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[严重微血管病性溶血性贫血作为不明原发癌综合征的首发表现。多药化疗下血液学快速缓解]

[Severe microangiopathic hemolytic anemia as first manifestation of a CUP syndrome. Rapid hematologic remission under polychemotherapy].

作者信息

Abdel Samie Ahmed, Sandritter Bettina, Theilmann Lorenz

机构信息

Medizinische Klinik II, Städtisches Klinikum Pforzheim, Akademisches Lehrkrankenhaus der Universität Heidelberg, Pforzheim.

出版信息

Med Klin (Munich). 2004 Mar 15;99(3):148-53. doi: 10.1007/s00063-004-1023-8.

DOI:10.1007/s00063-004-1023-8
PMID:15024487
Abstract

BACKGROUND

Microangiopathic hemolytic anemia (MAHA) represents a rare manifestation of malignant tumors. This complication, which is mainly lethal within a few weeks, is most commonly observed in association with mucin-producing adenocarcinomas, especially gastric cancer. The pathogenesis of tumor-associated mechanical hemolysis remains unclear. A standard therapy of this hematologic disturbance is not established.

CASE REPORT

The authors report on a 51-year-old patient, who presented himself in their clinic with severe anemia, thrombopenia and deterioration of his general condition. Extensive investigations revealed signet-ring carcinoma metastases in the whole vertebral column, pelvis and bone marrow. The primary tumor could neither be located endoscopically nor in imaging examinations. Under palliative chemotherapy with cisplatin, epirubicin and 5-fluorouracil continuous infusion, a rapid objective and subjective improvement could be achieved, which was reflected in normalization of the blood picture and regression of the osteoplastic bone metastases indicating partial remission. 9 months later, the patient died because of a new progression of his disease.

CONCLUSION

MAHA can be the first manifestation of malignant tumors. Upon the diagnosis of a MAHA, rapid diagnostics should be done to detect a possible underlying tumor, as effective antineoplastic therapy can induce both tumor and hematologic remission.

摘要

背景

微血管病性溶血性贫血(MAHA)是恶性肿瘤的一种罕见表现。这种并发症主要在几周内致命,最常见于与产生黏液的腺癌相关,尤其是胃癌。肿瘤相关性机械性溶血的发病机制仍不清楚。尚未确立针对这种血液系统紊乱的标准治疗方法。

病例报告

作者报告了一名51岁患者,该患者因严重贫血、血小板减少和全身状况恶化到他们的诊所就诊。广泛检查发现整个脊柱、骨盆和骨髓中有印戒细胞癌转移。无论是通过内镜检查还是影像学检查均未发现原发肿瘤。在接受顺铂、表柔比星和5-氟尿嘧啶持续输注的姑息化疗后,病情迅速出现客观和主观改善,表现为血象恢复正常以及成骨性骨转移灶消退,提示部分缓解。9个月后,患者因疾病新进展死亡。

结论

MAHA可能是恶性肿瘤的首发表现。诊断MAHA后,应迅速进行诊断以检测可能的潜在肿瘤,因为有效的抗肿瘤治疗可诱导肿瘤缓解和血液学缓解。

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