恶性组织细胞增生症。诊断依据染色体、免疫及组织病理分析。

E. Øster-jørgensen, M. Rønne, N. Jensen, N. Grunnet
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引用次数: 4

摘要

例女性,38岁,持续发热数月,无感染性或免疫性疾病征象。57复方博来霉素显像显示肝脏浓度异常。诊断性剖腹手术后,患者死于腹部出血。肝脏活检显示恶性细胞浸润。死亡前不久骨髓涂片显示有组织细胞。死亡前一天从患者身上获得的培养淋巴细胞显示t淋巴细胞缺乏。对染色体的研究表明,有几个克隆具有多倍体。恶性组织细胞增多症的诊断是基于红细胞吞噬、免疫和染色体畸变。如果恶性组织细胞增多症是原发疾病,不同疾病的终末期或继发于免疫功能不全(如病毒感染)的并发症的迹象是一个悬而未决的问题。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Malignant histiocytosis. The diagnosis based on chromosomal, immunologic and histiopathologic analysis.
: A 38-year-old female suffered for several months from persistent fever without signs of infectious or immunologic disease. 57Co-bleomycin scintigraphy revealed abnormal concentration in the liver. Following a diagnostic laparotomy, the patient died from abdominal haemorrhage. Malignant cell infiltration was demonstrated in a biopsy from the liver. Histiocytic cells were demonstrated in a bone marrow smear obtained shortly before death. Investigation of cultured lymphocytes obtained from the patient the day before she died showed deficiency of T-lymphocytes. Investigations of chromosomes delineated several clones with polyploidi. The diagnosis of malignant histiocytosis was based on erythrophagocytosis, and immunologic and chromosomal aberrations. If malignant histiocytosis is the primary disease, a terminal stage of different diseases or a sign of complication secondary to immuno incompetence (e.g. virus infection) is an open question.
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