Matloub Y H, Brunning R D, Arthur D C, Ramsay N K
Division of Pediatric Hematology-Oncology, University of Minnesota, Minneapolis.
Cancer. 1993 Jan 1;71(1):264-8. doi: 10.1002/1097-0142(19930101)71:1<264::aid-cncr2820710140>3.0.co;2-8.
Bone marrow aplasia preceding acute lymphoblastic leukemia (ALL) is a rare condition that usually affects children. The ALL generally follows the recovery of normal blood counts and most commonly occurs within 6 months of the onset of aplasia. The case of a patient with severe aplastic anemia is reported in whom ALL developed 15 months after the initial diagnosis of aplastic anemia. A literature search found 23 cases of ALL after a period of aplasia or hypoplasia. This patient's disease, however, was different from all previously reported ones. The severe aplasia lasted 15 months before being followed by ALL. There was no recovery of blood counts before the onset of ALL.
A review of the literature found 23 case reports in which aplasia or hypoplasia preceded ALL; these patients also had pancytopenia of the peripheral blood. Excluded from this review were patients whose bone marrow was hypoplastic, but who did not have pancytopenia because these did not have "aplastic anemia" as their initial disease.
Analysis of the reported patients showed that most were girls 10 years of age or younger. There was an overwhelming prevalence of fever, which in several instances, might have had an infectious cause. ALL most commonly occurred within 6 months of the aplasia and usually followed the recovery of normal blood counts.
Patients with ALL after a prolonged period of aplasia have several common characteristics including female sex, young age, and the prevalence of fever, often associated with an infectious illness. ALL usually follows the recovery of blood counts and occurs within 6 months of the onset of aplasia. The pathophysiology of this patient's disease(s) is still unclear. He could have had two unrelated disorders or a two-step leukemic process that followed a stem cell "insult." This patient had an antecedent hepatitis A infection 3 months before aplasia occurred. However, the authors were unable to identify with certainty any other event that might have caused additional bone marrow injury.
急性淋巴细胞白血病（ALL）之前出现的骨髓再生障碍是一种罕见病症，通常影响儿童。ALL一般在正常血细胞计数恢复后出现，最常见于再生障碍开始后的6个月内。本文报告了1例严重再生障碍性贫血患者，其在再生障碍性贫血初步诊断15个月后发生ALL。文献检索发现23例再生障碍或发育不全一段时间后发生ALL的病例。然而，该患者的疾病与之前所有报道的病例均不同。严重再生障碍持续了15个月后才出现ALL。ALL发病前血细胞计数未恢复。
文献回顾发现23例ALL之前出现再生障碍或发育不全的病例报告；这些患者外周血也有全血细胞减少。骨髓发育不全但无全血细胞减少的患者被排除在本回顾之外，因为这些患者最初疾病并非“再生障碍性贫血”。
对报告患者的分析表明，大多数是10岁及以下的女孩。发热极为普遍，在某些情况下可能有感染原因。ALL最常见于再生障碍后6个月内，通常在正常血细胞计数恢复后出现。
再生障碍长时间后发生ALL的患者有几个共同特征，包括女性、年轻以及发热普遍，常与感染性疾病相关。ALL通常在血细胞计数恢复后出现，发生在再生障碍开始后的6个月内。该患者疾病的病理生理学仍不清楚。他可能患有两种不相关的疾病，或者是干细胞“损伤”后发生的两步白血病过程。该患者在再生障碍发生前3个月有甲型肝炎感染。然而，作者无法确定任何其他可能导致额外骨髓损伤的事件。
