Mihál V, Hajdúch M, Dusek J, Jarosová M, Weigl E, Pikalová Z, Indrák K, Safárová M, Janostáková A
Department of Pediatrics, Faculty of Medicine, Olomouc, Czech Republic.
Neoplasma. 1997;44(2):133-6.
A five-year-old girl, initially diagnosed as having acute lymphoblastic leukemia (ALL; FAB-L1) relapsed with ALL 4 months after completion of chemotherapy (BFM 83). The initial ALL presentation and subsequent ALL relapse were analyzed using conventional morphology, cytochemistry, cytogenetics and immunophenotyping. The results were consistent with a diagnosis of B-lymphocyte precursor ALL. Bone marrow leukemic cells revealed a 46, XX karyotype at diagnosis and a 46, XX, del(7) (q22; qter) when the girl first relapsed. The case was managed with a BFM REZ-ALL 90 protocol. Upon completion of the first cycle of the protocol, severe myelosuppression developed. This was treated with GM-CSF. Three days later, however, GM-CSF was stopped because the WBC reached 1.1 x 10(9) per liter with 60% of blasts in peripheral blood. Laboratory characteristics were typical of AML. Cytogenetic analysis revealed 46, XX, del(7) (q22; qter) karyotype as before. The bcr-abl fusion gene was not detected. Myeloid blasts were placed in a culture and maintained at 37 degrees C and 7.5% CO2 for two weeks. During this period, formation of hemopoietic colonies was observed and subsequently analyzed using histology and electron microscopy. This showed that the colonies consisted of differentiating erythroid, megakaryocytic and myeloid cells. Further, the chemosensitivity of leukemic cells was examined in both "lymphoid" and "myeloid" relapse instances. While the "lymphoid" phenotype was characterized by good sensitivity to corticosteroids, a typical feature of the "myeloid" phenotype was a high resistance to corticosteroids with marginally increased sensitivity to ARA-C.
一名五岁女孩最初被诊断为急性淋巴细胞白血病（ALL；FAB-L1），在完成化疗（BFM 83方案）4个月后复发。使用传统形态学、细胞化学、细胞遗传学和免疫表型分析了最初的ALL表现及随后的ALL复发情况。结果符合B淋巴细胞前体ALL的诊断。骨髓白血病细胞在诊断时显示为46, XX核型，女孩首次复发时为46, XX, del(7)(q22; qter)。该病例采用BFM REZ-ALL 90方案治疗。在方案的第一个周期完成后，出现了严重的骨髓抑制。用粒细胞巨噬细胞集落刺激因子（GM-CSF）进行了治疗。然而，三天后，由于白细胞计数达到每升1.1×10⁹且外周血中原始细胞占60%，GM-CSF被停用。实验室特征符合急性髓系白血病（AML）。细胞遗传学分析显示核型与之前一样为46, XX, del(7)(q22; qter)。未检测到bcr-abl融合基因。将髓系原始细胞置于培养中，在37℃和7.5%二氧化碳条件下维持两周。在此期间，观察到造血集落的形成，随后用组织学和电子显微镜进行分析。结果表明这些集落由分化的红系、巨核系和髓系细胞组成。此外，还在“淋巴细胞性”和“髓细胞性”复发情况下检测了白血病细胞的化学敏感性。“淋巴细胞性”表型对皮质类固醇敏感，而“髓细胞性”表型的典型特征是对皮质类固醇高度耐药，对阿糖胞苷（ARA-C）的敏感性略有增加。
