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Myelofibrosis with myeloid metaplasia (MMM) is currently classified as a classic (ie, BCR-ABL-negative) myeloproliferative disorder characterized by anemia, multiorgan extramedullary hematopoiesis, constitutional symptoms, and premature death from either leukemic transformation or other disease complications. Stem cell transplantation can be curative, but many patients either are not appropriate candidates or do not choose to accept the significant risks associated with transplantation. Current pharmacologic therapy has been beneficial mainly in terms of palliating disease-associated cytopenias, constitutional symptoms, splenomegaly, and other organ damage from excess myeloproliferation. Novel treatment strategies are under investigation, including targeted inhibition of JAK2V617F, the activating tyrosine kinase point mutation present in about half of patients with MMM. In this article, we review both the old and new pharmacologic options for MMM.  相似文献   
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M Balázs  G Vadász  I Koncz  E Simon 《Orvosi hetilap》1991,132(39):2143-2146
15 patients with congestive gastropathy were reported including clinical and pathological characteristics of the disease. Every patient had alcoholic liver cirrhosis and portal hypertension. 6 patient's stomach was resected while in 2 further cases the disease was found at autopsy. In additional 7 cases the characteristic microvascular changes were observed in endoscopic biopsy specimens from the gastric mucosa. The authors presume that this disease has an acute and a chronic stage. In the acute stage dilated capillaries are present under the surface, not related to the inflammation of gastric mucosa. This phenomenon was described in the literature. In the chronic stage there are dilated and tortuous vessels in the submucosal layer surrounded by collagenous connective tissue. The authors suppose that the thick and fibrotic submucosal layer causes microcirculatory disturbances in the gastric mucosa. The impaired microcirculation may cause extensive ulcers with profuse and sometimes lethal bleeding.  相似文献   
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Stuttering and tetanic syndrome   总被引:1,自引:0,他引:1  
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The hepatic circulation of patients with hilar carcinoma and icterus was studied by isotope technique. A marked alternation in blood flow was observed, that is that the ratio of the circulation of the hepatic artery and the portal vein became balanced. By elimination of the icterus, the hepatic circulation normalized. This allowed the conclusion that the change in blood flow must have rather been due to the mechanical icterus and the increased pressure of the bile duct than to the tumorous infiltration and therefore the earliest possible elimination of the icterus is urgently indicated.  相似文献   
49.
This paper studies all cases affected by 5 parotiditis from January to September 1986 (1767 cases) and 1987 (124 cases), as well as all patients with rubella reported in the same period in 1986 (584 cases) and 1987 (5 cases) in the province of Pinar del Río. The rise in the incidence of the two diseases with an annual basis per 100,000 population during 1986 in the different municipalities is emphasized. The overtly downward trend of these two diseases in 1987 after the administration of the triple viral vaccine--93% for mumps and 99.15% for rubella--is discussed. It was found that these two diseases affected more often school children (5-14 years). It was found that these two diseases are not frequent under the first year of life (76.4 and 120.1 cases per 100,000 population respectively). The percentage of vaccine effectiveness was documented--97.4% for parotiditis and 100% for rubella.  相似文献   
50.
The different types of instruments used for monitoring pharmaceutical dissolution testing are presented. Their features and the need for automation are critically discussed. The advantages of flow injection analysis in this respect are illustrated by a variety of examples clearly showing its adaptability to the different problems posed by other automatic and non-automatic alternatives.  相似文献   
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