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慢性淋巴细胞白血病患者的肉芽肿性耶氏肺孢子菌肺炎:文献综述及发病机制假说

Granulomatous Pneumocystis jiroveci Pneumonia in a patient with chronic lymphocytic leukemia: a literature review and hypothesis on pathogenesis.

作者信息

Otahbachi Mohammed, Nugent Kenneth, Buscemi Dolores

机构信息

Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas, USA.

出版信息

Am J Med Sci. 2007 Feb;333(2):131-5. doi: 10.1097/00000441-200702000-00014.

DOI:10.1097/00000441-200702000-00014
PMID:17301596
Abstract

BACKGROUND

Pneumocystis jiroveci pneumonia occurs frequently in patients with immunodeficiency syndromes, especially AIDS. Approximately 5% of AIDS patients have atypical granulomatous histology.

CASE REPORT/METHODS: A 75-year-old woman with chronic lymphocytic leukemia was treated with alemtuzumab (campath-1H) 3 times weekly for 12 weeks. After completion of therapy she presented with dyspnea, hypoxemia, and bilateral infiltrates. Bronchoscopy with biopsy revealed Pneumocystis organisms with granulomatous history. She responded well to trimethoprim-sulfamethoxazole.

RESULTS/LITERATURE REVIEW: Our literature review identified 19 patients without AIDS who had granulomatous Pneumocystis infection. These patients often had nodular infiltrates on x-rays and negative bronchoalveolar lavage study findings. Most patients required open lung biopsies. Histologic specimens frequently revealed necrosis. These patients responded well to therapy.

CONCLUSION

The limited information available from these studies suggests that these patients have immune reconstitution-like syndrome related to either increasing numbers of CD4+ lymphocytes following therapeutic suppression or impaired modulation of CD4+ function. This unusual clinical presentation may delay diagnosis and effective therapy.

摘要

背景

耶氏肺孢子菌肺炎常见于免疫缺陷综合征患者,尤其是艾滋病患者。约5%的艾滋病患者有非典型肉芽肿组织学表现。

病例报告/方法:一名75岁慢性淋巴细胞白血病女性患者接受阿仑单抗(campath-1H)治疗,每周3次,共12周。治疗结束后,她出现呼吸困难、低氧血症和双侧肺部浸润。支气管镜检查及活检发现有肉芽肿形成的耶氏肺孢子菌。她对复方新诺明反应良好。

结果/文献综述:我们的文献综述确定了19例无艾滋病的肉芽肿性肺孢子菌感染患者。这些患者胸部X线检查常显示结节状浸润,支气管肺泡灌洗研究结果为阴性。大多数患者需要进行开胸肺活检。组织学标本常显示坏死。这些患者对治疗反应良好。

结论

这些研究提供的有限信息表明,这些患者患有免疫重建样综合征,可能与治疗性抑制后CD4+淋巴细胞数量增加或CD4+功能调节受损有关。这种不寻常的临床表现可能会延迟诊断和有效治疗。

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