Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation. It occurs because of severe inflammation due to uncontrolled proliferation of activated lymphocytes and histiocytes, characterized by the production of excessive levels of cytokines. Virus-associated HLH is a well-known entity, and parvovirus B19 is one of the common causes. Parvovirus B19 can also affect blood cell lineages. Therefore, HLH may be accompanied by several diseases such as cytopenia, aplastic anemia, and myelodysplastic syndrome. Herein, we report the case of a patient with hereditary spherocytosis who was diagnosed with parvovirus B19-induced HLH and aplastic crisis. A 7-year-old girl presented to our hospital with fever, pleural effusion, pancytopenia, hepatosplenomegaly, and hypotension. A bone marrow biopsy was performed under the suspicion of HLH, which revealed hemophagocytes. The diagnostic criteria for HLH were met, and prompt chemoimmunotherapy was initiated considering the clinically unstable situation. Her health improved rapidly after initiating treatment. Further study revealed that she had hereditary spherocytosis, and parvovirus B19 had caused aplastic crisis and HLH. The patient’s clinical progress was excellent, and chemoimmunotherapy was reduced and discontinued at an early stage. This case shows that aplastic crisis and HLH can coexist with parvovirus B19 infection in patients with hereditary spherocytosis. Although the prognosis was good in this case of HLH caused by parvovirus B19, early detection and active treatment are essential.

摘要

噬血细胞性淋巴组织细胞增多症（HLH）是一种病理性免疫激活综合征。它的发生是由于活化的淋巴细胞和组织细胞的不受控制的增殖导致的严重炎症，其特征是产生过量水平的细胞因子。病毒相关 HLH 是一个众所周知的实体，细小病毒 B19 是常见的原因之一。细小病毒 B19 也可以影响血细胞谱系。因此，HLH可能伴有血细胞减少、再生障碍性贫血和骨髓增生异常综合征等多种疾病。在此，我们报告一例被诊断为细小病毒 B19 诱导的 HLH 和再生障碍危象的遗传性球形红细胞增多症患者的病例。一名 7 岁女孩因发热、胸腔积液、全血细胞减少、肝脾肿大和低血压来我院就诊。在怀疑 HLH 的情况下进行了骨髓活检，结果显示有噬血细胞。符合HLH的诊断标准，考虑到临床不稳定情况及时启动化学免疫治疗。开始治疗后，她的健康状况迅速好转。进一步研究表明，她患有遗传性球形红细胞增多症，细小病毒 B19 引起了再生障碍危象和 HLH。患者临床进展良好，早期减少化疗免疫治疗。该病例表明，在遗传性球形红细胞增多症患者中，再生障碍危象和 HLH 可与细小病毒 B19 感染并存。虽然这例由细小病毒 B19 引起的 HLH 预后良好，但早期发现和积极治疗至关重要。考虑到临床不稳定的情况，立即开始化学免疫治疗。开始治疗后，她的健康状况迅速好转。进一步研究表明，她患有遗传性球形红细胞增多症，细小病毒 B19 引起了再生障碍危象和 HLH。患者临床进展良好，早期减少化疗免疫治疗。该病例表明，在遗传性球形红细胞增多症患者中，再生障碍危象和 HLH 可与细小病毒 B19 感染并存。虽然这例由细小病毒 B19 引起的 HLH 预后良好，但早期发现和积极治疗至关重要。考虑到临床不稳定的情况，立即开始化学免疫治疗。开始治疗后，她的健康状况迅速好转。进一步研究表明，她患有遗传性球形红细胞增多症，细小病毒 B19 引起了再生障碍危象和 HLH。患者临床进展良好，早期减少化疗免疫治疗。该病例表明，在遗传性球形红细胞增多症患者中，再生障碍危象和 HLH 可与细小病毒 B19 感染并存。虽然这例由细小病毒 B19 引起的 HLH 预后良好，但早期发现和积极治疗至关重要。细小病毒B19引起了再生障碍危机和HLH。患者临床进展良好，早期减少化疗免疫治疗。该病例表明，在遗传性球形红细胞增多症患者中，再生障碍危象和 HLH 可与细小病毒 B19 感染并存。虽然这例由细小病毒 B19 引起的 HLH 预后良好，但早期发现和积极治疗至关重要。细小病毒B19引起了再生障碍危机和HLH。患者临床进展良好，早期减少化疗免疫治疗。该病例表明，在遗传性球形红细胞增多症患者中，再生障碍危象和 HLH 可与细小病毒 B19 感染并存。虽然这例由细小病毒 B19 引起的 HLH 预后良好，但早期发现和积极治疗至关重要。