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骨髓增殖性疾病中的局灶性脾病变:与致命结局的关联。

Focal splenic lesions in myeloproliferative disease: association with fatal outcome.

作者信息

Görg Christian, Riera-Knorrenschild Jordi, Görg Konrad, Zugmaier Gerhard

机构信息

Department of Internal Medicine, Philipps University, Baldingerstrasse, 35043 Marburg/Lahn, Germany.

出版信息

Ann Hematol. 2004 Jan;83(1):14-7. doi: 10.1007/s00277-003-0753-7. Epub 2003 Oct 3.

Abstract

BACKGROUND

A nodular tumor of the spleen in patients with myeloproliferative disease (MPD) is a very rare form of splenic involvement. The aim of the study was to describe the clinical data, sonographic patterns, and prognosis of nodular splenic infiltration in patients with MPD.

MATERIALS AND METHODS

During a 20-year period, nodular splenic lesions were found in 10 out of 183 patients with MPD. Retrospectively, splenic size, echomorphology of the lesions, clinical data, sonographic follow-up, and survival were analyzed.

RESULTS

In 9 out of 10 patients the lesions were hyperechoic--in one patient hypoechoic. In 3 patients the lesions were solitary. Seven patients had multiple nodular lesions. Low platelet count was seen in 8 patients; blast crisis was seen in 7 patients. The mean survival time was 2.9 months after detection of the splenic lesions. In one patient, autopsy confirmed the diagnosis of myelosarcoma of the spleen.

CONCLUSION

The appearance of nodular splenic lesions in MPD is associated with blast crisis and a short survival. Definite histologic or cytologic findings associated with splenic nodules in MPD have not been identified yet. Myelosarcoma of the spleen is the most probable diagnosis suggested.

摘要

背景

骨髓增殖性疾病(MPD)患者出现脾脏结节状肿瘤是脾脏受累的一种非常罕见的形式。本研究的目的是描述MPD患者脾脏结节状浸润的临床资料、超声表现及预后。

材料与方法

在20年期间,183例MPD患者中有10例发现脾脏结节性病变。回顾性分析脾脏大小、病变的超声形态、临床资料、超声随访及生存情况。

结果

10例患者中有9例病变为高回声,1例为低回声。3例患者病变为单发,7例有多发性结节性病变。8例患者血小板计数低,7例患者出现原始细胞危象。发现脾脏病变后平均生存时间为2.9个月。1例患者经尸检确诊为脾脏髓肉瘤。

结论

MPD患者脾脏结节状病变的出现与原始细胞危象及生存期短有关。MPD患者脾脏结节相关的确切组织学或细胞学表现尚未明确。脾脏髓肉瘤是最可能的诊断。

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