【摘要】 目的探讨隐匿性弥漫性癌肿相关微血管病性溶血性贫血(MAHA)患者的临床特点,有利于癌肿的早期诊断和治疗。方法报道4例隐匿性弥漫性癌肿相关MAHA病例,并综合国内相关文献进行分析。结果近30年间国内报道19例隐匿性弥漫性癌肿相关MAHA患者,包括现在报道4例共23例患者,中位年龄45岁(16～86岁),男8例,女15例,突出的临床表现包括MAHA相关症状、发热、多处骨痛、神经精神异常、皮肤黏膜出血以及上腹痛伴呕血或黑便,发生率分别为78%、48%、48%、35%、35%和26%。实验室检查:Hb中位数52(26～89)g/L,血小板中位数35(5～98)×109/L;12例患者凝血功能检查7例有PT延长等异常,其中4例并发DIC;11例患者骨髓涂片和病理活检发现转移癌细胞。经病理诊断的21例患者中腺癌17例,其中胃癌11例。结论隐匿性弥漫性癌肿继发MAHA患者多为腺癌,骨痛、骨髓涂片和活检以及动态凝血纤溶检查,有助于与血栓性微血管病性溶血性贫血(TTP)的早期鉴别诊断,及时发现转移癌的证据,进一步胃镜等检查有利于尽快确定原发癌的部位。更多还原

【Abstract】 Objective To investigate clinical features of occult disseminated malignancy-associated microangiopathic hemolytic anemia(MAHA) in order to facilitate early diagnosis and therapy.Methods Four patients with metastatic carcinoma in which MAHA was the initial manifestation were reported and previously published cases in mainland of China were reviewed.Results In a review of literature from 1979 to 2010,19 additional cases with widespread neoplasms which were initially associated with MAHA were found.The median age of the 23 patients,including the present four,was 45 years(range: 16-86),eight were men and fifteen women.The prominent initial manifestations were MAHA,fever,diffused bone pain,neurological symptoms,mucocutaneous hemorrhage,and upper abdominal pain with concomitant hematemesis or hematochezia(78%,48%,48%,35%,35%,and 26% of cases,respectively).Peripheral blood counts showed marked anemia and moderate thrombocytopenia.Median hemoglobin level and platelet count were 52 g/L(range,26-89 g/L) and 35×109/L(range,5-98×109/L),respectively.Abnormalities of laboratory data of blood coagulation,such as prolonged prothrombin time,were found in 7 of 12 patients and four patients were complicated with disseminated intravascular coagulation.Marrow aspiration and(or) biopsy disclosed tumor invasion in eleven patients.Seventeen of 21 patients diagnosed by biopsy had disseminated adenocarcinoma,including 11 with gastric cancer,2 with lung cancer,1 with ovarial cancer,and 3 with cancer of unknown origin.Conclusion MAHA as an initial manifestation of metastatic malignancy predominantly occurs in metastatic adenocarcinoma.The symptom of bone pain,marrow aspiration and biopsy,and monitoring of hemostatic parameters are critical to differential diagnosis of thrombotic thrombocytopenic purpura(TTP) and early recognition of metastatic carcinoma.Further investigations,such as gastroscopy,could determine the origin of the neoplasm.更多还原