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非热带性“特发性脾肿大”:对1969年描述的10例患者的随访研究

Non-tropical 'idiopathic splenomegaly': a follow-up study of ten patients described in 1969.

作者信息

Dacie J V, Galton D A, Gordon-Smith E C, Harrison C V

出版信息

Br J Haematol. 1978 Feb;38(2):185-93. doi: 10.1111/j.1365-2141.1978.tb01035.x.

DOI:10.1111/j.1365-2141.1978.tb01035.x
PMID:638068
Abstract

The later history is described of four of 10 patients who were reported in 1969 as suffering from non-tropical 'idiopathic splenomegaly'. Two of the four patients developed malignant lymphomas 6 years and 2 years, respectively, after splenectomy but the two other patients have lived for 17 and 15 years, respectively, without developing any signs of a malignant tumour. Thus, four of the original 10 patients have developed malignant's lymphomas. The histology of the patients' spleens has been reviewed in the light of their clinical history, but no criteria have been found which are of clear prognostic value. However, with hindsight it was possible to recognize some cytological abnormality in the spleens in three out of the four patients who developed malignant lymphomas, and it appears that unless the cell population in both the red and white pulp is strictly normal, the patient is likely to develop a malignant lymphoma. The tumours which subsequently developed showed no special or characteristic features. The title 'idiopathic splenomegaly' is clearly unsatisfactory but the alternative 'chronic lymphocytic lymphoma with hypersplenism', although appropriate for the illness of the patients who developed malignant lymphomas, is inappropriate for those in which the pathological process seems not to be neoplastic.

摘要

本文描述了1969年报告的10例非热带“特发性脾肿大”患者中4例的后续病史。4例患者中,2例分别在脾切除术后6年和2年发生恶性淋巴瘤,而另外2例患者分别存活了17年和15年,未出现任何恶性肿瘤迹象。因此,最初的10例患者中有4例发生了恶性淋巴瘤。根据患者的临床病史对其脾脏组织学进行了回顾,但未发现具有明确预后价值的标准。然而,事后看来,在发生恶性淋巴瘤的4例患者中的3例脾脏中可以识别出一些细胞学异常,而且似乎除非红髓和白髓中的细胞群完全正常,否则患者很可能发生恶性淋巴瘤。随后发生的肿瘤没有显示出特殊或特征性表现。“特发性脾肿大”这个名称显然不尽人意,但替代名称“伴有脾功能亢进的慢性淋巴细胞淋巴瘤”,虽然适用于发生恶性淋巴瘤的患者的疾病,但对于那些病理过程似乎不是肿瘤性的患者并不适用。

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引用本文的文献

1
An inherited disorder with splenomegaly, cytopenias, and vision loss.一种伴有脾肿大、血细胞减少和视力丧失的遗传性疾病。
Am J Med Genet A. 2012 Mar;158A(3):475-81. doi: 10.1002/ajmg.a.34437. Epub 2012 Feb 3.
2
Splenectomy in patients with undiagnosed splenomegaly.未确诊脾肿大患者的脾切除术。
Postgrad Med J. 1994 Apr;70(822):288-91. doi: 10.1136/pgmj.70.822.288.
3
Hereditary hepatosplenomegaly.遗传性肝脾肿大
West J Med. 1980 Jan;132(1):70-4.
4
Splenectomy for malignant lymphomas.恶性淋巴瘤的脾切除术。
World J Surg. 1985 Jun;9(3):444-8. doi: 10.1007/BF01655280.
5
Splenectomy for splenomegaly and secondary hypersplenism.因脾肿大和继发性脾功能亢进而行脾切除术。
World J Surg. 1985 Jun;9(3):437-43. doi: 10.1007/BF01655279.