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眼睑出血与非典型淀粉样变性

Eyelid bleeding and atypical amyloidosis.

作者信息

Landa Gennady, Aloni Eyal, Milshtein Asher, Marcovich Arie, Mozes Marta, Pollack Ayala

机构信息

Department of Ophthalmology, Kaplan Medical Center, Rehovot 76100, Israel.

出版信息

Am J Ophthalmol. 2004 Sep;138(3):495-6. doi: 10.1016/j.ajo.2004.03.030.

Abstract

PURPOSE

To describe a case of atypical systemic amyloidosis in a patient who presented with recurrent subcutaneous bleeding of the eyelids and auricles.

DESIGN

Observational case report.

METHODS

A 52-year-old man with chronic cardiac failure, hypertension, weight loss, and impotence presented with recurrent bleeding of the eyelids and auricles, and was investigated for suspected amyloidosis.

RESULTS

Biopsy specimens taken from the bone marrow, salivary gland, abdominal subcutaneous fat, and gastric mucosa, and eyelids were all negative for Congo red. Results of a conjunctival biopsy, which were returned after the patient's death, and cardiac muscle autopsy were positive for Congo red staining, indicating amyloidosis.

CONCLUSION

The findings in this case show that the presentation of amyloidosis may be atypical. Ophthalmologists should consider a diagnosis of amyloidosis in patients with periorbital bleeding, even if biopsies are negative for Congo red. A conjunctival biopsy may be useful in establishing a diagnosis of primary systemic amyloidosis.

摘要

目的

描述一例表现为眼睑和耳廓反复皮下出血的非典型系统性淀粉样变性病例。

设计

观察性病例报告。

方法

一名52岁男性,患有慢性心力衰竭、高血压、体重减轻和阳痿,出现眼睑和耳廓反复出血,因疑似淀粉样变性接受检查。

结果

取自骨髓、唾液腺、腹部皮下脂肪、胃黏膜和眼睑的活检标本刚果红染色均为阴性。患者死后返回的结膜活检结果以及心肌尸检刚果红染色呈阳性,表明为淀粉样变性。

结论

该病例的发现表明淀粉样变性的表现可能是非典型的。眼科医生对于眶周出血的患者应考虑淀粉样变性的诊断,即使活检刚果红染色为阴性。结膜活检可能有助于原发性系统性淀粉样变性的诊断。

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