恶性组织细胞病37例分析

Analysis of 37 Cases of Malignant Histiocytosis

目的:加强对恶性组织细胞病(恶组)的认识,提高诊断准确率。方法:对已确诊的37例恶组病例进行回顾性分析和总结。结果:临床表现以发热、乏力及进行性衰竭为主(100.0%),其余依次是淋巴结肿大(86.5%)、脾肿大(67.6%)、肝肿大(64.9%)、出血(62.2%)、双下肢水肿(16.2%)、腹痛(13.5%)、皮肤巩膜黄染(10.8%)及其他一些少见表现。外周血常规检查三系减少者64.9%、二系减少者21.6%、一系减少者13.5%,其中WBC减少者占64.9%、正常者21.6%、升高者13.5%,Hb降低者100.0%,PLT降低者86.5%,查见异常细胞者29.7%。肝功能异常者43.2%,肾功能异常者8.1%,血清铁蛋白及心肌酶谱极度增高者各2.7%。经1次骨穿确诊者43.2%、经多次骨穿或淋巴结活检或尸检确诊者56.8%。结论:本病临床表现及实验室检查结果复杂多样,容易漏诊、误诊,临床及病理检验人员应提高对本病的认识,及时行骨髓穿刺或活检,必要时行淋巴结活检,以临床表现为基础,细胞形态学和病理学检查为主要诊断依据,是减少误诊、提高诊断准确率的关键。

Objective：To increase the knowledge of malignant histiocytosis and the accuracy rate of diagnosis. Methods：We retrospectively analyzed the final diagnosed 37 cases of malignant histiocytosis. Results：The symptoms of this disease were fever, fatigue and progressive exhaustion （ 100% ）, lymph node swelling （86.5%）, enlarged spleen （67.6%） , enlarged liver （64.9%） , hemorrhage （62.2 % ）, lower limb edema （ 16.2 % ）, stomachache （ 13.5 % ） , skin and sclera stained yellow （ 10.8 % ） and other uncommon manifestation. The patients whose 3 lineages decreased daring blood routine examination accounted for 64.9%, 2 lineages decreased 21.6% and 1 lineages decreased 13.5%. Among of them, the patients whose WBC decreased accounted for 64.9% , being normal 21.6 %, and increased 13.5 %. The patients whose Hb decreased accounted for 100.0 %, and PLT decreased 86.5 %. Abnormal cell could be found in 29.7 percent cases. The patients whose liver function was abnormal accounted for 43.2%, renal function being abnormal 8.1%, and blood serum ferritin and cardiac muscle zymogram extremely increase 2.7 %. The patients who were finally diagnosed by once bone marrow aspiration accounted for 43.2 %, by many times bone marrow aspiration or lymph node biopsy or autopsy 56.8%. Conclusions：The symptoms and examination results of this disease are various which often lead to missed diagnosis and misdiagnosis . Both doctors and laboratory assistants should improve knowledge of this disease. Bone marrow aspiration or biopsy, even lymph node biopsy should be done promptly if they are necessary. The diagnosis of this disease which is based on symptoms, meanwhile taken the morphocytology and pathological examination as principal evidences, is the key to reduce misdiagnosis.