PDF(Pubmed)
Abstract：
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially life-threatening syndrome characterized by excessive immune activation and tissue inflammation. This case report describes the early diagnosis of HLH in an adult patient who initially presented with a febrile syndrome associated with low back pain. The patient, a 33-year-old male, exhibited bicytopenia, hepatosplenomegaly, and hyperferritinemia without a previous diagnosis of sickle cell disease (SCD). Diagnostic challenges arose due to the overlapping clinical manifestations of SCD and HLH and their uncommon association. However, timely recognition and intervention were achieved through comprehensive diagnostic evaluations, including a bone marrow biopsy. The patient was promptly started on an appropriate therapeutic regimen, which led to significant clinical improvement. This case underscores the importance of considering HLH in the differential diagnosis of adults presenting with hematologic abnormalities and systemic inflammation. Early diagnosis and treatment are critical to improving outcomes for patients with this complex and severe disorder.
摘要：
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但可能危及生命的综合征，其特征是过度的免疫激活和组织炎症。此病例报告描述了成年患者对HLH的早期诊断，该患者最初患有与腰背痛相关的发热综合征。病人,一个33岁的男性,表现出双细胞减少症，肝脾肿大,和先前未诊断为镰状细胞病（SCD）的高铁蛋白血症。由于SCD和HLH的重叠临床表现及其不常见的关联，出现了诊断挑战。然而,通过全面的诊断评估，实现了及时的识别和干预，包括骨髓活检.患者迅速开始接受适当的治疗方案，这导致了显著的临床改善。该病例强调了在表现为血液学异常和全身性炎症的成人的鉴别诊断中考虑HLH的重要性。早期诊断和治疗对于改善这种复杂和严重疾病患者的预后至关重要。
