Langerhans Cell Histiocytosis Complicated with Hemophagocytic Lymphohistiocytosis:Two Case Reports
10.3870/j.issn.1004-0781.2025.12.019
- VernacularTitle:朗格汉斯细胞组织细胞增生症合并噬血细胞综合征2例
- Author:
Yanqing CHEN
1
;
Jianxin DUN
1
;
Ai ZHANG
1
;
Aiguo LIU
1
;
Qun HU
1
Author Information
1. 华中科技大学同济医学院附属同济医院儿童血液与肿瘤科,武汉 430030
- Publication Type:Journal Article
- Keywords:
Langerhans cell histiocytosis;
Hemophagocytic lymphohistiocytosis,children
- From:
Herald of Medicine
2025;44(12):2005-2009
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze cases of Langerhans cell histiocytosis(LCH)initially presenting with hemophagocytic lymphohistiocytosis(HLH)in order to enhance clinicians'awareness and avoid misdiagnosis or missed diagnosis.Methods Clinical characteristics of two cases of LCH with HLH as the initial symptom cases were retrospectively collected and analyzed.Results Both patients were male and admitted with"fever and bicytopenia".The physical examination revealed scattered rashes and hepatosplenomegaly.Based on the laboratory results,HLH was initially considered in both children.In case 1,the underlying disease was not immediately identified,but a skin biopsy later confirmed LCH.The patient's symptoms improved with LCH-directed therapy;however,a secondary pulmonary infection triggered progressive hemophagocytosis,which was successfully controlled after adding HLH treatment.In Case 2,HLH treatment initially stabilized the fever.After a skin biopsy confirmed LCH,combined LCH therapy was administered,and the symptoms improved.Both patients received regular chemotherapy according to the CCHG-LCH-2019 protocol.During follow-up,Case 1 developed a BRAF gene mutation and was treated with oral trametinib in addition to chemotherapy.As of the latest follow-up,both patients remained in stable condition.Conclusion Children with HLH and accompanied by a rash should actively search for the underlying cause.A skin biopsy should be performed promptly to clarify the diagnosis.Once the diagnosis is confirmed,active treatment is necessary,a single treatment approach should be avoided,and timely assessment of the condition should be conducted to achieve personalized treatment.
