Diagnostic Approach to Fever with Hepatosplenomegaly in Children

Initial Evaluation

The American Academy of Pediatrics recommends a complete blood count with differential to assess for cytopenias, thrombocytopenia, and abnormal cells in children presenting with fever and hepatosplenomegaly 1, 2
A comprehensive metabolic panel including liver function tests is essential in the initial evaluation of children with fever and hepatosplenomegaly 2
Inflammatory markers such as CRP and procalcitonin should be measured to support the diagnosis of conditions like HLH 2

Abdominal ultrasound with Doppler is recommended to assess liver and spleen morphology, detect focal lesions, and evaluate for portal hypertension in children with fever and hepatosplenomegaly 1
Chest radiograph is indicated if respiratory symptoms are present in children with fever and hepatosplenomegaly 2
Bone marrow biopsy may be necessary to identify hemophagocytosis, storage cells, or malignant infiltration in children with fever and hepatosplenomegaly 1

Lysosomal storage diseases such as acid sphingomyelinase deficiency (ASMD/Niemann-Pick disease) should be considered in children with fever and hepatosplenomegaly, particularly if splenomegaly is massive 1
Genetic testing for storage disorders (SMPD1 gene) is recommended if clinical suspicion is high 1

Urinary tract infections are common in febrile children but typically do not cause hepatosplenomegaly, according to the American Academy of Pediatrics 3, 4