Background Castleman’s disease (CD) is a heterogeneous group of lymphoproliferative disorders associated in a subset of cases with HIV and human herpes virus 8 (HHV-8). CD comprises at least two distinct diseases (unicentric and multicentric) with different approaches. There are currently no licensed drugs for the management of CD in paediatric patients.

Purpose To describe a paediatric case of CD in which an antagonist of the interleukin (IL)-6 receptor (tocilizumab) was successfully used.

Material and methods A retrospective case report and literature search related to the treatment of CD was carried. The information was obtained from electronic medical records, PubMed and Uptodate.

Results An 11-year-old boy (weight 32.2 kg, p3–10) was admitted to the paediatric ward for the study of asthenia, anorexia and body weight loss of 5 months’ duration. Remarkable laboratory test values at that time included haemoglobin 9.8 mg/dL, MCV 76.2 fL, CRP 65 mg/L, ESR 90 mm and transferrin saturation 7%. His renal and liver function tests were within normal limits. During the study of the toxic syndrome, screening for several infections, including Epstein–Barr virus, cytomegalovirus, tuberculosis, HHV-8 and HIV had negative results. Additionally, an alteration in IL-6 (54 pg/mL) was detected. PET-SCAN showed multiple adenopathies suggesting a lymphoproliferative syndrome. The adenopathy biopsy was not conclusive, but having excluded other pathologies, the case was considered multicentric CD based on the symptoms and analytical results. Given the age of the patient, treatment with tocilizumab 8 mg/kg/month was initiated despite not being licensed for its use in CD. After five doses, the patient showed clinical improvement but acute phase reactants were still high and symptoms reappeared a few days before the next dose. Consequently, the frequency of the treatment was reduced to 8 mg/kg/21 days. After a year of treatment, the patient showed a great response with standardisation of acute phase reactants (CRP 0.2 mg/L, ESR 6 mm) and body weight gain of 22 kg (current weight 54 kg).

Conclusion In this case of a paediatric patient with CD, the use of tocilizumab (off-label use) was shown to be safe and effective when reducing the intervals of administration to 21 days. Nevertheless, more studies are needed to demonstrate its efficacy and safety profile.

No conflict of interest