SYNOPSIS In intermittent acute porphyria there are no specific alterations in the ultrastructure of the liver. In contrast, the constant characteristic of porphyria cutanea tarda is siderosis of the hepatocytes and Kupffer cells. Therefore, between the two types of hepatic porphyria there are no similar ultrastructural lesions. It is still doubtful whether the presence of hepatosiderosis in porphyria cutanea tarda is connected with the hepatopathy or is secondary to an alteration in the porphyrinic metabolism. The role played by lysosomes is discussed.
SYNOPSISThe morphology of red cells damaged by 2-aminoethylisothiouronium bromide (AET) has been compared to that of PNH cells by transmission and scanning electron microscopy. The main features of the effect of AET as demonstrated by the scanning electron microscope were spherical and deformed cells, with surface craters and relatively large pits. The transmission electron microscope revealed loss of surface structure, an unusual amount of irregularly shaped electrondense material and fine pits in the cell membranes. The paroxysmal nocturnal haemoglobinuria cells showed, by transmission electron microscope, electron-dense material scattered over the cell and fine pitting; by scanning electron microscope, larger craters were seen and swellings protruded from the cell surface. Although the AET changes were not identical with the natural abnormality of paroxysmal nocturnal haemoglobinuria, the two types of cells were sufficiently similar to each other to support the proposition that AET cells have many of the characteristics of paroxysmal nocturnal haemoglobinuria.
A case of von Recklinghausen's neurofibromatosis, hospitalized for progressive anemia, secondary to chronic hemorrhage from the digestive tract, is reported. The imponent involvement of the skin (innumerable nodules of different size and volume), skeleton (marked cifoskoliosis giving a monstruous aspect to the patient) and visceral organs (multiple localizations of the digestive and urinary tract) is illustrated. A functional study of endocrine system suggested by a marked somatic hypoevolutism and right testis retention failed to show any pathological change.
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