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Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder in which the accumulation and growth of circulating myeloid progenitors in the spleen lead to pathologic enlargement of the organ with resulting mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Medical therapy and splenic irradiation may be of benefit in certain patients, yet many may still require splenectomy to palliate their symptoms. Although there is no clear survival advantage to splenectomy in MMM, the procedure can result in substantial palliation of symptoms. However, the surgical procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern. The management of splenomegaly and the role of splenectomy in MMM are discussed in this review. 相似文献
125.
G I Ingram 《Haemophilia》2000,6(6):719-722
Abdominal surgery became routinely possible over a hundred years ago, after the introduction of general anaesthesia and sterile procedures. Abdominal surgery for haemophiliacs had to wait another 60 or 70 years for adequate control of haemostasis. This paper traces its gradual achievement from the 1920s to the 1970s through a series of reports of appendectomies, gastric and intestinal operations, gall bladder operations and splenectomies in patients with haemophilia of varying degrees of severity. A short-lived flurry of interest in splenectomy as a proposed treatment for haemophilia is also mentioned. 相似文献
126.
为研究脾切除术、脾动脉结扎术对肝硬化门脉高压症 (PHT)大鼠血浆肿瘤坏死因子 (TNF α)的影响 ,研究PHT时TNF α与一氧化氮合酶 (NOS)间的关系 ,建成PHT大鼠模型 ,用放射免疫分析法动态检测TNF α ,根据吸光度的大小测定血浆NOS活性 ,并观察术后 4周内各组的病死率。发现 :PHT大鼠血浆TNF α质量浓度较正常对照组明显增高 (P <0 .0 1 ) ;PHT脾切除组术后 3d、7d、1 4d血浆TNF α质量浓度与实验对照组比较有显著性差异 (P <0 .0 1 ) ;脾动脉结扎组TNF α的质量浓度虽比实验对照组升高 ,但无显著性差异。实验对照组血浆NOS活性明显高于正常对照组 (P <0 .0 1 ) ,且与TNF α显著正相关 (P <0 .0 1 ,r =0 .946) ;脾动脉结扎组NOS活性显著低于实验对照组及脾切除组 (P <0 .0 1 )。PHT时血浆TNF α的质量浓度显著增高 ,提示 :TNF α可能在PHT形成中起重要作用。与脾动脉结扎组相比较 ,脾切除组血浆TNF α的质量浓度显著增高 ,血浆NOS活性以及病死率亦显著增高 ,提示 :脾动脉结扎术后脾脏仍可发挥一定作用 ,PHT时应保留脾脏。 相似文献
127.
采用间接免疫荧光法测定门脉性肝硬变病人脾切除前后外周血T淋巴细胞亚群的变化,同时测定血清免疫球蛋白。结果表明,门脉性肝硬变病人外周血T淋巴细胞亚群及其分布是正常的,血清免疫球蛋白IgG升高。脾切除后外周血总T淋巴细胞和辅助细胞(Th)降低,抑制细胞(Ts)升高,Th/Ts比值下降;血清免疫球蛋白IgM降低。提示机体的细胞免疫和体液免疫都有紊乱。 相似文献
128.
R V Smalley J Connors R L Tuttle S Anderson W Robinson J K Whisnant 《American journal of hematology》1992,41(1):13-18
Twenty patients with previously untreated hairy cell leukemia were randomized to undergo either splenectomy or to receive interferon alfa-N1, a highly purified natural alpha interferon, as primary therapy. A response in the peripheral blood elements to a hemoglobin greater than 110 gm/l, a granulocyte count greater than 1 x 10(9)/l, and a platelet count greater than 100 x 10(9)/l (Catovsky criteria) was noted in all ten patients receiving alpha interferon but in only three of the patients undergoing splenectomy (P = less than .01). Median time to response was longer in the ten interferon patients (153 days) than in the three splenectomy responders (20 days). Median time to treatment failure was significantly greater in the alpha interferon patients (greater than 18 months) than in the splenectomy patients (less than 1 month). Survival was no different since patients relapsing following splenectomy subsequently responded to alpha interferon. A significant decrease in leukemic bone marrow infiltration was observed in seven of ten patients receiving alpha interferon and in none of the patients undergoing splenectomy. Side effects, primarily infections, were more frequent in patients receiving interferon. Alpha interferon is preferable to splenectomy as initial treatment for hairy cell leukemia. 相似文献
129.
Abstract: Up to 30% of patients with idiopathic thrombocytopenic purpura (ITP) are refractory to standard therapy with corticosteroids and splenectomy. The treatment of refractory ITP is barely effective, but promising results of interferon therapy were reported recently. In a prospective study 21 refractory ITP patients were treated with interferon alpha 2B at 3 million Units three times a week for a period of 4 weeks. Ten out of 21 patients had a response, of whom 2 had a complete response of 3.5 and 7 months duration, after which both relapsed. One other patient retreated after relapse has a complete response of 24 + months. The characteristics of the responding patients were not different from the non-responding patients. Adverse effects were minimal. Short treatment with interferon in refractory ITP patients may be justified and the results reported here challenge us to elucidate the mechanism of action of interferon. 相似文献
130.
目的:通过门脉高压症(PHT)脾次全切除术后细胞免疫和体液免疫的变化,初步评价脾次全切除术在门脉高压症中的作用。方法:建立肝硬化PHT大鼠疾病模型。实验分5组,每组10只。正常对照组(NCG),实验对照组(ECG),全脾切除组(TSG),保留正常睥脏50%的脾部分切除组(R50%、NSG), 保留正常脾脏大小的睥部分切除组(RNSG)。术后4周对各组进行血常规、免疫球蛋白、T淋巴细胞亚群的测定及病理学检查。结果:术后4周R50%NSG组与RNSG组与NCG组和ECG组血常规、免疫球蛋白和T淋巴细胞亚群水平差异无统计学意义。ECG光镜下见假小叶,再生的肝细胞结节肝细胞索排列紊乱等肝硬化表现脾包膜增厚,脾实质充血严重。脾内纤维严重增生。R50%NSG和RNSG光镜下见肝纤维化程度减轻,脾包膜变薄,脾实质轻度充血。结论:PHT残余脾脏具有一定的生理功能;保留部分脾脏的脾部分切除术在一定程度上能够维持机体的体液免疫和细胞免疫。 相似文献