Prolonged fever, pancytopenia, and splenomegaly - is it sarcoidosis?

CASE REPORTS

Prolonged fever, pancytopenia, and splenomegaly - is it sarcoidosis?

Carolina Amaro Gonçalves¹, Ana Dias Curado¹, Catarina Salgado², Isabel Esteves³, Filipa Oliveira Ramos⁴, Anabela Ferrão².

¹Pediatric Department, Centro Hospitalar e Universitário Lisboa Norte - Hospital de Santa Maria, Lisbon, Portugal,
²Pediatric Haematology Unit, Pediatric Department, Centro Hospitalar e Universitário Lisboa Norte - Hospital de Santa Maria, Lisbon, Portugal,
³Pediatric Infectious Disease and Immunodeficiency Unit , Department of Pediatrics, Centro Hospitalar e Universitário Lisboa Norte - Hospital de Santa Maria, Lisbon, Portugal,
⁴Pediatric Rheumatology Unit, Centro Hospitalar e Universitário Lisboa Norte - Hospital de Santa Maria, Lisbon, Portugal.

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Abstract
Childhood Sarcoidosis is a chronic multisystem pinflammatory disease of unknown etiology. We report a case of a 9-year-old female who presented with nasal bleeding. She started with fever that persisted for 24 days associated with progressive splenomegaly, pancytopenia, and generalized lymphadenopathy. An extensive investigation was performed excluding infectious, neoplastic, autoimmune and immunodeficiency causes. Histological examination of a lymph node revealed multiple non-caseous granulomas, which together with the elevation of ACE and multiorgan involvement, suggested the diagnosis of sarcoidosis. She started oral prednisolone and after 4-month of follow-up there was a significant clinical and analytic improvement. This case represents a challenging diagnosis of an uncommon pediatric disease that should be considered in the differential diagnosis of a multisystemic disease with constitutional symptoms even in the absence of pulmonary involvement.

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