Tassies D, Feliu E, Villamor N, Urbano-Ispizua A, Cervantes F, Campo E, Ordi J, Vernet M, Pujol R, López R
Servicio de Hematología, Hospital Clínic i Provincial Barcelona.
Med Clin (Barc). 1990 Nov 10;95(16):618-20.
Reactive leukocytosis has been reported in patients with non-Hodgkin's lymphoma of different histologic types. On the other hand, the blastic crisis of chronic myelocytic leukemia (CML) can sometimes be localized outside the bone marrow and simulate lymphoma, particularly when the blasts are of lymphoid lineage and the blastic crisis is the presenting feature of the disease. We report two patients in whom the differential diagnosis between lymphoblastic lymphoma with reactive leukocytosis and blastic crisis of CML outside the bone marrow was raised. They were two males aged 32 and 22 years, respectively, with lymphadenopathy (and one with splenomegaly), who were initially diagnosed of T lymphoblastic lymphoma. In both cases, leukocytosis was detected with myelemia and dysgranulopoiesis in the onset in one of them and when lymphadenopathy reappeared after remission in the other one. In addition, one patient had marked eosinophilia. In the bone marrow there was marked granulopoietic hyperplasia, with a reduction of fatty cells, and the granulocyte alkaline phosphatase index was reduced. However, the cytogenetic study did not disclose the existence of Philadelphia (Ph) chromosome, and bcr/abl molecular rearrangement was also not observed in the molecular study of both cases. We discuss the basic aspects of differential diagnosis between T lymphoblastic lymphoma with leukemoid reaction and T lymphoid lymphadenopathic blastic crisis of Ph-negative, bcr/abl-negative CML.
不同组织学类型的非霍奇金淋巴瘤患者中曾有反应性白细胞增多的报道。另一方面,慢性粒细胞白血病(CML)的原始细胞危象有时可局限于骨髓外并酷似淋巴瘤,尤其是当原始细胞为淋巴系且原始细胞危象是该疾病的首发特征时。我们报告了两名患者,他们出现了伴有反应性白细胞增多的淋巴母细胞淋巴瘤与骨髓外CML原始细胞危象之间的鉴别诊断问题。他们分别是两名32岁和22岁的男性,有淋巴结病(其中一人有脾肿大),最初被诊断为T淋巴母细胞淋巴瘤。在这两例中,均检测到白细胞增多,其中一例在发病时伴有髓细胞血症和粒细胞生成异常,另一例在缓解后淋巴结病复发时出现白细胞增多。此外,一名患者有明显的嗜酸性粒细胞增多。骨髓中可见明显的粒细胞生成增生,脂肪细胞减少,粒细胞碱性磷酸酶指数降低。然而,细胞遗传学研究未发现费城(Ph)染色体的存在,在两例的分子研究中也未观察到bcr/abl分子重排。我们讨论了伴有类白血病反应的T淋巴母细胞淋巴瘤与Ph阴性、bcr/abl阴性CML的T淋巴系淋巴结病性原始细胞危象之间鉴别诊断的基本要点。