engleski

Castleman disease presented with prolonged fever of unknown origin

Fever is the most common cause for which children and their caregivers seek medical attention. If accompanied by elevated blood inflammatory markers, infectious diseases are suspected. However, if microbiological findings are negative, and if there is no response to antimicrobial therapy, more careful and comprehensive evaluation is required. Amongst many disorders, one of the rare but often misdiagnosed cause is Castleman disease (CD). This is a heterogeneous group of lymphoproliferative disorders with similar histopathologic features divided into three types. Unicentric CD involves one or more enlarged lymph node(s) in a single region of a body while multicentric Castleman disease involves multiple regions of lymphadenopathy and is further subclassified in HHV-8 positive and HHV-8 negative/idiopathic type. We present a case of a 4.5-year-old boy who came to our pediatric emergency department with a history of intermittent fever for almost a month, cough, skin rash with spontaneous regression, fatigue, periodic leg pain, night sweats, and weight loss. The physical examination showed few mildly enlarged cervical lymph nodes, pale skin, antalgic gait and fever. Routine blood tests revealed elevated C- reactive protein level and erythrocyte sedimentation rate, anemia, and thrombocytosis. He was admitted to a Pediatric Rheumatology department for evaluation. Other laboratory tests showed elevated fibrinogen, ferritin and d-dimer levels, with high C3, IL-6 and TNF-alpha levels. Microbial cultures (blood cultures, nasopharyngeal and throat swab) were sterile, and serology for Cytomegalovirus, Epstein-Barr virus, Human parvovirus B19 and Bartonella henselae were negative, while serology for Mycoplasma pneumoniae (IgM and IgG) was positive. Azithromycin was prescribed, but fever and elevated inflammatory blood markers persisted and other procedures were performed to rule out rheumatic disease. Radiogram and bone scintigraphy were unremarkable, but an ultrasound revealed a retroperitoneal mass. MRI was performed, and retroperitoneal mass measuring 3x2.5 cm, arising from the pancreas and suspected to be a neuroendocrine tumor or enlarged lymph node, along with hepatosplenomegaly was described. FDG-positron emission tomography (PET)/CT showed a mass near the pancreas with moderately increased FDG uptake. Complete surgical resection of the mass was performed, and the histopathological evaluation revealed lymphoid hyperplasia consistent with the hyaline vascular type of Castleman disease. An immunohistochemical study excluded HHV-8 positivity. After the surgical procedure, the patient recovered completely. On follow up visits he was without fever or other symptoms suggestive of the disease, with normal laboratory findings. CD with systemic manifestations is rare in children emphasizing the need for thorough evaluation of unexplained prolonged symptoms.

Castleman disease ; fever of unkown origin ; inflammation ; pediatric

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