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Mod Rheumatol. 2018 Jan;28(1):161-167. doi: 10.1080/14397595.2017.1366093. Epub 2017 Sep 7.

Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan.

Author information

1
a Division of Hematology and Immunology, Medicine , Kanazawa Medical University , Ishikawa , Japan.
2
b Unit of Advanced Preventive Medical Sciences , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan.
3
c Center for Bioinformatics and Molecular Medicine , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan.
4
d Division of Hematology, Department of Medicine , Keio University School of Medicine , Tokyo , Japan.
5
e Chemotherapy and Oncology Center , Osaka University Hospital , Osaka , Japan.
6
f Division of Clinical Oncology and Hematology, Department of Internal Medicine , Jikei University School of Medicine , Tokyo , Japan.
7
g Department of Hematology , Takamatsu Red Cross Hospital , Japan.
8
h Louis Pasteur Center for Medical Research , Kyoto , Japan.
9
i Department of Emergency , Japanese Red Cross Nagoya Daiichi Hospital , Nagoya , Japan.
10
j Department of Hematology , JA Mie Kouseiren Matsusaka Central General Hospital , Matsusaka , Japan.
11
k Department of Hematology , Itami City Hospital , Itami , Japan.
12
l Osaka Rheumatology Clinic , Tokyo Medical University , Osaka , Japan.
13
m Department of Respiratory Medicine , Yodogawa Christian Hospital , Osaka , Japan.
14
n Department of Pathology , Kansai Rosai Hospital , Hyogo , Japan.
15
o Department of Diagnostic Pathology , Cytology Osaka International Cancer Institute Hospital , Osaka , Japan.
16
p Rheumatic Diseases Center , Hohnan Kakogawa Hospital , Kakogawa , Japan.
17
q Hematology/Respiratory Medicine Kanazawa University Faculty of Medicine , Institute of Medical Pharmaceutical and Health Sciences , Kanazawa , Japan.
18
r Department of Organic Fine Chemicals , The Institute of Scientific and Industrial Research, Osaka University , Osaka , Japan.

Abstract

OBJECTIVES:

To determine the tentative diagnostic criteria and disease severity classification for Castleman disease (CD) and describe the clinical and pathologic features among human herpesvirus 8 (HHV-8) negative idiopathic multicentric CD (iMCD) in the Japanese population.

METHODS:

We established the working groups for the research of CD in Japan and had meetings to discuss and define the tentative diagnostic criteria and disease severity classification for CD. We subsequently analyzed 142 patients classified into iMCD by using the nationwide Japanese patient registry.

RESULTS:

We proposed the preliminary diagnostic criteria and disease severity classification for CD based on our discussion. In addition, we made a proposal for the disease activity score. We identified clinical and pathological features of patients with iMCD diagnosed by these diagnostic criteria. In the disease severity classification, 37, 33 and 30% patients were categorized into mild, moderate and severe diseases, respectively.

CONCLUSION:

This is the first proposal for diagnosis and classification of CD by the Japanese group. Further studies are required to validate whether they can distinguish CD from other inflammatory diseases and to determine their sensitivity and specificity.

KEYWORDS:

Castleman disease; TAFRO syndrome; diagnostic criteria; disease severity classification; idiopathic multicentric Castleman disease

PMID:
28880697
DOI:
10.1080/14397595.2017.1366093
[Indexed for MEDLINE]

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