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[毛细胞白血病的特殊特征]

[Specific characteristics of hairy cell leukemia].

作者信息

Hartmann D, Obrecht J P, Gudat F, Hess M, Dürig P, Nissen C, Schmidt R, Pietra C D

出版信息

Schweiz Med Wochenschr. 1978 Jun 24;108(25):962-5.

PMID:663587
Abstract

Report on a now 58-year-old female patient who presented in 1969 with the following objective findings: anemia, thrombocytopenia, lymphocytosis and hepatosplenomegaly. The first first diagnosis was lymphosarcoma, but up to 1976 nearly ten other diagnoses were established, e.g. reticulum sarcoma, leukemic lymphosarcoma, CML, ALL, CLL and Waldenström's disease. The patient did not respond to combined chemotherapy, but splenectomy brought about significant improvement in anemia and thrombocytopenia. She is now doing well without cytotoxic treatment. Hairy cell leukemia was diagnosed on the basis of electron microscopic findings in peripheral lymphocytes and histologic findings in bone marrow and spleen. The tartrate-resistant acid phosphatase was positive. Immunologic tests in the hairy cells showed surface immunoglobulins of monoclonal origina and the T-cells were shown to be decreased. The tests for phagocytosis and nonspecific esterase were negative. The hairy cells in this patient exhibited no colony stimulating activity in culture studies with bone marrow from 4 normal donors, as compared with the stimulating activity of normal monocytes in the same assay. In the light of this case report the question is discussed whether the hairy cells are mainly cells with lymphocytic (B-lymphocytes) or monocytic characteristics. On this question the findings in our patient support the hypothesis of a B-cell nature for the hairy cells.

摘要

报告一位现年58岁的女性患者,其于1969年出现以下客观检查结果:贫血、血小板减少、淋巴细胞增多及肝脾肿大。最初诊断为淋巴肉瘤,但至1976年又确立了近十种其他诊断,如网状细胞肉瘤、白血病性淋巴肉瘤、慢性粒细胞白血病、急性淋巴细胞白血病、慢性淋巴细胞白血病及瓦尔登斯特伦病。患者对联合化疗无反应,但脾切除使贫血和血小板减少有显著改善。她目前未经细胞毒性治疗,情况良好。根据外周淋巴细胞的电子显微镜检查结果以及骨髓和脾脏的组织学检查结果,诊断为毛细胞白血病。耐酒石酸酸性磷酸酶呈阳性。毛细胞的免疫试验显示表面免疫球蛋白为单克隆来源,且T细胞减少。吞噬作用和非特异性酯酶试验均为阴性。与同一试验中正常单核细胞的刺激活性相比,该患者的毛细胞在来自4名正常供体的骨髓培养研究中未表现出集落刺激活性。鉴于此病例报告,讨论了毛细胞主要是具有淋巴细胞(B淋巴细胞)还是单核细胞特征的细胞这一问题。在这个问题上,我们患者的检查结果支持毛细胞具有B细胞性质的假说。

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