Veerakul Gavivann, Sanpakit Kleebsabai, Tanphaichitr Voravarn S, Mahasandana Chularatana, Jirarattanasopa Nongnapa
J Med Assoc Thai. 2002 Aug;85 Suppl 2:S530-41.
Fifty-two pediatric patients were diagnosed with secondary hemophagocytic lymphohistiocytosis (HLH) at the Department of Pediatrics, Siriraj Hospital between 1989 and 1998. Of these, 15 were infection-associated (IAHS), 25 were malignancy-associated (MAHS) and 12 were idiopathic HLH. Causative organisms for IAHS were Salmonella (3), Staphylococcus (2), enterobactor (2), dengue virus (3), malaria (2) and one each of Ebstein Barr virus (EBV), Serratia marcesens and Penicillium maneffei. Unlike those reported in adults and in the Western literature, 47 of 52 children in the present series were immunocompetent hosts. In addition, the proportion of MAHS was higher than expected (48.1%). Twenty-two of 25 MAHS presented with hemophagocytic syndrome and were subsequently found to have malignant diseases. Sixty per cent of MAHS (15 cases) were associated with non-Hodgkin's lymphoma (NHL), mainly T-cell. Other malignancies included acute leukemias (7) MDS (1), Langerhans cell histiocytosis (1) and histiocytic sarcoma (1). Treatment approaches were specific therapy for individuals with known causes. Supportive treatment with blood components transfusions, steroid, intravenous immunoglobulins (IVIG), and chemotherapeutic agents, mainly vinblastine and etoposides, were used in indicated cases. Of the 52 cases, 15 (28.8%) had a fatal outcome during the acute phase, and other 4 died of their subsequent malignant diseases. There was a statistically significant association between poorer prognosis and patients' age < 3 years (p= 0.004) or MAHS (p=0.005).
Secondary HLH is not uncommon in Thai children who are immunocompetent. Malignancies, particulary NHL, are highly suspicious especially for cases not responsive to conventional therapy. Poor prognostic factors are age less than 3 years and MAHS.
1989年至1998年间，诗里拉吉医院儿科诊断出52例儿童继发性噬血细胞性淋巴组织细胞增生症（HLH）。其中，15例为感染相关型（IAHS），25例为恶性肿瘤相关型（MAHS），12例为特发性HLH。IAHS的致病微生物包括沙门氏菌（3例）、葡萄球菌（2例）、肠杆菌（2例）、登革热病毒（3例）、疟疾（2例），以及爱泼斯坦-巴尔病毒（EBV）、粘质沙雷氏菌和马尔尼菲青霉菌各1例。与成人及西方文献报道不同的是，本系列52例儿童中有47例为免疫功能正常宿主。此外，MAHS的比例高于预期（48.1%）。25例MAHS中有22例出现噬血细胞综合征，随后被发现患有恶性疾病。60%的MAHS（15例）与非霍奇金淋巴瘤（NHL）相关，主要为T细胞型。其他恶性肿瘤包括急性白血病（7例）、骨髓增生异常综合征（1例）、朗格汉斯细胞组织细胞增生症（1例）和组织细胞肉瘤（1例）。治疗方法是针对已知病因的个体进行特异性治疗。在有指征的病例中，采用输血、类固醇、静脉注射免疫球蛋白（IVIG）和化疗药物（主要是长春碱和依托泊苷）等支持性治疗。52例中，15例（28.8%）在急性期死亡，另外4例死于随后的恶性疾病。预后较差与患者年龄<3岁（p = 0.004）或MAHS（p = 0.005）之间存在统计学显著关联。
继发性HLH在免疫功能正常的泰国儿童中并不少见。恶性肿瘤，尤其是NHL，高度可疑，特别是对于对传统治疗无反应的病例。预后不良因素为年龄小于3岁和MAHS。
