Multicentric Castleman Disease Initially Diagnosed as Disseminated Tuberculosis in an Adolescent Boy in Sokoto, Nigeria.

BACKGROUND: Castleman disease (CD) is a rare lymphoproliferative clinicopathological entity characterized by non-neoplastic lymph node hypertrophy with distinct histopathology. It has a wide spectrum of presentation ranging from benign localized unicentric CD to idiopathic multicentric CD and human herpes virus (HHV) associated disease which are potentially life-threatening. Treatment includes steroids, chemotherapy and immunotherapy. It has not been reported in Nigerian children.

CASE SUMMARY: A 13-year-old boy presented with fever and body swelling of four weeks, cough of one week and difficulty in breathing of three days duration. On examination he was acute on chronically ill-looking, in respiratory distress, with significant generalized lymphadenopathy, bilateral pedal oedema and hepatosplenomegaly. Disseminated tuberculosis (TB) and lymphoproliferative disease were considered. He had leucocytosis with predominant neutrophils, elevated erythrocyte sedimentation rate and inflammatory cells on bone marrow cytology. GeneXpert and retroviral screen were negative. Chest X-ray showed mediastinal shadows. Abdominal ultrasound scan showed hypoechoic lobulated masses with branching margins. Lymph node biopsy showed encapsulated lymphoid tissue composed of proliferating large-sized atretic lymphoid follicles with expanded mantle zones and lymphocytes arranged in strata giving an onion-skin appearance characteristic of CD. HHV was not excluded. Caregivers were counselled on chemotherapy but defaulted.

CONCLUSION: CD is rare but presents similarly to disseminated TB or lymphoma clinically. Efforts and provisions should be made for early histological diagnosis in suspected cases.