title: Extramedullary hematopoiesis of the liver in a child with sickle cell disease: A rare complication.
abstract: We present the case of a 7-year-old Cameroonian girl with sickle cell disease (SCD) who presented with progressive abdominal distension, fever, severe anemia, respiratory distress, and fatigue. Abdominal ultrasound showed a 15.3 cm × 11.5 cm × 15.5 cm solid echogenic mass within the left lobe of the liver. Fine-needle aspiration showed features of extramedullary hematopoiesis (EMH). Despite transfusions, antibiotics, and initiation of hydroxyurea the patient died of respiratory failure during the hospital stay. There is a paucity of information on EMH in the pediatric sickle cell population, especially from resource-limited settings such as western Africa. EMH, however, is a known complication of SCD and should be considered in patients presenting with mass lesions in the setting of chronic anemia. With limited therapeutic interventions for EMH, including radiation and hydroxyurea, the emphasis should be on improving overall treatment of patients with chronic and untreated hemolytic anemia, especially in low-income countries.
authors.affiliation: Mbingo Baptist Hospital, Mbingo, Cameroon.
authors.forename: Angela
authors.initials: A
authors.lastname: Barrier
authors.affiliation: Mbingo Baptist Hospital, Mbingo, Cameroon.
authors.forename: Simo
authors.initials: S
authors.lastname: Willy
authors.affiliation: Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, Texas, USA.
authors.forename: Jeremy S
authors.initials: JS
authors.lastname: Slone
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D044963:Biopsy, Fine-Needle; D002648:Child; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D008099:Liver / Q000473:pathology*
keywords: extramedullary hematopoiesis; hydroxyurea; resource-limited country; severe anemia; sickle cell disease