CAJ | 학술논문

目的总结分析4例内脏利什曼病(VL)相关性噬血细胞性淋巴组织细胞增生症(HLH)患儿的临床资料,提高继发性HLH诊治水平.方法回顾性总结两家医院近期诊断的4例VL-HLH患儿的临床特征及诊治经过.结果本组4例患儿男3例、女1例,均来自于明确VL疫区或具有明确疫区生活史.以长期发热、肝脾肿大、外周血全血细胞减少为显著临床表现,结合相关实验室检查均符合HLH诊断标准.2例患儿接受HLH化疗并有效,表现为体温恢复正常和脾脏回缩等,但持续贫血为突出临床特征.病程早期骨髓检查和(或)rk39免疫试纸条法检测均无阳性发现,是导致漏诊和误诊的重要原因.结论 VL是导致继发性HLH的1种少见基础疾病.对来自VL疫区或具有疫区生活史的患儿,HLH鉴别诊断时应充分考虑VL可能性,需反复骨髓检查和(或)免疫学检查,早期诊断,及时予VL特异性治疗.
목적 총결분석4례내장리십만병(VL)상관성서혈세포성림파조직세포증생증(HLH)환인적림상자료,제고계발성HLH진치수평.방법 회고성총결량가의원근기진단적4례VL-HLH환인적림상특정급진치경과.결과 본조4례환인남3례、녀1례,균래자우명학VL역구혹구유명학역구생활사.이장기발열、간비종대、외주혈전혈세포감소위현저림상표현,결합상관실험실검사균부합HLH진단표준.2례환인접수HLH화료병유효,표현위체온회복정상화비장회축등,단지속빈혈위돌출림상특정.병정조기골수검사화(혹)rk39면역시지조법검측균무양성발현,시도치루진화오진적중요원인.결론 VL시도치계발성HLH적1충소견기출질병.대래자VL역구혹구유역구생활사적환인,HLH감별진단시응충분고필VL가능성,수반복골수검사화(혹)면역학검사,조기진단,급시여VL특이성치료.
Objective The clinical features of four cases of visceral leishmaniasis (VL)-associated hemophagocytic lymphohistiocytosis(VL-HLH)were retrospectively analyzed for the purpose of helping the diagnosis of secondary HLH. Method Clinical data of three childhood cases of VL-HLH documented in our hospital and one case diagnosed in the Capital Institute of Pediatrics was reviewed retrospectively,with particular emphasis on peculiar clinical manifestations and on clues to the diagnosis of this relatively rare disease entity.Result Three children were from endemic areas of VL, and the other one had lived in endemic area for one year,which was revealed by detailed history-taking.Clinically,VL-HLH is characterized by persistent fever, hepatosplenomegaly and pancytopenia, which is similar to those of HLH, and is one of the important reasons of delayed diagnosis or misdiagnosis. Based on the HLH-2004 protocol, all the four cases met the diagnostic criteria of HLH. In addition, bone marrow aspirate and immunologic detection of VL-specific antibody via rk39 dipstick test during the early disease course of VL-HLH yielded negative results.Two cases who received HLH-targeted therapy responded reasonably well, with rapid temperature normalization and spleen retraction. Nevertheless,Hb remained lower than normal,which we believed to be related to persistent red cell destruction by the invading parasite Leishmania donovani. Conclusion VL,a parasitic disease caused by Leishmania donovani, which is currently endemic just in 6 provinces in China, shares similar clinical picture of HLH and is an easily ignored underlying cause of secondary HLH.We suggest that VL should be in the list of differential diagnosis for any patients with HLH who lives in or has a definite travel history to endemic areas.Repeated bone marrow studies are highly warranted to make a definite diagnosis of VL, because bone marrow aspirate or rk39 dipstick test during early disease course might yield negative results.Although VL-HLH responds quite well to HLH-tailored chemotherapy,specific therapy against VL must be given to prevent disease recurrence, and HLH-targeted chemotherapy might be discontinued to prevent chemotherapy-related toxicities.