小儿噬血细胞性淋巴组织细胞增生症(pHLH)是一种潜在的威胁生命的疾病,具有严重的诊断和治疗困难。这项研究的目的是描述临床表现,诊断挑战,以及Mukalla医院评估的儿童噬血细胞淋巴组织细胞增生症(HLH)的结果,也门。回顾性分析了2010年1月至2022年5月期间收治的20例HLH患者的病历数据。演示时的中位年龄为3.5±5.1岁。男性:女性比例为1:1。转诊至医院的中位时间为30±64天。95%的病例最常见的临床表现是发热和面色苍白,脾肿大(85%)。肝肿大,胸部,肾脏和神经表现被检测到80%,45%,15%和20%的病例,分别。在60%的病例中检测到骨髓吞噬作用。16例患者符合HLH诊断标准,11例患者(55%)接受HLH2004方案.在20名患者中,3名(15%)患者还活着。14名患者死亡,总死亡率为82.35%。所有死亡率都是由于HLH疾病伴多器官衰竭。在治疗期间或完全康复后,有五名患者出现复发。pHLH是具有高死亡率的具有挑战性的紧急情况。高度的临床怀疑对于早期发现和干预以改善预后至关重要。
Paediatric haemophagocytic lymphohistiocytosis (pHLH) is a potentially life-threatening condition with significant diagnostic and therapeutic difficulties. The purpose of this study was to describe the clinical presentation, the diagnostic challenges, and the outcomes of haemophagocytic lymphohistiocytosis (HLH) in children assessed at Mukalla Hospital, Yemen. Data from 20 medical records of HLH patients admitted between January 2010 and May 2022 were retrospectively analysed. The median age at presentation was 3.5 ± 5.1 years. Male: female ratio was 1:1. The median time for referral to the hospital was 30 ± 64 days. The most common clinical manifestations were fever and pallor in 95% of cases, and splenomegaly (85%). Hepatomegaly, chest, renal and neurological manifestations were detected in 80%, 45%, 15% and 20% of cases, respectively. Bone marrow
haemophagocytosis was detected in 60% of cases. Sixteen patients fulfilled the HLH diagnostic criteria, and 11 patients (55%) received the HLH 2004 protocol. Out of the 20 patients, three (15%) patients are alive. Fourteen patients died, with overall mortality of 82.35%. All mortalities were due to HLH disease with multi-organ failure. Relapse was noticed in five patients either during treatment or after full recovery. pHLH is a challenging emergency with a high mortality rate. High clinical suspicion is essential for early detection and intervention to improve the prognosis.