Idiopathic multicentric Castleman disease: a mysterious case of generalized lymphadenopathy

Nikhil Sebastian; Shoaib Nawaz P. N.; Ramya Ananthkrishnan; Kumanan J.; Archana Lakshmanan; Thirumalairaj Raja

doi:10.18203/2320-6012.ijrms20232455

Authors

Nikhil Sebastian Department of Medical Oncology, Apollo Cancer Centre, Chennai, Tamil Nadu, India
Shoaib Nawaz P. N. Department of Medical Oncology, Apollo Cancer Centre, Chennai, Tamil Nadu, India
Ramya Ananthkrishnan Department of Medical Oncology, Apollo Cancer Centre, Chennai, Tamil Nadu, India
Kumanan J. Department of Medical Oncology, Apollo Cancer Centre, Chennai, Tamil Nadu, India
Archana Lakshmanan Department of Pathology, Apollo Cancer Centre, Chennai, Tamil Nadu, India
Thirumalairaj Raja Department of Medical Oncology, Apollo Cancer Centre, Chennai, Tamil Nadu, India

DOI:

https://doi.org/10.18203/2320-6012.ijrms20232455

Keywords:

Idiopathic multicentric, Castleman disease, IgG4 disease, IL-6, Tocilizumab

Abstract

Castleman disease is a syndrome with significant clinico-pathological overlap between malignancy, autoimmune causes and infectious etiologies. It is a spectrum and can vary in extent from unicentric to multicentric disease with generalized lymphadenopathy, organ involvement, constitutional symptoms and cytopenias, and in severity from non-severe to severe disease with TAFRO symptoms. Idiopathic multicentric Castleman disease (iMCD) is a diagnosis of exclusion after multiple causes as per diagnostic criteria are excluded. Treatment varies between the disease severity types with anti-IL 6 antibodies for non-severe disease to cytotoxic chemotherapy agents for severe disease with TAFRO symptoms. We hereby report a case of a non-severe type of iMCD with a prolonged course and delayed arrival at the diagnosis, owing to the rarity of this condition, which stresses the need for a reduced threshold to consider MCD, early in the differential diagnosis. Interestingly there were positive auto-antibodies and elevated IgG4 levels in this patient, but applying strict criteria helps to distinguish the diagnosis.

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