Dunphy C H, Kitchen S, Saravia O, Velasquez W S
Department of Pathology, St. Louis University Health Sciences Center, Missouri, USA.
Am J Hematol. 1996 Jan;51(1):85-9. doi: 10.1002/(SICI)1096-8652(199601)51:1<85::AID-AJH14>3.0.CO;2-A.
Acute myelofibrosis (AMF), as defined by an acute panmyelopathy associated with marked megakaryocytic hyperplasia and marrow fibrosis, appears to be a stem cell disorder. Even though it is most difficult to distinguish from various myeloproliferative and myelodysplastic disorders as well as acute myelogenous leukemia, it has rarely been reported to terminate as acute lymphoblastic leukemia (ALL). Only five cases have been reported in the literature; two from the pediatric literature and only three from the adult literature. Of the three adult cases, two were defined by light microscopy alone. Among the cases with follow-up (3/5), all died within 2 weeks to 2 months of diagnosis. We report an additional case in an adult; the ALL was defined by morphology, flow cytometric immunophenotyping, and cytogenetic analysis. The interval from diagnosis of AMF to ALL was 3 months. Our patient was treated with standard therapy for ALL, was in complete remission at last follow-up (3 months off maintenance therapy), and represents the only reported case who attained a complete remission. There are too few cases to determine the prognostic significance of termination of AMF in an acute leukemia of lymphoid origin vs. myeloid origin.
急性骨髓纤维化（AMF）定义为一种伴有显著巨核细胞增生和骨髓纤维化的急性全髓病，似乎是一种干细胞疾病。尽管它极难与各种骨髓增殖性疾病、骨髓增生异常综合征以及急性髓系白血病相区分，但很少有报道称其会转变为急性淋巴细胞白血病（ALL）。文献中仅报道了5例；2例来自儿科文献，仅有3例来自成人文献。在这3例成人病例中，2例仅通过光学显微镜确诊。在有随访记录的病例中（5例中的3例），所有患者均在诊断后2周内至2个月内死亡。我们报告了另外1例成人病例；该ALL通过形态学、流式细胞术免疫表型分析和细胞遗传学分析确诊。从AMF诊断到ALL的间隔时间为3个月。我们的患者接受了ALL的标准治疗，在最后一次随访时（停止维持治疗3个月）完全缓解，是唯一报道达到完全缓解的病例。由于病例太少，无法确定AMF转变为淋巴系起源与髓系起源的急性白血病的预后意义。
