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In situ mapping identifies distinct vascular niches for myelopoiesis.

In contrast to nearly all other tissues, the anatomy of cell differentiation in the bone marrow remains unknown. This is owing to a lack of strategies for examining myelopoiesis-the differentiation of myeloid progenitors into a large variety of innate immune cells-in situ in the bone marrow. Such strategies are required to understand differentiation and lineage-commitment decisions, and to define how spatial organizing cues inform tissue function. Here we develop approaches for imaging myelopoiesis in mice, and generate atlases showing the differentiation of granulocytes, monocytes and dendrit…

Placental Pathology in Down Syndrome-Associated Transient Abnormal Myelopoiesis.

Transient abnormal myelopoiesis is a hematopoietic disorder that occurs in up to 10% of neonates with Down syndrome. It is characterized by leukocytosis and the presence of circulating blast cells harboring truncating GATA1 mutations with variable multiorgan system involvement. Placental involvement of transient abnormal myelopoiesis is infrequently described. Placental examination and identifying features related to transient abnormal myelopoiesis could be one of the early, if not the only, means of diagnosis of this condition in affected stillbirths, premature infants, and a subset of asympt…

Transient Abnormal Myelopoiesis: A Varied Spectrum of Clinical Presentation.

Transient myeloproliferative disorder (TMD) is a condition seen almost exclusively in newborns with Down syndrome (DS). It can have a spectrum of clinical presentation ranging from being asymptomatic with incidental detection to a stormy course and fatal outcome. We describe three cases of TMD having different clinical presentation, course, complications and outcome. All but one had Down's phenotype; one of them had a severe disease with tumor lysis syndrome and died of liver failure, while the other one had pericardial effusion and cardiac failure but survived. The third patient had a very be

Transient abnormal myelopoiesis in neonates: GATA get the diagnosis.

Transient abnormal myelopoiesis occurs exclusively in patients with Down syndrome (constitutional trisomy 21), manifests in the neonatal period, and is characterized by circulating megakaryoblasts with varied degrees of multisystem organ involvement. In most cases, this process resolves spontaneously by 3 to 6 months of age, but for some, the disease can be fatal. Affected patients are particularly prone to develop acute megakaryoblastic leukemia in early childhood. Somatic GATA1 mutations are believed to be pivotal in the development of transient abnormal myelopoiesis and have proven to be a …

Transient abnormal myelopoiesis of infancy associated with trisomy 21.

PURPOSE: A unique myelodysplastic syndrome referred to as transient abnormal myelopoiesis (TAM) has been reported to occur primarily in infants with Down's syndrome (DS) or other abnormalities of chromosome 21. This disorder raises basic questions regarding the pathogenesis of leukemia, yet its natural history is poorly documented and derives from small series and isolated case reports. PATIENTS AND METHODS: To better characterize TAM, we accumulated data on 35 cases identified through a questionnaire mailed to pediatric oncologists in the United States. These cases, pooled with two that we r…

Trisomy 21, transposition of the great arteries and abnormal myelopoiesis.

Down syndrome is a well-recognised genetic condition associated with several comorbidities. Although CHD is common in Down syndrome, transposition of the great arteries is exceptionally rare. We describe a neonate with Down syndrome who presented with transient abnormal myelopoiesis and transposition of the great arteries. Down syndrome may accelerate pulmonary hypertension in transposition of the great arteries and is associated with poor outcomes.

Myelopoiesis in immunologically classified subgroups of childhood acute lymphocytic leukemia.

Soft agar culture studies of 43 immunologically characterized patients with childhood acute lymphocytic leukemias (ALL) are presented. The immunologic subsets studied include "null"-cell, pre-B-cell, and T-cell leukemias. Abnormal myelopoiesis, including high peripheral blood and low marrow, colony-forming cell numbers, low colony-stimulating activity, and normal maturation of colony-forming cells in vitro was noted in each group as previously described for immunologically uncharacterized ALL. We conclude that immunologic subsets of childhood lymphoblastic leukemia cause similar abnormalities …

Mielopoiesis временно динамичен и регулируется образом жизни, чтобы изменить множественный склероз.

Моноциты и нейтрофилы из миэлоидной линии способствуют множеству склероза (MS), но динамика миэлопоисиса во время MS неясна. Здесь мы обнаружим связь между стилем жизни, миэлопоисисом и нейроинфламмацией. В мышах с аутоиммунным энцефаломиэлитом (RR-EAE) миэлопоисис в бедренной части, позвоночнике и селезенке поднимается до возникновения болезни и во время ремиссии, перед пиками клинической инвалидности и нейро-воспаления. В прогрессивном EAE (P-EAE) позвоночник mielopoiesis устойчиво поднимается во всех болезнях, в то время как бедра и селезенка mielopoies поднимаются до того, как они упадут п

Совпадение в аннотации оригинала
Monocytes and neutrophils from the myeloid lineage contribute to multiple sclerosis (MS), but the dynamics of myelopoiesis during MS are unclear. Here we uncover a disease stage-specific relationship between lifestyle, myelopoiesis and neuroinflammation. In mice with relapsing-remitting experimental autoimmune encephalomyelitis (RR-EAE), myelopoiesis in the …

Impact of age and sex on myelopoiesis and inflammation during myocardial infarction.

Of all the different risk factors known to cause cardiovascular disease (CVD), age and sex are considered to play a crucial role. Aging foll … ht the importance of age and sex as crucial risk factors by discussing their role during MI-induced myelopoiesis while emphasizing the current status of myeloid cell sub-sets. We further put forth the need for designing and executing age and sex interaction studies aimed to determine the appropriate age and sex to develop personalized therapeutic strategies po …

Transient Abnormal Myelopoiesis: An Abnormal Course and the Efficacy of Delayed Treatment.

Transient abnormal myelopoiesis (TAM) is observed in a few neonates with Down syndrome. While a large proportion undergo complete remission without any treatment, some of them can develop myeloid leukemia of Down syndrome (ML-DS) in the future. Without proper treatment, mortality can be high. Here we have described an interesting and difficult-to-treat case of a neonatal with Down syndrome who presented with anemia, thrombocytopenia, and 75% blasts. We came across multiple challenges in treatment due to severe pneumonia.

Recent advances in the understanding of transient abnormal myelopoiesis in Down syndrome.

Neonates with Down syndrome (DS) have a propensity to develop the unique myeloproliferative disorder, transient abnormal myelopoiesis (TAM). TAM usually resolves spontaneously in ≤3 months, but approximately 10% of patients with TAM die from hepatic or multi-organ failure. After remission, 20% of patients with TAM develop acute myeloid leukemia associated with Down syndrome (ML-DS). Blasts in both TAM and ML-DS have trisomy 21 and GATA binding protein 1 (GATA1) mutations. Recent studies have shown that infants with DS and no clinical signs of TAM or increases in peripheral blood blasts can hav…

Myelopoiesis in the omentum of normal mice and during abdominal inflammatory processes.

Coelomic cavities are relatively isolated from the systemic circulation of blood cells. Resident cell populations have a proper phenotype and kinetics, maintaining their steady-state populations and their responsiveness to local inflammatory reactions, in which the number and quality of coelomic cells can be greatly increased and modified. We have addressed the question of whether the increase in cell infiltrate in the inflamed abdominal cavity is sustained by the proliferation of myeloid cells in the omentum, and if so what are the characteristics of the progenitor cells involved and how the

Impaired myelopoiesis in congenital neutropenia: insights into clonal and malignant hematopoiesis.

A common feature of both congenital and acquired forms of bone marrow failure is an increased risk of developing acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). Indeed, the development of MDS or AML is now the major cause of mortality in patients with congenital neutropenia. Thus, there is a pressing clinical need to develop better strategies to prevent, diagnose early, and treat MDS/AML in patients with congenital neutropenia and other bone marrow failure syndromes. Here, we discuss recent data characterizing clonal hematopoiesis and progression to myeloid malignancy in congen

Exchange Transfusion and Cytarabine for Transient Abnormal Myelopoiesis in Hydrops Fetalis.

Most cases of transient abnormal myelopoiesis (TAM) in neonates with Down syndrome (DS) resolve spontaneously; however, DS-TAM neonates with hydrops fetalis (HF) show poor clinical outcomes. We report three infants with DS-TAM and HF who were treated with exchange transfusion (ET) followed by low-dose cytarabine (LD-CA). All of them survived without developing liver failure, acute leukemia, or other serious adverse events. Our results suggest that this combination treatment with ET and LD-CA would be safe, tolerable and effective as an novel approach for DS-TAM patients with HF.

Prenatal diagnosis of transient abnormal myelopoiesis in a Down syndrome fetus.

We report a case of transient abnormal myelopoiesis in a Down syndrome fetus diagnosed at 28(+3) weeks of gestation that rapidly progressed to intrauterine death 10 days later. Fetal hepatosplenomegaly with cerebral ventriculomegaly, although not specific, may be a suggestive finding of Down syndrome with transient abnormal myelopoiesis. Prompt fetal blood sampling for liver function test and chromosomal analysis are mandatory for early detection and management.

Dysregulated myelopoiesis and enhanced glutaminase-dependent neutrophil survival in preclinical obesity.

Obesity is increasingly recognized as a state of chronic low-grade inflammation associated with altered immune cell function, yet the mechan … eliance on glutamine metabolism for survival under hypoxia. These findings demonstrate dysregulated myelopoiesis and altered neutrophil behavior in preclinical obesity, providing mechanistic insight into the early immune consequences of metabolic dysfunction.

Systemic inflammation impairs myelopoiesis and interferon type I responses in humans.

Systemic inflammatory conditions are classically characterized by an acute hyperinflammatory phase, followed by a late immunosuppressive pha … terized by diminished expression of type I interferon (IFN)-responsive genes in monocytes, impaired myelopoiesis and a pronounced attenuation of the immune response on a secondary LPS challenge 1 week after the first. The infMono gene program and impaired myelopoiesis were also detected in patient cohorts with bacterial sepsis and coronavirus disease. IFNβ tre …