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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。
Objective To investigate the clinical features of patients with concealed diffuse cancer-associated microangiopathy and hemolytic anemia (MAHA), which is helpful for the early diagnosis and treatment of cancer. Methods Four cases of MAHA associated with occult diffuse carcinoma were reported, and the related domestic literatures were analyzed. Results In the past 30 years, 19 cases of occult diffuse cancer-associated MAHA were reported in our country, including 4 cases (23 cases), median age 45 years old (16-86 years old), 8 males and 15 females, Clinical manifestations include MAHA-related symptoms, fever, multiple bone pain, neuropsychiatric disorders, mucocutaneous bleeding and upper abdominal pain associated with hematemesis or melena, with rates of 78%, 48%, 48%, 35%, 35%, and 26 %. Laboratory tests: Hb median 52 (26-89) g / L, median platelet 35 (5 ~ 98) × 109 / L; 12 patients with coagulation test in 7 patients with PT prolongation and other abnormalities, including 4 cases Concurrent DIC; 11 patients with bone marrow smears and pathological biopsy found metastatic cancer cells. 21 cases of pathological diagnosis of adenocarcinoma in 17 cases, of which 11 cases of gastric cancer. Conclusions Most of the patients with occult diffuse cancers secondary to MAHA are adenocarcinoma, bone pain, bone marrow smears and biopsy and dynamic coagulation and fibrinolysis, which are helpful for the early differential diagnosis of thrombolytic hemolytic anemia (TTP) , Timely detection of metastatic evidence, further gastroscopy and other tests will help determine the site of primary cancer as soon as possible.