Clinicopathologic characteristics of 342 patients with multicentric Castleman disease in Japan

Abstract

Objectives: To assess the clinicopathologic features of Multicentric Castleman disease (MCD) patients in Japan. Methods: We assessed baseline data for 342 Japanese MCD patients with a biopsy-proven diagnosis, enrolled in a prospective, observational study for tocilizumab treatment. Results: Of 342 patients, 86.0% had plasma-cell type. None had a family history of MCD. Median disease duration of MCD was 3.7 years. Mean body weight and body mass index tended to be lower than those in the general Japanese population. The most common clinical presentations besides lymphadenopathy included fatigue (61.7%), pulmonary involvement (42.7%), and splenomegaly (41.8%). Secondary amyloidosis was reported in 34 patients (9.9%). Laboratory abnormalities included decreased hemoglobin and albumin, and increased acute-phase proteins, serum immunoglobulins, and interleukin-6 (IL-6). IL-6 levels among the MCD patients tested in this study were correlated with levels of albumin, hemoglobin, triglyceride, total cholesterol, C-reactive protein, fibrinogen and immunoglobulin G (Spearman's correlation coefficient, jrj ¼ 0.28-0.59). Conclusion: The clinical features and laboratory abnormalities are similar to those previously reported in other countries, besides higher rates of pulmonary involvement, secondary amyloidosis, and ECG abnormalities. Our results imply that IL-6 is involved in MCD pathogenesis. These findings would be informative for diagnosis and appropriate treatment for MCD. ARTICLE HISTORY

Multicentric Plasma-Cell Type Castleman Disease Masquerading As Hodgkin Lymphoma: A Case Report

“…1,2 CD can be categorized histomorphologically into three distinct subtypes, namely: hyaline vascular, plasma cell, and mixed hyaline vascular plasma cell types. 1,2,[4][5][6][7][8] CD also has two clinicopathological presentations, namely: unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD) forms, wherein the unicentric forms are more common. 1,2,5,6,8 The UCD form presents as a solitary (unifocal) lymph node lesion especially in the mediastinum and less commonly in extrathoracic sites and may not be accompanied by systemic symptoms such as fever, night sweats, fatigue, weight loss, splenomegaly, anemia, and hypergammaglobulinaemia.…”

“…1,2,[4][5][6][7][8] CD also has two clinicopathological presentations, namely: unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD) forms, wherein the unicentric forms are more common. 1,2,5,6,8 The UCD form presents as a solitary (unifocal) lymph node lesion especially in the mediastinum and less commonly in extrathoracic sites and may not be accompanied by systemic symptoms such as fever, night sweats, fatigue, weight loss, splenomegaly, anemia, and hypergammaglobulinaemia. 1,2,5,6,8 The MCD form, on the other hand, presents as a multifocal lymph node lesion with generalized lymphadenopathy, especially in the neck region, associated with more aggressive systemic symptoms such as fever, night sweats, fatigue, cachexia, splenomegaly, cytopenia, and hypoglobulinemia/hyperglobulinemia, hence resembling a malignant (Hodgkin's) lymphoma.…”

“…1,2,5,6,8 The UCD form presents as a solitary (unifocal) lymph node lesion especially in the mediastinum and less commonly in extrathoracic sites and may not be accompanied by systemic symptoms such as fever, night sweats, fatigue, weight loss, splenomegaly, anemia, and hypergammaglobulinaemia. 1,2,5,6,8 The MCD form, on the other hand, presents as a multifocal lymph node lesion with generalized lymphadenopathy, especially in the neck region, associated with more aggressive systemic symptoms such as fever, night sweats, fatigue, cachexia, splenomegaly, cytopenia, and hypoglobulinemia/hyperglobulinemia, hence resembling a malignant (Hodgkin's) lymphoma. 1,2,5,6,8 Furthermore, this MCD form can be subcategorized into three subtypes, namely: human herpes virus-8 associated MCD (HHV8-MCD), polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes-associated MCD (POEMS-MCD), and idiopathic MCD (iMCD).…”

“…1,2,5,6,8 The MCD form, on the other hand, presents as a multifocal lymph node lesion with generalized lymphadenopathy, especially in the neck region, associated with more aggressive systemic symptoms such as fever, night sweats, fatigue, cachexia, splenomegaly, cytopenia, and hypoglobulinemia/hyperglobulinemia, hence resembling a malignant (Hodgkin's) lymphoma. 1,2,5,6,8 Furthermore, this MCD form can be subcategorized into three subtypes, namely: human herpes virus-8 associated MCD (HHV8-MCD), polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes-associated MCD (POEMS-MCD), and idiopathic MCD (iMCD). 2,6,8,9 Additionally, iMCD is further subdivided into two types, namely: iMCD associated with thrombocytopenia, anasarca, fever, reticulin Multicentric Plasma-Cell Type Castleman Disease Ekwere, Eziagu myelofibrosis and renal dysfunction and organomegaly (iMCD-TAFRO) and iMCD not associated with TAFRO hence not otherwise specified (iMCD-NOS).…”

“…1,2,5,6,8 Furthermore, this MCD form can be subcategorized into three subtypes, namely: human herpes virus-8 associated MCD (HHV8-MCD), polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes-associated MCD (POEMS-MCD), and idiopathic MCD (iMCD). 2,6,8,9 Additionally, iMCD is further subdivided into two types, namely: iMCD associated with thrombocytopenia, anasarca, fever, reticulin Multicentric Plasma-Cell Type Castleman Disease Ekwere, Eziagu myelofibrosis and renal dysfunction and organomegaly (iMCD-TAFRO) and iMCD not associated with TAFRO hence not otherwise specified (iMCD-NOS). 2,8 It is also noted that nonidiopathic MCDs occur in the background of human immunodeficiency virus (HIV) infection especially in association with Kaposi sarcoma herpesvirus (KSHV) or HHV8 as well as in association with secondary amyloidosis.…”