骨髓坏死患者的临床特点、预后因素及误诊分析

Clinical Characteristics,Prognostic Factors and Misdiagnosis of Bone Marrow Necrosis Patients

目的:分析骨髓坏死(BMN)患者的临床特征、诊断及预后,避免误诊、漏诊或延误治疗。方法:回顾性分析2010年1月至2017年12月徐州医科大学附属医院收治的51例BMN患者的临床资料,总结其原发病种类、病因、临床表现、实验室检查及影像学表现、治疗转归及预后影响因素,并对误诊原因进行分析。结果:51例BMN患者中,原发病为血液肿瘤32例,实体肿瘤所致骨髓转移癌14例,良性病变者5例。患者从出现症状到确诊BMN的时间为7 d-6个月,中位时间为35 d。25.5%BMN患者存在误诊和漏诊。51例BMN患者中,贫血占100%,发热占58.8%,全身骨痛占52.9%,出血占29.4%,淋巴结肿大占37.3%,肝脾肿大占19.6%。幼红-幼粒细胞贫血占84.3%,二系细胞减少占51.0%,全血细胞减少占25.5%,单系细胞减少占23.5%。血清学检查无特异性。1次骨髓穿刺确诊者38例,多部位穿刺确诊者7例,骨髓涂片的诊断符合率为88.2%。51例患者中,41例行骨髓活检,骨髓活检的诊断符合率为75.6%。13例BMN患者脊柱/骨盆MRI显示多发椎体异常信号,16例患者的PET-CT显像示骨髓弥漫性摄取FDG低下,骨髓浸润时伴局部摄取增高。46例合并BMN恶性疾病的患者中,35例死亡(76.1%),中位生存期为25 d。32例血液肿瘤患者中,12例早期死亡,20例针对原发病积极化疗后,11例BMN消失,9例于1周-3个月内死亡。14例骨髓转移癌合并BMN患者于2周-3个月内死亡。5例继发于非恶性疾病的BMN患者,4例经对症支持治疗,坏死灶消失,现仍存活。对BMN患者预后的影响因素进行多因素Logistic回归分析,结果显示,BMN预后与原发病因素(良恶性)密切相关。误、漏诊主要原因:原发病隐匿,症状无一定规律,对主要临床特点和血液学异常缺乏认识和警惕性,未及时行多部位骨髓穿刺和骨髓活检等。结论:BMN主要伴发于血液肿瘤及实体瘤骨髓转移,其预后与原发病性质及本身危重性密切相关,临床上对剧烈骨痛、发热、肝脾淋巴结肿大、血细胞减少伴幼红-幼粒细胞贫血者,应高度警惕BMN的存在。多部位骨髓穿刺和骨髓活检可相互弥补各自不足,二者联合检查可以提高BMN诊断符合率和病因诊断阳性率。

Objective:To analyze the clinical characteristics,diagnosis and prognostic factors of bone marrow necrosis(BMN)patients,aim to avoid misdiagnosis,missed diagnosis or delayed treatment.Methods:The clinical data of 51 BMN patients treated in the Affiliated Hospital of Xuzhou Medical University from January 2010 to December2017 were retrospectively analyzed.The types of primary disease,etiology,clinical manifestations,laboratory tests,radiological findings,treatment outcomes and prognostic factors were summrized,and the reasons for misdiagnosis were analyzed.Results:Among 51 BMN patients,the hematologic tumor was detected out in 32 patients;solid tumors causedBMN was detected out in 14 patients,benign lesions for 5 patients.The time of interval from the appearance of symptoms to the confirmation of BMN was 7 days to 6 months,with a median of 35 days.Misdiagnosis and missed diagnosis occurred in 25.5%of the BMN patients.Anemia was found in all of the 51 BMN patients,fever accounted for 58.8%,systemic bone pain for 52.9%,bleeding for 29.4%,lymphadenectasis for 37.3%,and hepatosplenomegaly for 19.6%.Leukoerythroblastic anemia accounted for 84.3%,bicytopenia for 51.0%,pancytopenia for 25.5%,and monocytopenia for 23.5%.The serologic test revealed no specific results.The first bone marrow aspiration were 38 patients and multi-site puncture were 7 patients.The diagnostic coincidence rate of bone marrow smear was 88.2%.Among 51 BMN patients,41 patients received bone marrow biopsy,and the diagnostic coincidence rate of bone marrow biopsy was 75.6%.The abnormal signals were found in multiple vertebral bodies by spinal/pelvic MRI scan in 13 BMN patients;PET-CT scan revealed a diffuse pattern of low FDG uptake in the bone marrow in 16 patients,with a local increase in FDG uptake accompanied by bone marrow involvement.For 46 patients with BMN combined with malignancies,among which35 patients died(76.1%)and the median survival time was 25 days.Among the 32 patients with hematologic tumors,early death occurred in 12 patients,BMN disappeared in 11 out of 20 patients received active chemotherapy for the primary disease,9 patients died within 1 week to 3 months.Fourteen patients combined with bone marrow metastatic carcinoma died within 2 weeks to 3 months.Focal necrosis disappeared in 4 out of 5 BMN patients secondary to nonmalignant diseases after symptomatic supportive treatment and still alived.Multiple logistic regression was performed to analyze factors affecting the prognosis of BMN patients,the result showed that the prognosis of BMN was closely related to the factors of primary disease(benign and malignant).The reasons for misdiagnosis and missed diagnosis were as follows:hidden onset of the primary disease,nonspecific symptoms,insufficient understanding and alertness of the physicians regarding the primary clinical characteristics and hematological abnormalities,and failure to receive multiple sites bone marrow punctures or bone marrow biopsies.Conclusion:BMN usually occurs concomitantly to hematologic tumors and bone marrow metastases from solid tumors.Its prognosis is closely related to the nature and severity of the primary disease and its own severity.In the clinic,BMN should be suspected in patients with severe bone pain,fever,hepatosplenomegaly,hemocytopenia,lymphadenectasis and leukoerythroblastic anemia.Bone marrow puncture at multiple positions and bone marrow biopsy can compensate for each other in the diagnosis of BMN.The combined use of the two methods can improve the diagnostic coincidence rate of BMN,and the positive rate of the etiological diagnosis of BMN.