Baylor College of Medicine Children's Foundation Uganda, Kampala, Uganda.
Baylor College of Medicine and Texas Children's Hospital, Houston, TX. USA.
Afr Health Sci. 2021 Jun;21(2):683-686. doi: 10.4314/ahs.v21i2.25.
Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy and is characterised by hyperproliferation of malignant lymphocytes in the bone marrow. Rarely, ALL may be preceded by a period of pancytopenia and bone marrow hypoplasia which spontaneously recovers. This phenomenon, which has not before been described in T-cell ALL, is referred to as transient bone marrow hypoplasia.
A 5-year-old boy who presented with high-grade fever and generalised lymphadenopathy, was found to have pancytopenia on peripheral blood count and bone marrow hypoplasia. He was observed over a one-month period during which his bone marrow and peripheral blood counts recovered spontaneously. Symptoms recurred after 4 months and he was found to have blast infiltration of the bone marrow and diagnosed with T-cell ALL.
Cases of transient bone marrow hypoplasia or overt aplastic anemia with spontaneous recovery and then followed by B-cell ALL or Acute Myeloid Leukemia have been described previously in the medical literature. This is the first case of transient bone marrow hypoplasia resulting into ALL of T-cell immunophenotype. While marrow hypoplasia preceding ALL remains poorly understood, it suggests an antecedent environmental insult to lymphoid progenitors or a germline abnormality that predisposes to lymphoid dysplasia. This may provide clues to the hitherto unknown pathophysiological process and etiological factors that precede the majority of childhood ALL cases. This case enlightens pediatricians about the existence of such rare cases so as to periodically follow up children with pancytopenia and/or bone marrow hypoplasia for prolonged periods even after apparent recovery.
急性淋巴细胞白血病(ALL)是最常见的儿童恶性肿瘤,其特征是骨髓中恶性淋巴细胞的过度增殖。罕见情况下,ALL 可能先出现一段时间的全血细胞减少和骨髓发育不良,这些症状会自发恢复。这种现象以前在 T 细胞 ALL 中没有描述过,被称为一过性骨髓发育不良。
一名 5 岁男孩因高热和全身淋巴结肿大就诊,在外周血象检查中发现全血细胞减少和骨髓发育不良。他在一个月的时间里接受了观察,在此期间,他的骨髓和外周血象自发恢复。4 个月后症状再次出现,骨髓中发现有原始细胞浸润,诊断为 T 细胞 ALL。
以前在医学文献中曾描述过一过性骨髓发育不良或明显再生障碍性贫血伴自发性恢复,随后发生 B 细胞 ALL 或急性髓细胞白血病的病例。这是首例导致 T 细胞免疫表型 ALL 的一过性骨髓发育不良病例。虽然 ALL 前骨髓发育不良的机制尚不清楚,但它提示淋巴细胞前体细胞受到先前环境损害或存在导致淋巴细胞发育不良的种系异常。这可能为以前未知的大多数儿童 ALL 病例的病理生理过程和病因因素提供线索。本病例提醒儿科医生注意到这种罕见病例的存在,以便在全血细胞减少和/或骨髓发育不良明显恢复后,对儿童进行长时间定期随访。