中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2014年
7期
628-632
,共5页
肖莉%管贤敏%孟岩%苏庸春%宪莹%肖剑文%崔颖慧%于洁
肖莉%管賢敏%孟巖%囌庸春%憲瑩%肖劍文%崔穎慧%于潔
초리%관현민%맹암%소용춘%헌형%초검문%최영혜%우길
淋巴组织细胞增多症,嗜血细胞性%儿童%回顾性研究%HLH-2004方案
淋巴組織細胞增多癥,嗜血細胞性%兒童%迴顧性研究%HLH-2004方案
림파조직세포증다증,기혈세포성%인동%회고성연구%HLH-2004방안
Lymphohistiocytosis,hemophagocytic%Children%Retrospective study%HLH-2004 protocol
目的 探讨我国儿童噬血细胞性淋巴组织细胞增生症(HLH)的临床及实验室检查特点.方法 回顾性分析217例HLH患儿的临床及实验室检查资料.结果 217例患儿中男女比例为1.11∶1,中位发病年龄为3岁5个月(6个月~16岁9个月),发病高峰年龄为1~5岁(占61.3%).感染相关HLH最多见(占77%,其中92.2%为EB病毒感染),其余为肿瘤相关HLH、自身免疫性疾病相关HLH等.临床以持续高热(100.0%)、肝脏肿大(92.6%)、脾脏肿大(88.4%)为突出表现,超过半数患儿有中枢神经系统及呼吸系统受累表现.实验室检查结果显示铁蛋白升高最为显著(98.0%),其次为骨髓噬血现象(90.7%)及凝血功能障碍(76.5%);所有患儿均有不同程度的肝功能损害;外周血淋巴细胞分类异常常见.结论 HLH是一组异质性疾病,病因及临床表现多样;HLH-2004诊断方案对于诊断HLH有其理论基础及临床可操作性;肝功能损害相关指标、外周血淋巴细胞分类、中枢神经系统和呼吸系统受累表现对HLH的诊断具有参考价值.
目的 探討我國兒童噬血細胞性淋巴組織細胞增生癥(HLH)的臨床及實驗室檢查特點.方法 迴顧性分析217例HLH患兒的臨床及實驗室檢查資料.結果 217例患兒中男女比例為1.11∶1,中位髮病年齡為3歲5箇月(6箇月~16歲9箇月),髮病高峰年齡為1~5歲(佔61.3%).感染相關HLH最多見(佔77%,其中92.2%為EB病毒感染),其餘為腫瘤相關HLH、自身免疫性疾病相關HLH等.臨床以持續高熱(100.0%)、肝髒腫大(92.6%)、脾髒腫大(88.4%)為突齣錶現,超過半數患兒有中樞神經繫統及呼吸繫統受纍錶現.實驗室檢查結果顯示鐵蛋白升高最為顯著(98.0%),其次為骨髓噬血現象(90.7%)及凝血功能障礙(76.5%);所有患兒均有不同程度的肝功能損害;外週血淋巴細胞分類異常常見.結論 HLH是一組異質性疾病,病因及臨床錶現多樣;HLH-2004診斷方案對于診斷HLH有其理論基礎及臨床可操作性;肝功能損害相關指標、外週血淋巴細胞分類、中樞神經繫統和呼吸繫統受纍錶現對HLH的診斷具有參攷價值.
목적 탐토아국인동서혈세포성림파조직세포증생증(HLH)적림상급실험실검사특점.방법 회고성분석217례HLH환인적림상급실험실검사자료.결과 217례환인중남녀비례위1.11∶1,중위발병년령위3세5개월(6개월~16세9개월),발병고봉년령위1~5세(점61.3%).감염상관HLH최다견(점77%,기중92.2%위EB병독감염),기여위종류상관HLH、자신면역성질병상관HLH등.림상이지속고열(100.0%)、간장종대(92.6%)、비장종대(88.4%)위돌출표현,초과반수환인유중추신경계통급호흡계통수루표현.실험실검사결과현시철단백승고최위현저(98.0%),기차위골수서혈현상(90.7%)급응혈공능장애(76.5%);소유환인균유불동정도적간공능손해;외주혈림파세포분류이상상견.결론 HLH시일조이질성질병,병인급림상표현다양;HLH-2004진단방안대우진단HLH유기이론기출급림상가조작성;간공능손해상관지표、외주혈림파세포분류、중추신경계통화호흡계통수루표현대HLH적진단구유삼고개치.
Objective To investigate the incidence,clinical symptoms,signs and laboratory features of childhood hemophagocytic lymphohistiocytosis (HLH) in China.Methods A retrospective study was performed on 217 pediatric patients with HLH who were admitted to Children' s Hospital of Chongqing Medical University from January 2006 to April 2013.All patients' medical records were reviewed and analyzed.Results The Male to female ratio was 1.11 ∶ 1.The median onset age was 3 years and 5 months old (range of 6 months old to 16 years and 9 months old),and the age of onset peaked between 1-5 years old (61.3%).The most common causes of HLH was infection,especially Epstein-Barr virus-associated HLH (71.0%).Other causes included malignant hemophagocytic syndrome (MAHS),macrophage activation syndrome (MAS) and so on.The outstanding clinical manifestations including persistent fever (100.0%),hepatomegaly (92.6%),splenomegaly (88.4%),and more than half of cases with central nervous system involvement and pulmonary manifestations.Laboratory data indicated that the most prominent abnormality was elevated ferritin (98.0%),and the others were hemophagocytosis in bone marrow (90.7%) and coagulation abnormalities (76.5%).Abnormal lymphocytes classification is very common in HLH.Conclusions HLH is a heterogeneous disease,with a variety of the etiology and clinical manifestations.HLH-2004 diagnostic protocol had theoretical basis and clinical operability.The hepatitis damages related indicators,lymphocytes classification,central nervous system involvement and pulmonary performance can be used as reference value for HLH diagnosis.