Castleman diseases: a journey into translational immunology

David Boutboul, MD, PhD
Assistant Professor & Hospital Practitioner
Clinical Immunology, Hôpital Cochin, Paris
INSERM U1163 Laboratory, Institut Imagine, Paris

In person: 
IRCM Auditorium
110, avenue des Pins O, H2W 1R7 Montreal

About this conference
Castleman diseases (CD) are rare lymphoproliferative disorders encompassing distinct clinicopathological entities. 

The unicentric form of the disease (UCD) usually involves a single lymph node station, exhibits hyaline vascular pathological changes with follicular dendritic cell expansion, and may associate with specific and life-threatening complications including paraneoplastic pemphigus (PNP) and follicular dendritic cell sarcoma. The best treatment option is complete surgical excision but up to half of the patients cannot undergo surgery, due to unresectable tumors and/or severe extension of the PNP lesions to the bronchial mucosa (bronchiolitis obliterans). In these patients, an efficient medical approach has to be defined, as no current medical treatment has demonstrated to lower morbidity and mortality in this setting. Recent advances in UCD pathophysiology will be discussed, which have led to the emergence of novel targeted therapies. 

Multicentric Castleman diseases (MCD) also include several lymphoproliferative disorders with close histopathological patterns. One MCD subset is associated with Kaposi Sarcoma-associated Herpes Virus/Human Herpesvirus 8 (KSHV/HHV8) infection and mainly affects HIV-infected individuals or transplant recipients. The course of KSHV+ MCD is marked by flares causing fever, lymphadenopathy, splenomegaly, effusion, cytopenia, hypoalbuminemia, and high serum C-reactive protein level reflecting a high degree of systemic inflammation. Life-threatening complications may occur, including hemophagocytic lympho-histiocytosis, and KSHV+ MCD is therefore considered a medical emergency. Moreover, patients with KSHV+ MCD are at high risk of developing aggressive lymphoma during follow-up. Novel diagnostic tools allowing rapid diagnosis of KSHV+ MCD will be presented and hypotheses regarding viral-induced genomic instability and lymphoma susceptibility will be discussed.

About David Boutboul
Dr David Boutboul graduated from the Université Paris Descartes in 2008 after a residency in Clinical Immunology. He studied the genetic basis of Common Variable Immune Deficiency (CVID)-associated lymphoproliferative disorders during his PhD at Université Paris Diderot and joined the team of Dr S. Latour and Pr A. Fischer in 2015 at Institut Imagine for a postdoctoral fellowship focusing on the genetics of EBV susceptibility and IKZF1 germline mutations in children with Inborn Errors of Immunity. He's now Associate Professor of Clinical Immunology at Hôpital Cochin, Paris. He recently developed a research activity in the U1163 Unit at Institut Imagine, leading a group working on the pathophysiology of Castleman diseases (Unicentric and KSHV-related Castleman diseases). 

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