Schouten T J, Hustinx T W, Scheres J M, Holland R, de Vaan G A
Cancer. 1983 Oct 1;52(7):1229-36. doi: 10.1002/1097-0142(19831001)52:7<1229::aid-cncr2820520717>3.0.co;2-n.
Malignant histiocytosis (MH) was diagnosed on the cytologic and cytochemical features of the malignant cells present in bone marrow smears from an infant and a child. The diagnosis of MH was confirmed by light and electron microscopic studies on bone marrow and skin biopsy specimens, and bone marrow and liver biopsy specimens, respectively. Both patients showed a deterioration while receiving prednisone monotherapy, but they responded well to a combination of vincristine and cyclophosphamide. The infant has remained disease-free for 52+ months now, but the child died of a relapse 11 months after diagnosis. Cytogenetic studies of blood and/or bone marrow cells were performed before treatment. In the infant, a pathologic cell line with a translocation t(8;16)(p11;p13) was found; this abnormality was no longer present after remission was obtained. In the second patient, a hyperdiploid cell line with major karyotypic anomalies was found. When studied in relapse and shortly before death, additional chromosomal abnormalities were seen. The data from this study show that prednisone should be used with caution in MH, and that it should be omitted from combination chemotherapy when adverse effects are noted during short-term monotherapy. Also, cytogenetic studies should be performed more often in MH to determine the significance and possible nonrandomness of chromosomal abnormalities in this disease.
根据一名婴儿和一名儿童骨髓涂片恶性细胞的细胞学和细胞化学特征诊断为恶性组织细胞增生症（MH）。分别对骨髓和皮肤活检标本、骨髓和肝脏活检标本进行光镜和电镜研究，证实了MH的诊断。两名患者在接受泼尼松单一疗法时病情均恶化，但对长春新碱和环磷酰胺联合治疗反应良好。该婴儿目前已无病生存52多个月，但该儿童在诊断后11个月死于复发。治疗前对血液和/或骨髓细胞进行了细胞遗传学研究。在该婴儿中，发现了一条具有t(8;16)(p11;p13)易位的病理细胞系；缓解后该异常不再存在。在第二名患者中，发现了一条具有主要核型异常的超二倍体细胞系。在复发时和死亡前不久进行研究时，发现了其他染色体异常。本研究数据表明，MH患者使用泼尼松应谨慎，短期单一疗法出现不良反应时应从联合化疗中停用。此外，应更频繁地对MH患者进行细胞遗传学研究，以确定该疾病染色体异常的意义和可能的非随机性。
