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儿童青少年骨髓单核细胞白血病:姑息化疗病例报告及适用于资源有限中心的文献综述

Juvenile Myelomonocytic Leukemia in a Child: A Case Report of Palliative Chemotherapy and Literature Review Applied to Limited Resources Centers.

作者信息

Yolanda Natharina, Gunawan Stefanus, Mantik Max F J, Veerman Anjo J P

机构信息

Department of Child Health, Faculty of Medicine, Sam Ratulangi University, Manado, Indonesia.

Amsterdam University Medical Center, VU University, Amsterdam, Netherlands.

出版信息

Case Rep Hematol. 2022 Nov 16;2022:1185140. doi: 10.1155/2022/1185140. eCollection 2022.

Abstract

Juvenile myelomonocytic leukemia (JMML) is a rare hematopoietic malignancy in children, with an incidence of 1.2 per million children per year. At this moment, we present a case report and a brief literature review of JMML in a child, primarily focused on its applicability in low-middle income countries. A 3.5-year-old male was referred to our tertiary center due to pallor, enlarging abdomen and neck mass, recurrent fever, and chronic diarrhea. Initial laboratory workup showed hemoglobin of 6.4 g/dl, white blood cell of 315.62 × 10/L, and platelet of 17 × 10/L. Blood smears showed 10% suspected blasts, 17% myelocytes, and 17% metamyelocytes with thrombocytopenic crisis. The HbF level was 5.8%. BCR-ABL gene tested negative. The patient was diagnosed with juvenile myelomonocytic leukemia. Considering that HSCT could not be done in our center and lack other financial possibilities to seek treatment abroad, the family agreed to do the palliative treatment. The patient was treated with oral 6-mercaptopurine and subcutaneous cytarabine. Four weeks after receiving 6-mercaptopurine, the white blood cell count decreased to 10.6 × 10/L and the spleen size was half of the original size. The patient continued chemotherapy until week 15, chemotherapy was stopped, but 16 weeks after the diagnosis of JMML, he developed severe thrombocytopenia, endophthalmitis, and sepsis and passed away. As a conclusion, in JMML cases in developing countries without HSCT, palliative chemotherapy is acceptable, and palliative care is an important aspect.

摘要

青少年粒单核细胞白血病(JMML)是儿童中一种罕见的造血系统恶性肿瘤,每年发病率为百万分之1.2。在此,我们呈现一例儿童JMML的病例报告及简要文献综述,主要关注其在低收入和中等收入国家的适用性。一名3.5岁男性因面色苍白、腹部及颈部肿块增大、反复发热和慢性腹泻被转诊至我们的三级医疗中心。初始实验室检查显示血红蛋白为6.4g/dl,白细胞为315.62×10⁹/L,血小板为17×10⁹/L。血涂片显示10%疑似原始细胞、17%早幼粒细胞和17%中幼粒细胞,伴有血小板减少危象。HbF水平为5.8%。BCR-ABL基因检测为阴性。该患者被诊断为青少年粒单核细胞白血病。考虑到我们中心无法进行造血干细胞移植(HSCT)且缺乏寻求国外治疗的其他资金渠道,家属同意进行姑息治疗。患者接受口服6-巯基嘌呤和皮下注射阿糖胞苷治疗。接受6-巯基嘌呤治疗四周后,白细胞计数降至10.6×10⁹/L,脾脏大小为原来的一半。患者继续化疗至第15周,化疗停止,但在诊断JMML后的第16周,他出现了严重的血小板减少、眼内炎和败血症并去世。结论是,在没有HSCT的发展中国家的JMML病例中,姑息化疗是可以接受的,姑息治疗是一个重要方面。

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