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Juvenile myelomonocytic leukemia presenting in an infant with a subdural hematoma
Child's Nervous System ( IF 1.4 ) Pub Date : 2021-01-06 , DOI: 10.1007/s00381-020-05013-7
William A Lambert 1 , Joseph A DiGiuseppe 2 , Tatiana Lara-Ospina 3 , Markus J Bookland 4, 5, 6 , Jonathan E Martin 4, 5 , David S Hersh 4, 5, 6
Affiliation  

Background

Juvenile myelomonocytic leukemia (JMML) is a rare childhood hematopoietic disorder typically presenting with hepatosplenomegaly, lymphadenopathy, pallor, fever, and cutaneous findings. The authors report the first case, to our knowledge, of JMML presenting in a pediatric patient with a subdural hematoma.

Case description

A 7-month old male with recurrent respiratory infections and a low-grade fever presented with a full fontanelle and an increasing head circumference and was found to have chronic bilateral subdural collections. Abusive head trauma, infectious, and coagulopathy workups were unremarkable, and the patient underwent bilateral burr holes for evacuation of the subdural collections. The postoperative course was complicated by the development of thrombocytopenia, anemia, and an acute subdural hemorrhage which required evacuation. Cytologic analysis of the subdural fluid demonstrated atypical cells, which prompted flow cytometric analysis, a bone marrow biopsy, and ultimately a diagnosis of JMML. Following chemotherapy, the patient’s counts improved, and he subsequently underwent a hematopoietic stem cell transplant.

Conclusion

Subdural collections may rarely represent the first presenting sign of hematologic malignancies. In patients with a history of recurrent infections and a negative workup for abusive head trauma, clinicians should include neoplastic etiologies in the differential for chronic subdural collections and have a low threshold for fluid analysis.



中文翻译:

幼年型粒单核细胞白血病患儿出现硬膜下血肿

背景

幼年型粒单核细胞白血病 (JMML) 是一种罕见的儿童造血系统疾病,通常表现为肝脾肿大、淋巴结肿大、脸色苍白、发热和皮肤表现。据我们所知,作者报告了第一例 JMML 出现在患有硬膜下血肿的儿科患者中。

案例描述

一名 7 个月大的男性,反复呼吸道感染和低烧,囟门饱满,头围增加,发现有慢性双侧硬膜下积液。虐待性头部外伤、感染和凝血功能障碍检查均无异常,患者接受了双侧钻孔以清除硬膜下积液。术后病程因血小板减少症、贫血和需要疏散的急性硬膜下出血而变得复杂。硬膜下液的细胞学分析显示非典型细胞,这促使流式细胞术分析、骨髓活检,并最终诊断为 JMML。化疗后,患者的计数有所改善,随后他接受了造血干细胞移植。

结论

硬膜下积液可能很少代表血液系统恶性肿瘤的第一个表现体征。对于有复发性感染史和虐待性头部外伤检查结果阴性的患者,临床医生应将肿瘤病因纳入慢性硬膜下积液的鉴别诊断中,并降低液体分析阈值。

更新日期:2021-01-06
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