Achakzai Humaira, Ghutai Gul, Khalil Daud, Amin Qazi Kamran, Ullah Waqar
Internal Medicine, Rehman Medical Institute, Peshawar, PAK.
General Medicine, Rehman Medical Institute, Peshawar, PAK.
Cureus. 2023 Jun 30;15(6):e41182. doi: 10.7759/cureus.41182. eCollection 2023 Jun.
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease characterized by excessive immune response activation. Numerous conditions, including infectious etiologies, are implicated in its development. We report the case of a 16-year-old girl with HLH associated with polyserositis and infection. A 16-year-old girl presented with a high-grade fever and abdominal pain that had been ongoing for 20 days. She had been treated for malaria at a local hospital but was referred to our hospital due to the worsening of her condition. On examination, she was found to have an enlarged liver and spleen, pale skin, and hypotension, with bilateral basal crackles on chest examination. Her blood profile revealed pancytopenia, elevated C-reactive protein, and a deranged coagulation profile. Peripheral smears showed anisocytosis, microcytes, hypochromia in RBCs, and a few platelet clumps. A bone marrow biopsy revealed increased megakaryocytes and hemophagocytes. Ultrasound and computed tomography of the abdomen and pelvis showed hepatosplenomegaly, pericholecystic edema, mild ascites, and long-segment diffuse colonic wall thickening, suggesting pancolitis. Blood culture revealed , which is rarely associated with HLH. The patient was started on the HLH-2004 protocol and showed improvement on the fourth day of initiating therapy, but due to a delayed diagnosis, the patient collapsed on the sixth day of admission. HLH is a rare but life-threatening disease with various underlying causes. The diagnosis of HLH is challenging, and early diagnosis and prompt treatment are crucial for a better prognosis. The association between HLH and infection is rare, and this case highlights the importance of considering unusual etiologies in HLH. Clinicians should be vigilant about this association, especially in endemic regions, to ensure early diagnosis and prompt treatment.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但可能致命的疾病，其特征为过度的免疫反应激活。许多情况，包括感染性病因，都与其发病有关。我们报告一例16岁患HLH并伴有多浆膜炎和感染的女孩病例。一名16岁女孩出现持续20天的高热和腹痛。她曾在当地医院接受疟疾治疗，但因病情恶化被转诊至我院。检查发现她肝脏和脾脏肿大、皮肤苍白、低血压，胸部检查有双侧肺底湿啰音。她的血液检查显示全血细胞减少、C反应蛋白升高以及凝血指标紊乱。外周血涂片显示红细胞大小不均、小红细胞、低色素性红细胞以及少量血小板聚集。骨髓活检显示巨核细胞和噬血细胞增多。腹部和盆腔超声及计算机断层扫描显示肝脾肿大、胆囊周围水肿、轻度腹水以及长节段弥漫性结肠壁增厚，提示全结肠炎。血培养发现……，这与HLH很少相关。患者开始采用HLH - 2004方案治疗，在开始治疗的第四天病情有所改善，但由于诊断延迟，患者在入院第六天病情恶化。HLH是一种罕见但危及生命的疾病，有多种潜在病因。HLH的诊断具有挑战性，早期诊断和及时治疗对于改善预后至关重要。HLH与……感染之间的关联罕见，该病例凸显了在HLH中考虑不寻常病因的重要性。临床医生对此关联应保持警惕，尤其是在流行地区，以确保早期诊断和及时治疗。
