Successful outcome in a case of idiopathic multicentric Castleman disease with atypical lymphadenopathy and kidney injury: Diagnostic challenges and treatment approach-Case report

Abstract

Idiopathic multicentric Castleman disease is a rare and complex disease characterized by systemic inflammation, lymphadenopathy, and multiorgan involvement. This case report presents a 66-year-old Chinese man with idiopathic multicenter Castleman disease without significant lymphadenopathy and challenging diagnosis. Patients present with fever, fatigue, loss of appetite, weight loss, and acute kidney injury. Initially, a urinary tract infection was suspected, but despite anti-infective treatment, the patient's symptoms persisted. Lymph node biopsy, although there is no significant lymphadenopathy, confirms idiopathic multicenter Castleman disease. Treatment includes thalidomide, cyclophosphamide, and dexamethasone, as well as supportive measures and infection control. After 8 months of follow-up, the patient's clinical symptoms, inflammatory markers and renal function were significantly improved, and there was no symptomatic recurrence. This case underscores the importance of considering idiopathic multicenter Castleman's disease in patients with persistent fever and systemic inflammation, even in the absence of significant lymphadenopathy. Early identification and accurate diagnosis of idiopathic multicenter Castleman's disease can lead to the initiation of targeted therapy strategies that ultimately yield favorable outcomes.

Keywords: Idiopathic multicentric Castleman disease; diagnosis; interleukin-6; systemic inflammation; treatment.

Figure 1.

Changes in levels of WBC and HGB of the patient during the observational period. WBC: white blood cell count, normal range: 3.5–9.5 × 10⁹/L; HGB: hemoglobin, normal range: 130–175 g/L.

Figure 2.

Changes in levels of CRP and IL-6 of the patient during the observational period. IL-6: serum interleukin-6, normal range: 0-pg/ml; CRP: serum C-reactive protein, normal range: 0–8 mg/L.

Figure 3.

Changes in levels of Scr and ALB of the patient during the observational period. Scr: serum creatinine, normal range: 57–111 umol/L; ALB: serum albumin, normal range: 40–55 g/L.

Figure 4.

The chest CT scan of the patient at admission (December 6, 2021) showed the presence of bilateral pneumonia with mild interstitial pulmonary edema and bilateral pleural effusion (a). The bilateral ground-glass opacities and pleural effusion had been absorbed after treatment (December 24, 2021) (b).

Figure 5.

Histological characterization of the left inguinal lymph node of the patient revealed the following findings. (a) The general view of the lymph node. The lymph node structure was partially preserved, but extensive fibrosis was observed in most areas. The lymph sinuses were open, and there was partial lymph follicle atrophy. Scattered plasma cells were present in the interfollicular regions, and occasional infiltration of eosinophils was observed at low magnification (b) and high magnification (c). (d) Immunohistochemical staining of CD138, which is specific to plasma cells.