We report the case of a 37-year old man presenting with a left ventricular cardiac thrombus in the setting of subclinical paroxysmal nocturnal hemoglobinura (PNH) developing two years after immunosuppressive therapy for thymoma-associated aplastic anemia. The literature regarding the interplay between autoimmunity and immunosuppression, aplastic anemia, thymoma and the emergence of PNH is revie...

Paroxysmal nocturnal haemoglobinuria (PNH) is characterized by nocturnal haemoglobinuria, haemolytic anaemia, and thrombosis [ 1]. Most patients have anatomical and functional renal abnormalities, although renal function deterioration often remains unrecognized. The pathogenesis could be related to iron deposition or to repeated vascular thrombosis. The present case illustrates that renal trans...

Paroxysmal nocturnal hemoglobinuria (PNH) and the stable phase of chronic myelogenous leukemia (CML) are the two hematological conditions known to be associated with low levels of leukocyte alkaline phosphatase (LAP) activity in peripheral blood polymorphonuclear cells (PMN). LAP mRNA levels were determined in PMN from PNH and CML patients by RNA blotting analysis. In CML, LAP mRNA is undetecta...

We report here on the first case of a child with bilateral periventricular nodular heterotopia (PNH) and Williams syndrome. Fluorescent in situ hybridization (FISH) analyses demonstrated a deletion of the elastin gene in the Williams syndrome critical region (WSCR). Further mapping by loss of heterozygosity analysis both by microsatellite marker and SNP profiling demonstrated a 1.5 Mb deletion ...

In patients with paroxysmal nocturnal hemoglobinuria (PNH) the RBCs, neutrophils (PMNsh monocytes, and platelets derived from the abnormal clone are deficient in the complement-regulatory protein decay-accelerating factor (DAF). RBC acetyicholinesterase (AChE) and leukocyte alkaline phosphatase (LAP) activities are also characteristically low. DAF, AChE, and LAP are known to be anchored within ...

Paroxysmal nocturnal hemoglobinuria (PNH) is clinically characterized by intravascular hemolysis, hemoglobinuria, iron deficiency anemia, and venous thrombosis. Pathophysiologically the disease has now been generally accepted as an acquired defect of phosphatidylinositol glycan (PIG)-anchored molecules on the cell surface of bone marrow-derived cells. This defect is functionally characterized b...

BACKGROUND Reducing neonatal mortality is a global priority, and improvements in postnatal health (PNH) practices in India are needed to do so. Intimate partner violence (IPV) may be associated with PNH practices, but little research has assessed this relationship. METHODS A cross-sectional analysis of data from a representative household sample of mothers of neonates 0-11 months old in Bihar...

The effect of five different reactions which activate complement (antibody activation, reduction in ionic strength, acidification, cobra venom factor (CoF) activation, and inulin activation) upon normal and PNH cells was investigated, using normal serum and serum devoid of the fourth component of complement (C4) activity from patients with hereditary angioneurotic edema (HANE) as a source of co...

N ABNORMALITY of the red cells of paroxysmal nocturnal hemoglobinuria ( PNH ) is responsible for their short life span in the circulation anu their lysis in vitro by certain factors present in normal plasma or serum. The hemolytic system in vitro is characteristically more active when the serum is made somewhat acid, about pH 6.8. The addition of thrombin to the serum is also capable of increas...

Lymphocyte function-associated antigen 3 (LFA-3) is a widely distributed cell surface glycoprotein that binds to the T lymphocyte CD2 surface glycoprotein. This interaction mediates CTL-target cell conjugate formation and adhesion of thymocytes to thymic epithelial cells. CD2 is also the E rosette receptor of T lymphocytes and mediates rosetting with autologous E by binding to LFA-3. We describ...