Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disorder thatmanifests with hemolytic anemia, bone marrow failure, and thrombosis. One of the earliest descriptions of PNH was by Dr Paul Strübing, who in 1882 described a 29-year-old man who presented with fatigue, abdominal pain, and severe nocturnal paroxysms of hemoglobinuria. Strübing deduced that the hemolysiswa...

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell disorder in which the blood cells demonstrate aberrant interactions with serum complement. In part, this is due to the absence of the complement regulatory protein, decay accelerating factor (DAF). A small number of patients with PNH have gone on to develop acute nonlymphocytic leukemia, which is thought to arise f...

Paroxysmal nocturnal hemoglobinuria (PNH) arises in the setting of bone marrow injury. Thus, management decisions must take into account whether symptoms are a consequence of the underlying marrow failure or of the expansion of the clone of the PIG-A mutant hematopoietic cells. The primary clinical manifestations of PNH are intravascular hemolysis and thrombophilia. Currently available options ...

Paroxysmal nocturnal hemoglobinuria is a clonal hematopoietic stem cell disease that manifests with intravascular hemolysis, bone marrow failure, thrombosis, and smooth muscle dystonias. The disease can arise de novo or in the setting of acquired aplastic anemia. All PNH patients to date have been shown to harbor PIG-A mutations; the product of this gene is required for the synthesis of glycosy...

pAROXYSMAL NOCTURNAL HEMOGLOBINURIA is a rare hemolytic disease characterized by intravascular hemolysis and excretion of hemoglobin in the urine during sleep. Crosby,’ in an extensive review of the literature, found reports of 123 cases of this condition and gave credit to StrUbing for first recognizing it as an entity in 1882. It was not until another description was published by Marchiafava2...

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male pati...

The clinical hallmark of paroxysmal nocturnal hemoglobinuria (PNH) is episodic hemoglobinuria, and it was this feature that captured the attention of European physicians in the latter half of the 19th century, resulting in careful observational studies that established PNH as an entity distinct from paroxysmal cold hemoglobinuria and march hemoglobinuria. Curiosity about the etiology of the noc...

Analysis by Gas Chromatography and Gas Chromatography/Mass Spectrometry of the essential oils obtained from leaves of Echinodorus grandiflorus ("Chapéu de couro") from two different populations (Big Leaves and Small Leaves), collected monthly between September 1998 and December 1999 revealed 17 components. Phytol was the major constituent for both populations. The main sesquiterpene representat...

Atmospheric conditions fundamentally influence the timing, intensity, energetics, and geography of avian migration. While radar is typically used to infer the influence of weather on the magnitude and spatiotemporal patterns of nocturnal bird migration, monitoring the flight calls produced by many bird species during nocturnal migration represents an alternative methodology and provides informa...

In 1953, Hara provided new combinations for many sectional and species names when he combined Jussiaea L. with Ludwigia L., and at the time, Ludwigiasect.Oligospermum (Micheli) H.Hara was the correct name for one well-defined section. However, subsequent changes to/clarifications of the botanical code have necessitated a change for that name in that now an autonym is treated as having priority ...