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[特发性充血性脾肿大——班替氏病]

[Idiopathic congestive splenomegaly--Banti's disease].

作者信息

Antunović P, Jesić R, Jovanović V

机构信息

Institute of Haematology, Clinical Centre of Serbia, Belgrade.

出版信息

Srp Arh Celok Lek. 1996 Mar-Apr;124(3-4):103-5.

PMID:9102816
Abstract

Banti's disease is a condition where congestive splenomegaly can be observed in the absence of intrahepatic or extrahepatic obstruction. The diagnosis is established by splenectomy, but it is necessary to exclude liver diseases or portal vein obstruction before surgery. The advanced stage of Banti's disease may be complicated by upper gastrointestinal haemorrhages; so splenectomy has both diagnostical and therapeutical benefits. Primary lesions of the small splenic arterioles are one of the offered explanations. A patient with no intrahepatic or extrahepatic obstruction, is described. We also excluded diseases which might be complicated by splenomegaly, part of them after splenectomy. Cytogenetics showed normal female pattern. The histologic examination revealed no liver disease, but the spleen was congestive with reduced lymphoid tissue. Immunohistologically, the reduced spleen white-pulp nodules were composed of polyclonal B-cells and T-cells in a normal distribution, discarding indolent lymphoprolipherative disorder. Over one year after splenectomy the patient had no trouble. In conclusion, we believe that Banti's disease is a condition clearly separated from Banti's syndrome. We also believe that splenectomy is the treatment of choice.

摘要

班替氏病是一种在无肝内或肝外梗阻情况下可观察到充血性脾肿大的病症。诊断通过脾切除术确立,但术前有必要排除肝脏疾病或门静脉梗阻。班替氏病晚期可能并发上消化道出血;因此脾切除术兼具诊断和治疗益处。脾小动脉的原发性病变是提出的解释之一。描述了一名无肝内或肝外梗阻的患者。我们还排除了可能并发脾肿大的疾病,其中部分疾病在脾切除术后得以排除。细胞遗传学显示为正常女性模式。组织学检查未发现肝脏疾病,但脾脏充血,淋巴组织减少。免疫组织学检查显示,脾脏白髓小结减少,由多克隆B细胞和T细胞组成,分布正常,排除了惰性淋巴细胞增殖性疾病。脾切除术后一年多,患者无任何问题。总之,我们认为班替氏病是一种与班替氏综合征明显不同的病症。我们还认为脾切除术是首选治疗方法。

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