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[血液系统恶性肿瘤中的脾脏]

[The spleen in hematologic malignancies].

作者信息

Kienle Dirk L

机构信息

Department Innere Medizin, Onkologie/Hämatologie, Kantonsspital Graubünden, Chur.

出版信息

Ther Umsch. 2013 Mar;70(3):163-9. doi: 10.1024/0040-5930/a000385.

Abstract

The spleen represents a major lymphatic and hematologic organ and, as such, is frequently involved in hematologic malignancies. Splenomegaly may constitute the first clinical sign leading to the diagnosis of a hematologic malignancy. Vice versa, the presence, or suspicion of a hematologic malignancy requires investigation of the spleen. In case of splenomegaly of unknown origin, directed history, clinical examination, and laboratory testing including a complete blood count with microscopic investigation of a peripheral blood smear, frequently allow to establish a tentative diagnosis. Whenever possible, further specific testing should be based on a thorough primary evaluation to avoid unnecessary diagnostic procedures. In light of the current diagnostic options, diagnostic splenectomy can usually be avoided to establish definitive diagnosis. Indolent lymphomas (chronic lymphocytic leukaemia, hairy cell leukaemia, splenic marginal zone lymphoma) and myeloproliferative neoplasms (chronic myeloid leukaemia, polycythemia vera, essential thrombocythemia, primary and secondary myelofibrosis) are the most prevalent hematologic malignancies associated with splenomegaly. Therapeutic options are highly differentiated depending on the underlying disease. Apart from very rare exceptions, therapeutic splenectomy can usually be avoided.

摘要

脾脏是主要的淋巴和血液学器官,因此常累及血液系统恶性肿瘤。脾肿大可能是导致血液系统恶性肿瘤诊断的首个临床体征。反之,存在或怀疑血液系统恶性肿瘤时,则需要对脾脏进行检查。对于病因不明的脾肿大,详细的病史、临床检查以及实验室检查(包括全血细胞计数和外周血涂片显微镜检查)通常有助于做出初步诊断。只要有可能,进一步的特异性检查应基于全面的初步评估,以避免不必要的诊断程序。鉴于目前的诊断方法,通常可避免进行诊断性脾切除术来确立明确诊断。惰性淋巴瘤(慢性淋巴细胞白血病、毛细胞白血病、脾边缘区淋巴瘤)和骨髓增殖性肿瘤(慢性髓性白血病、真性红细胞增多症、原发性血小板增多症、原发性和继发性骨髓纤维化)是与脾肿大相关的最常见血液系统恶性肿瘤。治疗方案因基础疾病而异。除极少数例外情况外,通常可避免进行治疗性脾切除术。

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