Karapinar Bülent, Yilmaz Deniz, Balkan Can, Akin Mehmet, Ay Yilmaz, Kvakli Kaan
Pediatric Intensive Care Unit, Faculty of Medicine, Ege University, Izmir, Turkey.
Pediatr Crit Care Med. 2009 May;10(3):285-90. doi: 10.1097/PCC.0b013e318198868b.
To report our experience in children with primary or secondary hemophagocytic lymphohistiocytosis (HLH) presented with multiple organ dysfunction syndrome (MODS) in pediatric intensive care unit (PICU).
The records of patients with a diagnosis of HLH and MODS between January 2005 and January 2008 were reviewed. The patients' characteristics, treatment modalities, and outcomes were assessed.
PICU of Ege University Hospital.
PATIENTS/SUBJECTS: Twelve children who were hospitalized in the PICU met the diagnostic criteria for HLH, and presented with MODS were entered into the study.
The median age of the patients was 3 years (range, 2 months-15.5 years). Six patients had a history of parental consanguinity and two had an affected sibling. Five of the patients were classified as primary HLH. All of the patients had hepatosplenomegaly, elevated ferritin levels, hypofibrinogenemia, anemia, thrombocytopenia, and hemophagocytosis in bone marrow examination at presentation. The median Pediatric Logistic Organ Dysfunction score of the patients at onset was 51 (range, 12-62). Four patients had six, four had five, two had four, and the remaining two had three organ dysfunctions. Organ dysfunction, other than hematologic dysfunction which was present in all patients, was most commonly seen in hepatic (n = 11, 91.7%), respiratory (n = 11, 91.7%), and cardiovascular systems (n = 10, 83.3%). Although nine patients showed neurologic dysfunction including convulsion and coma, renal failure was detected in five patients. Eleven patients were supported with mechanical ventilation and four patients required hemodialysis. Eight patients were treated according to the HLH 2004 treatment protocol, consisting of cyclosporine A, etoposide, and dexamethasone. The remaining four patients received only intravenous immunoglobulin and supportive treatment. Seven of the patients died.
HLH is a frequently lethal disease and with a clinical presentation similar to severe sepsis, MODS, disseminated intravascular coagulation, or septic shock, which are frequent diagnoses in the PICU. In the PICU, HLH should be considered in the case of prolonged fever, splenomegaly, cytopenia, and MODS. It is important for pediatricians and particularly pediatric intensivists to know the diagnostic criteria and possible clinical presentations of HLH so treatment is initiated promptly.
报告我们在儿科重症监护病房（PICU）中对原发性或继发性噬血细胞性淋巴组织细胞增生症（HLH）伴多器官功能障碍综合征（MODS）患儿的治疗经验。
回顾2005年1月至2008年1月期间诊断为HLH和MODS患者的病历。评估患者的特征、治疗方式和预后。
伊兹密尔大学医院PICU。
患者/受试者：12名入住PICU且符合HLH诊断标准并伴有MODS的儿童纳入本研究。
患者的中位年龄为3岁（范围2个月至15.5岁）。6名患者有父母近亲结婚史，2名患者有患病同胞。5名患者被归类为原发性HLH。所有患者在就诊时均有肝脾肿大、铁蛋白水平升高、纤维蛋白原血症、贫血、血小板减少，骨髓检查可见噬血细胞现象。患者发病时的中位儿科逻辑器官功能障碍评分为51分（范围12至62分）。4名患者有6项器官功能障碍，4名有5项，2名有4项，其余2名有3项器官功能障碍。除所有患者均存在的血液系统功能障碍外，器官功能障碍最常见于肝脏（n = 11，91.7%）、呼吸系统（n = 11，91.7%）和心血管系统（n = 10，83.3%）。虽然9名患者出现包括惊厥和昏迷在内的神经功能障碍，但5名患者检测到肾衰竭。11名患者接受机械通气支持，4名患者需要血液透析。8名患者按照HLH 2004治疗方案进行治疗，该方案包括环孢素A、依托泊苷和地塞米松。其余4名患者仅接受静脉注射免疫球蛋白和支持治疗。7名患者死亡。
HLH是一种常见的致死性疾病，临床表现类似于严重脓毒症、MODS、弥散性血管内凝血或感染性休克，这些是PICU中常见的诊断。在PICU中，对于长期发热、脾肿大、血细胞减少和MODS的病例应考虑HLH。儿科医生尤其是儿科重症监护医生了解HLH的诊断标准和可能的临床表现以便及时开始治疗非常重要。
