Pediatric Multicentric Castleman Disease Presented with Spontaneous Tumor Lysis Syndrome and Acute Kidney Injury

Case Report

Ann Hematol Oncol. 2022; 9(2): 1395.

Pediatric Multicentric Castleman Disease Presented with Spontaneous Tumor Lysis Syndrome and Acute Kidney Injury

Alqahtani MF¹*, Liedel JL¹, Pott T¹, Miles L³, Douglas D², Nagasubramanian R² and Kahana MD¹

¹Department of Pediatric Critical Care, Nemours Children’s Hospital, Orlando, FL; USA

²Department of Pediatric Hematology & Oncology, Nemours Children’s Hospital, Orlando, FL; USA

³Department of Pathology, Nemours Children’s Hospital, Orlando, FL; USA

*Corresponding author: Alqahtani MF, Department of Pediatric Critical Care, Nemours Children’s Hospital, 6535 Nemours Pkwy, Orlando, Florida, USA

Received: May 11, 2022; Accepted: June 20, 2022; Published: June 27, 2022


Castleman disease (CD) is a rare lymphoproliferative disorder that is uncommon in the pediatric population. Tumor lysis syndrome (TLS) is a rare presentation in CD even after chemotherapy. There is only one case report for pediatric patients with CD where treatment was complicated by TLS. In this case, we report spontaneous TLS and acute kidney injury in a pediatric patient with idiopathic multicentric Castleman disease.

Here, we present the case of a 14 year old male with no significant past medical history presented with TLS and AKI (Acute kidney injury). He was found to have idiopathic multicentric CD (iMCD) based on lymph node biopsy. The child was started on Siltuximab and high systemic steroid therapy with significant improvement. A short term continuous renal replacement therapy was required for fluid overload. The patient was discharged home within a month with normal renal function on maintenance therapy.

Spontaneous TLS is a rare but significant complication that should be considered in adults and pediatric patients with MCD. Careful monitoring and immediate intervention with supportive care are essential even before the initiation of therapy.

Keywords: Idiopathic Multicentric Castleman Disease; Castleman Disease; Tumor Lysis Syndrome; Pediatric Critical Care; Continuous Renal Replacement Therapy; Siltuximab


AKI: Acute Kidney Injury; CD: Castleman Disease; CT: Computerized Tomography; CRRT: Continuous Renal Replacement Therapy; HHV-8: Human Herpesvirus-8; HIV: Human Immunodeficiency Virus; iMCD: Idiopathic Multicentric Castleman Disease; IL-6: Interleukin-6; MCD: Multicentric Castleman Disease; POEMS: Plasma Cell Neoplasm Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, and Skin Changes Syndrome; PICU: Pediatric Intensive Care Unit; TLS: Tumor Lysis Syndrome; UCD: Unicentric Castleman Disease.

Case Presentation

A 14 year old male was referred from an outside hospital with a chief complaint of abdominal pain and distention for two weeks. Past medical history was significant for attention deficit hyperactive disorder on Risperidone, Methylphenidate, and Guanfacine. Vital signs on admission were unremarkable; blood pressure 117/66 mmHg, temperature 37.2°C, pulse rate 76 Beats/min, and respiration rate 21 Breaths/min. His physical examination was significant for abdominal distension, mild generalized tenderness without guarding or rebound, and no appreciable hepatomegalyor splenomegaly. Two mobile, non-tender lymph nodes, one in the right deep cervical area and the other in the right axillary lymph node, both approximately one centimeter, were palpable.

Initial laboratory studies were performed at admission revealed thrombocytosis and evidence of acute kidney injury (AKI) and TLS (Table 1). The patient was admitted to the pediatric intensive care unit (PICU) for presumed tumor lysis secondary to an unknown malignancy complicated by AKI. Treatment for TLS was begun, and additional evaluation for malignancy was started. Chest X-ray revealed only a small pleural effusion. The abdominal ultrasound was significant for hepatosplenomegaly and moderate ascites (Table 2).