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3.
Med Clin (Barc) ; 73(5): 198-208, 1979 Sep 15.
Artigo em Espanhol | MEDLINE | ID: mdl-491788

RESUMO

The literature published before October, 1977 on the so-called sea-blue histiocyte syndrome is reviewed. This is a new lipid thesaurismosis, and from a morphological point of view it is characterized by the appearance of large histiocytes in the organs of the reticuloendothelial system with numerous intracytoplasmic granules which take on a typical sea-blue or greenish color with Wright's or Giemsa stain. The exact nature of the accumulated substance has not yet been specifically determined, though it appears to be gluco- and/or phosphosphingolipid, essentially sphingomyelin. The specific biochemical alteration responsible for this chronic deposit has not been established, though a partial sphingomyelinase deficiency has been detected. Sea-blue histiocytes have been observed in two different situations, either as an acquired phenomenon or as a primary condition. Of the latter there have been sporadic cases and cases with a definite familial incidence. The clinical manifestations include enlargement of the liver and spleen, neurological symptoms, cirrhosis of the liver, hemorrhagic diathesis and purpura, chronic pneumopathies, eye or cutaneous disturbances, or no symptoms at all. The disease has a benign clinical course, and the prognosis is less favourable when clinical manifestations appear early in life. These cases have a greater tendency to develop neurological alterations. The final definition of the syndrome must await the clear identification of the accumulated material and the altered enzyme or metabolic pathway.


Assuntos
Histiócitos , Doenças de Niemann-Pick , Diagnóstico Diferencial , Histiócitos/enzimologia , Histiócitos/patologia , Humanos , Doenças de Niemann-Pick/enzimologia , Doenças de Niemann-Pick/etiologia , Doenças de Niemann-Pick/patologia
5.
Med Clin (Barc) ; 72(4): 158-65, 1979 Feb 25.
Artigo em Espanhol | MEDLINE | ID: mdl-431180

RESUMO

Alcohol causes different hematologic alterations on each of the three bone marrow cellular series. Its effect on the red series leads to the appearance of megaloblastic disturbances, erythroblastic vacuolization, iron metabolism abnormalities, and hemolytic syndromes. Megaloblastic disturbances may arise as a consequence of folic acid or vitamin B12 deficiency or of a direct toxic effect of ethanol on the erythroblasts. Iron metabolism alterations include reversible sideroblastic anemia, and hemosiderosis. The three hemolytic syndromes related to the consumption of ethanol are: acanthocytosis, stomatocytosis, and Zieve's syndrome. Alcohol induces leukopenia and functional deffects of the leukocytes; these facts explain the frequent susceptibility of chronic alcoholics to infection. Ethanol may act upon the megakaryocytic series to produce reversible thrombopenia and various alterations in platelet function. Thus alcohol exerts toxic effects on bone marrow, which interfere with the proliferation, maturation, release and survival of the three cellular series, either directly or by means of complex mechanisms related to the metabolism of folic acid, vitamin B12, pyridoxine, or iron. Alcoholism should therefore be considered as a possible cause whenever an obscure hematological condition comes under scrutiny.


Assuntos
Alcoolismo/sangue , Doenças Hematológicas/induzido quimicamente , Alcoolismo/complicações , Doenças da Medula Óssea , Eritrócitos/efeitos dos fármacos , Etanol/efeitos adversos , Ácido Fólico/metabolismo , Humanos , Absorção Intestinal , Ferro/metabolismo , Leucócitos/metabolismo , Megacariócitos/metabolismo , Piridoxina/metabolismo , Vitamina B 12/metabolismo
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