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Sickle Cell Hepatic Vaso-Occlusive Crisis: A Case Report
1Department of Hematology and Oncology, San Fernando General Hospital, San Fernando, Trinidad and Tobago, health.gov.tt.
Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a point mutation in the β-globin gene, resulting in hemoglobin S polymerization under hypoxic conditions. This process leads to erythrocyte dehydration, impaired deformability, hemolysis, and recurrent vaso-occlusion, which may culminate in acute painful crises and progressive organ damage. Hepatic involvement is a recognized but underreported complication of SCD and can range from mild biochemical abnormalities to life-threatening hepatic vaso-occlusive crisis (VOC). This case report presents a 25-year-old woman, with homozygous sickle cell anemia who developed a rapidly progressive hepatic VOC following a mild respiratory illness. Her clinical course was marked by acute abdominal pain, severe transaminitis, cholestasis, escalating transfusion requirements, and radiologic evidence of hepatomegaly. Clinical and biochemical improvement was observed following a multimodal management approach that included supportive care, corticosteroids, blood transfusion, and the addition of intravenous metronidazole. This case highlights hepatic VOC as a serious manifestation of SCD and explores the potential role of metronidazole as an adjunctive therapy, given its immunomodulatory and anti-inflammatory properties. Further investigation is warranted to clarify its therapeutic role in sickle cell-related vaso-occlusive complications.
Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a point mutation in the β-globin gene, resulting in hemoglobin S polymerization under hypoxic conditions. This process leads to erythrocyte dehydration, impaired deformability, hemolysis, and recurrent vaso-occlusion, which may culminate in acute painful crises and progressive organ damage. Hepatic involvement is a recognized but underreported complication of SCD and can range from mild biochemical abnormalities to life-threatening hepatic vaso-occlusive crisis (VOC). This case report presents a 25-year-old woman, with homozygous sickle cell anemia who developed a rapidly progressive hepatic VOC following a mild respiratory illness. Her clinical course was marked by acute abdominal pain, severe transaminitis, cholestasis, escalating transfusion requirements, and radiologic evidence of hepatomegaly. Clinical and biochemical improvement was observed following a multimodal management approach that included supportive care, corticosteroids, blood transfusion, and the addition of intravenous metronidazole. This case highlights hepatic VOC as a serious manifestation of SCD and explores the potential role of metronidazole as an adjunctive therapy, given its immunomodulatory and anti-inflammatory properties. Further investigation is warranted to clarify its therapeutic role in sickle cell-related vaso-occlusive complications.
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