Successful rituximab treatment of an elderly Japanese patient with HHV8-positive, HIV-negative multicentric Castleman disease

Human herpesvirus-8 (HHV8)-positive, human immunodeficiency virus (HIV)-negative multicentric Castleman disease (MCD) is a rare and age-related lymphoproliferative disorder caused by cytokine storm. Rituximab treatment is currently recommended because B-cell depletion eliminates the primary reservoi...

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Bibliographic Details
Published in:International journal of hematology 2022, Vol.115 (1), p.129-134
Main Authors: Kaga, Hajime, Kurahashi, Honami, Kubota, Arisa, Hatano, Yoshiaki, Nanjo, Hiroshi, Wakui, Hideki, Takahashi, Naoto
Format: Article
Language:English
Subjects:
Administration, Oral
Adult
Age
Age Factors
Aged
Aged, 80 and over
Asians
B-Lymphocyte Subsets - immunology
Case Report
Castleman Disease - diagnosis
Castleman Disease - drug therapy
Castleman Disease - immunology
Castleman Disease - virology
Castleman's disease
Cytokine storm
Cytokines
Depletion
Female
Hematology
HIV
Human immunodeficiency virus
Humans
Immunotherapy
Lymphadenopathy
Lymphocytes
Lymphocytes B
Male
Medicine
Medicine & Public Health
Middle Aged
Monoclonal antibodies
Oncology
Patients
Prednisolone - administration & dosage
Recurrence
Remission
Remission Induction
Rituximab
Rituximab - administration & dosage
Rituximab - therapeutic use
Signs and symptoms
Steroids
Tapering
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Description
Summary:Human herpesvirus-8 (HHV8)-positive, human immunodeficiency virus (HIV)-negative multicentric Castleman disease (MCD) is a rare and age-related lymphoproliferative disorder caused by cytokine storm. Rituximab treatment is currently recommended because B-cell depletion eliminates the primary reservoir for HHV8. We report the first case of effective rituximab treatment of a Japanese patient (an 87-year-old woman) with this disorder. Her inflammatory symptoms and lymphadenopathy improved after medium-dose steroid therapy, but these symptoms recurred during steroid tapering. After one course of rituximab therapy, she achieved sustained remission. HHV8-associated MCD should be considered as a possible diagnosis in HIV-negative patients with inflammatory symptoms and lymphadenopathy.
ISSN:0925-5710
1865-3774
DOI:10.1007/s12185-021-03222-7