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[血管内淋巴瘤(血管营养性大细胞淋巴瘤),腿部疼痛性肿胀伴治疗抵抗性发热的一种罕见鉴别诊断]

[Intravascular lymphomatosis (angiotrophic large-cell lymphoma), a rare differential diagnosis in painful swelling of the leg with treatment-resistant fever].

作者信息

Reiner M, Marschall F M, Beck P, Horny H P

机构信息

Medizinische Klinik, Kreiskrankenhaus Waiblingen.

出版信息

Dtsch Med Wochenschr. 1998 Oct 30;123(44):1303-7. doi: 10.1055/s-2007-1024171.

Abstract

HISTORY AND CLINICAL FINDINGS

For one month a 69-year-old woman had been suffering from increasingly painful and reddened swelling of both legs and induration of the skin of the left thigh, about 15 cm in diameter. In addition she had fever and rigors. Antibiotic treatment, begun because erysipelas was suspected, was ineffective and she was hospitalized. Although obese she was in a good general condition with no obvious abnormalities on routine lung, heart and neurological examination. No lymph nodes were palpated.

INVESTIGATIONS

Laboratory tests showed increased inflammatory parameters, marked rise in lactate dehydrogenase and a normochromic anaemia, hemoglobin of 9.5 g/dl. Doppler sonography excluded deep vein thrombosis, but marked chronic venous insufficiency was revealed. Extensive tests, including soft-tissue sonography, radiology and skin biopsy failed to establish a diagnosis. TREATMENT AND CAUSE: Antibiotic treatment was resumed because a diagnosis of only partly treated erysipelas was made. But several changes to a variety of antibiotics remained ineffective. Collagen disease was excluded by the biochemical and biopsy results. As the skin changes in both legs increased a skin and muscle biopsy from the indurated area of the left thigh was done: it showed intravascular large-cell lymphomatosis. A search for a paraneoplastic process revealed an adenocarcinoma of the ascending colon that was successfully resected. The patient died before the planned chemotherapy could be initiated.

CONCLUSION

In case of treatment-resistant fever associated with painful swelling of the leg and skin changes of unknown etiology a deep skin biopsy should be an early consideration to exclude such causes as lymphoma, including the very rare intravascular clear-cell lymphomatosis.

摘要

病史及临床检查结果

一名69岁女性,双腿疼痛、肿胀且发红加剧,左大腿皮肤硬结,直径约15厘米,持续了一个月。此外,她还伴有发热和寒战。因怀疑患有丹毒而开始使用抗生素治疗,但无效,随后她住院治疗。尽管肥胖,但她的一般状况良好,常规肺部、心脏和神经系统检查未发现明显异常。未触及淋巴结。

检查

实验室检查显示炎症指标升高,乳酸脱氢酶显著升高,伴有正色素性贫血,血红蛋白为9.5g/dl。多普勒超声检查排除了深静脉血栓形成,但显示有明显的慢性静脉功能不全。包括软组织超声、放射学检查和皮肤活检在内的多项检查均未能确诊。

治疗及病因

由于诊断为丹毒治疗不彻底,故重新开始抗生素治疗。但更换多种抗生素后仍无效。生化检查和活检结果排除了胶原病。随着双腿皮肤病变加重,对左大腿硬结部位进行了皮肤和肌肉活检:结果显示为血管内大细胞淋巴瘤。进一步检查副肿瘤性病变发现升结肠癌,已成功切除。患者在计划的化疗开始前死亡。

结论

对于伴有腿部疼痛性肿胀和不明病因皮肤改变且治疗抵抗性发热的病例,应尽早考虑进行深部皮肤活检,以排除淋巴瘤等病因,包括极为罕见的血管内透明细胞淋巴瘤。

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