCLv), evaluating efficacy, safety, and supportive care involved in the use of purine analogues (PAs), interferon, BRAF inhibitors, monoclonal antibodies, Bruton's tyrosine kinase inhibitors, and new immunotoxin, moxetumomab pasudotox-tdfk (MPT). An electronic literature search of PubMed (January 1958 to January 2019) was conducted in PubMed using the MESH terms hairy cell leukemia, hairy cell leukemia variant, cladribine, pentostatin, rituximab, interferon, vemurafenib, moxetumomab pasudotox.

Study Selection And Data Extraction: Studies written in the English language were considered for this article. Read More

Sci Rep 2019 Mar 5;9(1):3611. Epub 2019 Mar 5.

Servicio de Hematología y Oncología Médica, Hospital Universitario Morales Meseguer, Centro Regional de Hemodonación, Universidad de Murcia, IMIB-Arrixaca, Murcia, Spain.

Paroxysmal Nocturnal Hemoglobinuria (PNH) is a clonal disease of blood cells caused by the lack of glycosyl phosphatidyl inositol anchored proteins bound to the cell membrane. In consequence, erythrocytes lead to intravascular hemolysis upon complement activation, which promotes high risk of thrombosis, intravascular hemolytic anemia, and bone marrow failure in patients. The mechanisms of thrombosis in PNH are still poorly understood. Read More

Hematol Oncol Stem Cell Ther 2019 Mar 2. Epub 2019 Mar 2.

Division of Hematology and Medical Oncology, Knight Cancer Institute, Oregon Health and Science University, Portland, OR, USA.

Allogeneic hematopoietic stem cell transplantation (HSCT) is an integral therapy for patients with hematological malignancies, myelodysplasia, and bone marrow failure. Its use has been increasing over the past decade, as understanding of the treatment and its related toxicities has led to changes in patient selection, conditioning regimens, and post-transplant care. Older (age ≥65 years) patients are often considered unfit for transplantation; however, more recent data suggest that older patients, when selected appropriately, tolerate transplantation well. Read More

Int J Cardiol 2019 Feb 25. Epub 2019 Feb 25.

Department Medicine, Section of Diabetes and Metabolic Diseases, University of Padova, Italy.

Randomized, cross-sectional, and prospective studies have demonstrated that microvascular complications in patients with diabetes are not only the cause of blindness, renal failure and non-traumatic amputations, but also powerful predictors of cardiovascular complications. Beside the metabolic theory, the pathophysiology of diabetic microvascular complications is determined by the interaction among several factors, including epigenetic modifications and the reduced release of progenitor cells by the bone marrow, that contribute simultaneously to damage and impaired vascular protection against hyperglycemia. Identifying and preventing microvascular complications has the significant potential to reduce major adverse cardiovascular events. Read More

BMC Infect Dis 2019 Mar 4;19(1):220. Epub 2019 Mar 4.

Department of Infectious Diseases, Hospital Giselda Trigueiro, Universidade Federal do Rio Grande do Norte, 110 Cônego Monte Street, Natal, Rio Grande do Norte, RN 59037-170, Brazil.

Background: Cryptococcosis is a common opportunistic infection in patients infected by Human Immunodeficiency Virus (HIV) and is the second leading cause of mortality in Acquired Immunodeficiency Syndrome (AIDS) patients worldwide. The most frequent presentation of cryptococcal infection is subacute meningitis, especially in patients with a CD4+ T Lymphocytes count below 100 cells/μL. However, in severely immunosuppressed individuals Cryptococcus neoformans can infect virtually any human organ, including the bone marrow, which is a rare presentation of cryptococcosis. Read More

Virus Res 2019 Mar 1;265:20-29. Epub 2019 Mar 1.

Department of Veterinary Diagnostic and Production Animal Medicine, College of Veterinary Medicine, Iowa State University, Ames, USA. Electronic address:

Red bone marrow is physiologically unique in that it is both the major hematopoietic organ and a primary lymphoid organ. Porcine reproductive and respiratory syndrome virus (PRRSV) affects normal bone marrow functions. The cumulative effect of PRRSV infection is acute bone marrow failure, i. Read More

Cardiovasc Res 2019 Mar 4. Epub 2019 Mar 4.

Department of Surgery, Yong Loo Lin School of Medicine, National University of Singapore, Singapore.

Aims: The Toll-like receptor 7 (TLR7) is an intracellular innate immune receptor activated by nucleic acids shed from dying cells leading to activation of the innate immune system. Since innate immune system activation is involved in the response to myocardial infarction (MI), this study aims to identify if TLR7 is involved in post-MI ischemic injury and adverse remodeling after MI.

Methods And Results: TLR7 involvement in MI was investigated in human tissue from patients with ischemic heart failure, as well as in a mouse model of permanent left anterior descending artery occlusion in C57BL/6J wild type and TLR7 deficient (TLR7-/-) mice. Read More

F1000Res 2019 11;8:176. Epub 2019 Feb 11.

Department of Pediatric Oncology, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN, 55404, USA.

Fasting hypoglycemia is a known complication of mercaptopurine (6MP) maintenance therapy for acute lymphoblastic leukemia (ALL). It is associated with high levels of the methylated metabolite 6-methyl-mercaptopurine (6MMP). Symptoms of hypoglycemia include morning tremulousness, nausea and vomiting. Read More

Curr Oncol Rep 2019 Mar 2;21(3):28. Epub 2019 Mar 2.

UCLA Cardio-Oncology Program, Division of Cardiology, Department of Medicine, University of California at Los Angeles, Los Angeles, CA, USA.

Purpose Of Review: Due to advancements in oncologic treatment strategies and techniques, the number of survivors who have undergone hematopoetic stem cell transplant (HCT) continues to increase in the United States; this number is projected to reach 502,000 by the year 2030. There is significant interest within the field of cardio-oncology to identify cardiotoxicity and cardiovascular disease in the HCT population. Epidemiologic studies analyzing both short- and long-term cardiovascular effects, risk stratification modeling, cardioprotective strategies, and expert consensus documents for cardiotoxicity surveillance recommendations are reviewed. Read More

Biol Blood Marrow Transplant 2019 Feb 28. Epub 2019 Feb 28.

Bone Marrow Unit, Humanitas Cancer Center, Rozzano (Mi).

Introduction: The presence of donor-specific anti-HLA antibodies (DSA) is associated with a 10-fold increased risk of graft failure in haploidentical stem cell transplantation (Haplo-SCT). The Consensus Guidelines from the European Society for Blood and Marrow Transplantation (EBMT) set a Mean Fluorescence Intensity (MFI) > 1000 as a cut-off for DSA positivity. In the absence of an alternative donor, it is recommended that patients undergo desensitization therapy, especially with high DSA levels (>5,000 MFI). Read More

Mol Cancer Ther 2019 Mar 1. Epub 2019 Mar 1.

Department of Oncology and Hematology, BMT with section Pneumology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Epidermal growth factor receptor (EGFR) ectodomain variants mediating primary resistance or secondary treatment failure in cancer patients treated with cetuximab or panitumumab support the need for more resistance-preventive or personalized ways of targeting this essential pathway. Here, we tested the hypothesis that the EGFR nanobody 7D12 fused to an IgG1 Fc portion (7D12-hcAb) would overcome EGFR ectodomain-mediated resistance because it targets a very small binding epitope within domain III of EGFR. Indeed, we found that 7D12-hcAb bound and inhibited all tested cell lines expressing common resistance-mediating EGFR ectodomain variants. Read More

Biologicals 2019 Mar 26;58:64-72. Epub 2019 Feb 26.

Department of Molecular Medicine, School of Advanced Technologies in Medicine, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:

This study investigated the correlation between the hepatic level of miR-122 and the extent of liver tissue regeneration in CCl induced liver injury mice model following transplantation of menstrual blood-(MenSCs) and bone marrow-derived stem cells (BMSCs). Hepatic miR-122 levels were significantly up-regulated following administration of CCl (P < 0.01). Read More

Haematologica 2019 Feb 28. Epub 2019 Feb 28.

Nihon University School of Medicine.

Hemophagocytic lymphohistiocytosis is a life-threatening systemic hyperinflammatory disorder with primary and secondary forms. Primary hemophagocytic lymphohistiocytosis is associated with inherited defects in various genes that affect the immunological cytolytic pathway. Secondary hemophagocytic lymphohistiocytosis is not inherited, but complicates various medical conditions including infection, autoinflammatory/autoimmune disease, and malignant disease. Read More

J Clin Apher 2019 Feb 28. Epub 2019 Feb 28.

Department of Hematology, Hospital General Universitario de Alicante (institution where the work was performed), Alicante, Spain.

Introduction: Autologous bone marrow transplantation is a component of the malignant hemopathy therapy. The preferred mobilization and collection method is apheresis. The aim of this study is to compare three protocols analyzing the effect of plerixafor, higher dose of G-CSF and large volume leukapheresis (LVL). Read More

Genes (Basel) 2019 Feb 22;10(2). Epub 2019 Feb 22.

Laboratory of Molecular Gerontology, National Institute on Aging, NIH, NIH Biomedical Research Center, Baltimore, MD 21224, USA.

Fanconi anemia (FA) is a hereditary chromosomal instability disorder often displaying congenital abnormalities and characterized by a predisposition to progressive bone marrow failure (BMF) and cancer. Over the last 25 years since the discovery of the first linkage of genetic mutations to FA, its molecular genetic landscape has expanded tremendously as it became apparent that FA is a disease characterized by a defect in a specific DNA repair pathway responsible for the correction of covalent cross-links between the two complementary strands of the DNA double helix. This pathway has become increasingly complex, with the discovery of now over 20 FA-linked genes implicated in interstrand cross-link (ICL) repair. Read More

Cancers (Basel) 2019 Feb 22;11(2). Epub 2019 Feb 22.

Department of Oncology, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.

Acute Myelogenous Leukemia (AML) is a malignant disease of the hematopoietic cells, characterized by impaired differentiation and uncontrolled clonal expansion of myeloid progenitors/precursors, resulting in bone marrow failure and impaired normal hematopoiesis. AML comprises a heterogeneous group of malignancies, characterized by a combination of different somatic genetic abnormalities, some of which act as events driving leukemic development. Studies carried out in the last years have shown that AML cells invariably have abnormalities in one or more apoptotic pathways and have identified some components of the apoptotic pathway that can be targeted by specific drugs. Read More

Vet Clin Pathol 2019 Feb 27. Epub 2019 Feb 27.

Department of Veterinary Internal Medicine, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Bunkyo-Ku, Japan.

A 12-year-old castrated male mixed breed dog was presented with anorexia, lethargy, intermittent vomiting, diarrhea, and weight loss. Clinicopathologic and imaging abnormalities included pancytopenia, icterus, and splenomegaly with multiple minute hypoechogenic nodules. Bone marrow (BM) smears revealed 2. Read More

Rev Soc Bras Med Trop 2019 Feb 21;52:e20180233. Epub 2019 Feb 21.

Universidade Potiguar, Natal, RN, Brasil.

Visceral leishmaniasis (VL) in pregnant is considered rare. We present the case of a woman with 24 gestational weeks presenting fever, hepatosplenomegaly, pancytopenia, and inversion of albumin/globulin ratio. Anti-rK39 was positive and amastigotes were visualized on myelogram. Read More

Congenit Anom (Kyoto) 2019 Feb 27. Epub 2019 Feb 27.

Department of Animal Sciences, Faculty of Biological Sciences, Quaid-i-Azam University, Islamabad, Pakistan.

Fanconi anemia (FA) is a recessive disorder that predispose to bone marrow failure and multiple congenital anomalies in affected individuals worldwide. To date, twenty-two FA genes are known to harbor sequence variations in disease phenotype. Among these, mutations in the FANCA gene are associated with 60-70% of FA cases. Read More

J Cell Biochem 2019 Feb 25. Epub 2019 Feb 25.

Center for Molecular Medicine, Maine Medical Center Research Institute, Scarborough, Maine.

Osteoclasts (OCs) are large, multinucleated bone resorbing cells originating from the bone marrow myeloid lineage, and share a common progenitor with macrophages and dendritic cells. Bone marrow cells (BMCs) are a common source for in vitro osteoclastogenesis assays but are a highly heterogeneous mixture of cells. Protocols for in vitro osteoclastogenesis vary considerably thus hindering interpretation and comparison of results between studies. Read More

Bone Marrow Transplant 2019 Feb 25. Epub 2019 Feb 25.

Department of Medicine and Biosystemic Science, Kyushu University, Graduate School of Medical Sciences, Fukuoka, Japan.

Bone Marrow Transplant 2019 Feb 25. Epub 2019 Feb 25.

Shandong University School of Medicine, Jinan, China.

Limited to inadequate stem-cell doses, cord blood transplantation (UCBT) is accompanied by increased graft failure and delayed haematopoietic recovery. The conditioning regimen is critically important for engraftment, and numerous trials have been undertaken comparing the outcomes of IV Bu and TBI, but there are no comparative data for UCBT. We conducted a retrospective multicentre study to analyse the outcomes of IV Bu and TBI in UCBT patients with haematologic malignancies. Read More

Blood 2019 Feb 25. Epub 2019 Feb 25.

National Heart, Lung and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, MD, United States

The pro-inflammatory cytokine interferon-γ (IFNγ) has been implicated in human hematopoietic stem and progenitor cell (HSPC) depletion in immune-mediated bone marrow failure (BMF) syndromes. We show that IFNγ specifically prevents full engagement of thrombopoietin (TPO), a primary positive regulator of HSPC survival, to its receptor (c-MPL) via steric occlusion of the low-affinity binding site, contributing to perturbation of TPO-induced signaling pathways and decreased survival of human HSPCs. Eltrombopag, a synthetic small molecule mimetic of TPO that interacts with c-MPL at a position distinct from the extracellular binding site of TPO, bypasses this inhibition, providing an explanation for its clinical activity in BMF despite already elevated endogenous TPO levels. Read More

Cancer Genet 2019 Feb 9;231-232:36-40. Epub 2019 Jan 9.

Department of Internal Medicine, University of Michigan, 1524 Basic Science Research Building, Ann Arbor, MI 48109, USA; Program in Cellular and Molecular Biology, University of Michigan, Ann Arbor, MI, USA. Electronic address:

Myelodysplastic syndromes (MDS) are a heterogeneous category of myeloid neoplasms that represent the most common class of acquired bone marrow failure syndromes in adults. MDS is typically associated with a hypoproliferative macrocytic anemia, but atypical findings on initial diagnostic evaluations can raise concern for a distinct pathophysiological process and lead to the investigation of alternative etiologies. Here, we report a case of MDS with a concomitant hypoproliferative microcytic and hypochromic anemia that led to the identification of acquired hemoglobin H due to a novel somatic ATRX mutation. Read More

Clin Med Insights Case Rep 2019 15;12:1179547619828704. Epub 2019 Feb 15.

Department of Pathology, University of Iowa Health Care, Iowa City, IA, USA.

Cast Nephropathy/Light chain tubulopathy is usually present in patients with multiple myeloma and is very rare in patients with Waldenstrom Macroglobulinemia. There are very few case reports mentioned in the literature. We present an interesting case of Cast Nephropathy and light chain tubulopathy in an 81-year-old female patient with Waldenstrom Macroglobulinemia who required medical attention for worsening renal failure. Read More

Acta Haematol 2019 Feb 20;141(3):158-163. Epub 2019 Feb 20.

Division of Hematology, Department of Medicine, Jichi Medical University, Shimotsuke,

TAFRO syndrome, a rare systemic inflammatory disease, can lead to multiorgan failure without appropriate treatment. Although thrombocytopenia is frequently seen in patients with TAFRO syndrome, little is known about its pathogenesis. Moreover, while recent studies have reported the presence of an anterior mediastinal mass in some patients, the pathological status of this remains unclear. Read More

Kidney Int 2019 Feb 16. Epub 2019 Feb 16.

Selzman Institute for Kidney Health, Section of Nephrology, Department of Medicine, Baylor College of Medicine, Houston, Texas, USA. Electronic address:

Neointima formation is a major contributor to arteriovenous fistula (AVF) failure. We have previously shown that activation of the Notch signaling pathway contributes to neointima formation by promoting the migration of vascular smooth muscle cells (VSMCs) into the venous anastomosis. In the current study we investigated the mechanisms underlying the dedifferentiation and migration of VSMCs, and in particular the role of bone marrow-derived fibroblast specific protein 1 (FSP-1) cells, another cell type found in models of vascular injury. Read More