In aplastic anemia, immune destruction of hematopoietic cells results in bone marrow failure. Type 1 cytokines, especially IFN-gamma, have been implicated in the pathophysiology of T-cell-mediated, Fas-mediated stem cell apoptosis of hematopoietic cells. Here, we show that the transcription factor T-bet (T-box expressed in T cells) is increased in T cells from patients with aplastic anemia. Pat...

Aplastic anemia may be inherited or acquired. The distinction between these lies not in the age of the patient, but in the clinical and laboratory diagnoses. Adult hematologists must consider adult presentations of the inherited disorders, in order to avoid incorrect management of their patients. Physicians for adult patients must also realize that children with inherited disorders now survive ...

The inherited bone marrow failure syndromes are traditionally considered to be pediatric disorders, but in fact, many of the patients now are diagnosed as adults, and many diagnosed as children now live to reach adulthood. The most common of these rare disorders include Fanconi anemia, dyskeratosis congenita, Shwachman-Diamond syndrome and amegakaryocytic thrombocytopenia, which often develop a...

Parvovirus B19 (PV B19) is a nonenveloped single-stranded DNA virus that causes wide variety of diseases ranging from benign childhood infection such as slapped-cheek syndrome to life threatening hydrous fetalis in fetuses or aplastic anemic crises patients with hemolytic anemia. In immunocompromised hosts including organ transplant recipients, PV has incorporated into red blood cell progenitor...

human parvovirus (hpv) b19 induced aplastic crisis in a family leading to the diagnosis of hereditary spherocytosis (hs) is a very rare condition being barely reported in the literature. we herein report a 4-year-old girl, her brother, and their mother who all presented with progressive pallor and jaundice after a febrile illness. the hpv b19 was diagnosed using polymerase chain reaction (pcr) ...

There is general agreement that in children and adolescents with an HLA-identical or syngeneic sibling, bone-marrow transplantation (BMT) should be performed without delay. More controversial are young-to-middle-aged adults with an HLA-identical sibling. Because of comparable survival rates, some centers advocate BMT; others advocate immunosuppression as primary treatment. BMT cures severe apla...

Mean serum zinc and copper levels were measured in pediatric patients with idiopathic aplastic anemia. Zinc levels were significantly lower in patients compared to controls and correlated with severity of disease. Serum copper levels were significantly higher in patients. There was no correlation with severity of disease. Cu/Zn ratio also correlated with severity of disease.

GATA-2 deficiency was recently described as common cause of overlapping syndromes of immunodeficiency, lymphedema, familiar myelodysplastic syndrome or acute myeloid leukemia. The aim of our study was to analyze bone marrow and peripheral blood samples of children with myelodysplastic syndrome or aplastic anemia to define prevalence of the GATA2 mutation and to assess whether mutations in GATA-...

stances, future investigations will help clarify the division of labor among NK-cell subsets. Although further studies are necessary to more fully understand NK-cell responses to target cell recognition, the work by Fauriat et al sheds light on the functional heterogeneity of human CD56bright and CD56dim NK-cell subsets. This study also advances our knowledge on how multiple NK cell–activating ...