Hemophagocytic Lymphohistiocytosis Presenting as Neonatal Cholestasis: A Case Report
Publish place: The Iranian Journal of Neonatology، Vol: 14، Issue: 2
Publish Year: 1402
نوع سند: مقاله ژورنالی
زبان: English
View: 140
This Paper With 5 Page And PDF Format Ready To Download
- Certificate
- من نویسنده این مقاله هستم
استخراج به نرم افزارهای پژوهشی:
شناسه ملی سند علمی:
JR_IRJN-14-2_009
تاریخ نمایه سازی: 10 تیر 1402
Abstract:
Background:. Hemophagocytic lymphohistosis (HLH) is a life-threatening clinical syndrome that involves liver dysfunction and can range from mild dysfunction to severe fulminant insufficiency. Cholestasis, which is a frequent finding in many severe newborn illnesses, may also be a symptom of HLH. Therefore, HLH should be considered in the differential diagnosis of all cholestatic patients with cytopenia. In this report, we identified a case of HLH with cholestasis. The patient met at least seven out of the eight requirements of the HLH-۲۰۰۴ criteria. The infant had a stable fever, jaundice (conjugated hyperbilirubinemia), cytopenia, elevated liver enzymes, high ferritin, low fibrinogen, and triglyceride. Although urine, blood, and cerebrospinal fluid (CSF) culture tests were negative, CSF analysis revealed ۸۰ WBCs, including ۶۸% polymorphonuclear neutrophils (PMNs), more than ۱۰۰۰۰۰ RBCs, ۷۱ mg/dL sugar, ۸۲ mg/dL protein and ۱۰۲ U/L lactate dehydrogenase. Coagulation tests and TORCH screen were normal. We confirmed the diagnosis of HLH with a bone marrow aspiration test and started treatment with dexamethasone. An intravenous dose of dexamethasone (۴ mg) was administered, followed by ۱.۵ mg daily with pressure control, which stopped the fever after ۲۴ hours. Case report: The patient was a ۲۱-day-old infant with a birth weight of ۳۴۵۰ g. He developed fever and jaundice ۱۰ days after a normal delivery, and he was referred to Sarakhs Hospital when he was ۱۴ days old and hospitalized for ۳ days. Later, he was sent to Ghaem Hospital and admitted to the Neonatal Intensive Care Unit. The infant had a stable fever, jaundice (conjugated hyperbilirubinemia), cytopenia, elevated liver enzymes, high ferritin, low fibrinogen, and triglyceride. According to his mother, there was no history of any problems from birth, and the baby's jaundice started after the first week. Examinations showed abdominal distention and hepatosplenomegaly. Due to neonatal cholestasis and fever, he underwent a complete sepsis workup with vancomycin and cefotaxime. Although urine, blood, and cerebrospinal fluid (CSF) culture tests were negative, CSF analysis revealed ۸۰ WBCs, including ۶۸% polymorphonuclear neutrophils, more than ۱۰۰۰۰۰ RBCs, ۷۱ mg/dL sugar, ۸۲ mg/dL protein, and ۱۰۲ U/L lactate dehydrogenase. Coagulation tests and TORCH screen were normal. We confirmed the diagnosis of hemophagocytic lymphohistiocytosis (HLH) with a bone marrow aspiration test and started treatment with dexamethasone. An intravenous dose of dexamethasone (۴ mg) was administered, followed by ۱.۵ mg daily with pressure control, which stopped the fever after ۲۴ hours. Conclusion:HLH is uncommon in the neonatal stage, and aberrant clinical and laboratory findings suggestive of HLH can be found in a variety of conditions. The severity of this condition makes it crucial to get a diagnosis as soon as possible. In the presence of additional variables, such as cytopenia and hyperferritinemia, HLH should be considered in the differential diagnosis of cholestasis in a neonate.
Keywords:
Authors
Raheleh Faramarzi
Neonatal Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
Azadeh Darabi
Department of Pediatrics, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
Seyed Morteza Rasti Sani
Department of Pediatrics, School of Medicine, Heshmatie Hospital, Sabzevar University of Medical Sciences, Sabzevar, Iran
Hassan Boskabadi
Department of Pediatrics, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
Javad Mohamadi Taze Abadi
Department of diagnostic Radiologist Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
مراجع و منابع این Paper:
لیست زیر مراجع و منابع استفاده شده در این Paper را نمایش می دهد. این مراجع به صورت کاملا ماشینی و بر اساس هوش مصنوعی استخراج شده اند و لذا ممکن است دارای اشکالاتی باشند که به مرور زمان دقت استخراج این محتوا افزایش می یابد. مراجعی که مقالات مربوط به آنها در سیویلیکا نمایه شده و پیدا شده اند، به خود Paper لینک شده اند :