Melanie D. Mumau

Clusterin expression and germinal center morphometry help distinguish idiopathic multicentric castleman disease from select lymphadenopathies

Michael V. Gonzalez, Kaiwen Wang, Joseph Zinski, Melanie D. Mumau et autres

Idiopathic multicentric Castleman disease (iMCD) is a heterogeneous cytokine storm disorder involving systemic inflammation, multicentric lymphadenopathy with characteristic histopathology, and life-threatening multiple organ dysfunction. Patients can present with symptoms ranging from thrombocytopenia, anasarca, fever/elevated C-reactive protein (CRP), reticulin myelofibrosis, renal dysfunction, and …

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Dysregulated lymphocyte localization in idiopathic multicentric Castleman disease

Melanie D. Mumau, Michael Gonzalez, Katherine S. Forsyth, María Betina Pampena et autres

Idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening hematologic disease involving episodic flares of uncontrollable systemic inflammation by unknown causes. Hallmark features of iMCD include multiple enlarged lymph nodes with characteristic histopathological phenotypes and a potentially fatal, cytokine release syndrome. …

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T follicular helper cells are mis-localized in idiopathic multicentric castleman disease

Melanie D. Mumau, Katherine S. Forsyth, Michael V. Gonzalez, Joseph M. Zinski et autres

Abstract Idiopathic multicentric Castleman disease (iMCD) is an atypical hematologic disorder with significant morbidity and mortality. Defined by enlarged lymph nodes (LN) with characteristic histopathology, there are a range of clinical subtypes with the most severe form having symptoms including thrombocytopenia, anasarca, …

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Increased type I interferon signaling is a hallmark of idiopathic multicentric castleman disease

Katherine S. Forsyth, Michael Gonzalez, Joseph M. Zinski, Melanie D. Mumau et autres

Abstract Human herpesvirus (HHV)-8-negative, idiopathic multicentric Castleman disease (iMCD) is a rare hematologic illness involving progressive flares of inflammation, lymphoproliferation, and cytokine-induced organ failure. While there is an FDA-approved anti-interleukin-6 (IL-6) therapy, siltuximab, which is effective in 34-50% of iMCD patients, disease …

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Somatic mutation discovery in idiopathic multicentric castleman disease (iMCD) lymph node tissue identifies dysregulated intracellular calcium signaling as a potential disease mechanism and therapeutic target

Michael V. Gonzalez, Melanie D. Mumau, Joseph M. Zinski, Vikas Arige et autres

Abstract Background: Idiopathic multicentric Castleman disease (iMCD) is a rare, atypical lymphoproliferative disorder with significant morbidity and mortality and unknown etiology. Given the clinico-pathologic similarities between iMCD and several lymphomas, the field has long hypothesized that a rare population of somatically mutated …

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A patient-derived CABIN1 mutation recapitulates features of idiopathic multicentric Castleman disease in a mouse model

Dorottya I. Laczko, Patricia Tsao, Ruth-Anne Langan Pai, Joseph M. Zinski et autres

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Common connective tissue disorder and anti-cytokine autoantibodies are enriched in idiopathic multicentric castleman disease patients

Allan Feng, Michael V. Gonzalez, Müge Kalaycıoğlu, Xihui Yin et autres

Introduction: Idiopathic Multicentric Castleman Disease (iMCD) is a polyclonal lymphoproliferative disorder involving cytokine storms that can lead to organ failure and death. The cause of iMCD is unknown, but some clinical evidence suggests an autoimmune etiology. For example, connective tissue disorders (CTDs) …

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Identifying and Targeting TNF Signaling in Idiopathic Multicentric Castleman’s Disease

Melanie D. Mumau, Michael V. Gonzalez, Chunyu Ma, Abiola H. Irvine et autres

A patient with longstanding multicentric Castleman’s disease that had progressed after multiple therapies was entering hospice care. Inhibition of tumor necrosis factor will have led to remission for 24 months as of the publication of this letter.

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No evidence for active viral infection in unicentric and idiopathic multicentric Castleman disease by Viral-Track analysis

Ira D. Miller, Melanie D. Mumau, Saishravan Shyamsundar, Mateo Sarmiento Bustamante et autres

Castleman disease (CD) is a rare hematologic disorder characterized by pathologic lymph node changes and a range of symptoms due to excessive cytokine production. While uncontrolled infection with human herpesvirus-8 (HHV-8) is responsible for the cytokine storm in a portion of multicentric …

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Trafficking, Not Lymphoproliferation, Promotes Lymphadenopathy in Idiopathic Multicentric Castleman Disease

Melanie D. Mumau, Criswell L. M. Lavery, Abiola H. Irvine, Joseph M. Zinski et autres

Idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening hematologic illness characterized by multifocal lymphadenopathy for an unknown cause. Patients with iMCD experience periods of systemic inflammation due to cytokine release that includes interleukin-6 (IL-6). Treatment with IL-6 inhibition is effective …

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Lymph Node Transcriptomics in Idiopathic Multicentric Castleman Disease (iMCD) Identifies Pathogenic Mechanisms and Biomarkers Including Increased Clusterin Expression

Michael V. Gonzalez, Melanie D. Mumau, Joseph M. Zinski, Abiola H. Irvine et autres

Idiopathic multicentric Castleman disease (iMCD) is an atypical lymphoproliferative disorder with significant morbidity and mortality and a poorly understood pathophysiology. Defined by a characteristic lymph node (LN) histology, patients can present with a range of symptoms from thrombocytopenia, anasarca, fever, renal dysfunction, …

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Immune‐mediated thrombocytopenia and IL‐6‐mediated thrombocytosis observed in idiopathic multicentric Castleman disease

Ayelet I. Rubenstein, Sheila K. Pierson, Saishravan Shyamsundar, Mateo Sarmiento Bustamante et autres

Idiopathic multicentric Castleman disease (iMCD) is a rare haematological disorder characterized by generalized lymphadenopathy with atypical histopathological features and systemic inflammation caused by a cytokine storm involving interleukin-6 (IL-6). Three clinical subtypes are recognized: thrombocytopenia, anasarca, fever, renal dysfunction, organomegaly (iMCD-TAFRO); idiopathic …

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