. Author manuscript; available in PMC: 2022 Nov 14.

Published in final edited form as: Am J Hematol. 2021 Jul 28;96(10):1241–1252. doi: 10.1002/ajh.26292

Table 3.

Diagnostic Criteria for iMCD or CD

	Inclusion Criteria	Exclusion Criteria
Fajgenbaum et al. (2017) ^a	(Mandatory) Histopathologic features present in LN tissue Enlarged LNs in more than one LN station	(need 2 or more of 11 with at least 1 laboratory criterion) Laboratory Elevated ESR or CRP Anemia Thrombocytopenia or thrombocytosis Renal dysfunction or proteinuria Polyclonal hypergammaglobulinemia Hypoalbuminemia Clinical Constitutional symptoms Splenomegaly and/or hepatomegaly Fluid accumulation Eruptive cherry angiomata or violaceous papules Lymphocytic interstitial pneumonitis	Autoimmune/inflammatory disease Infection-related disorders Malignancy/lymphoproliferative disorders
Fujimoto et al. for CD (2018) ^b	(Mandatory) ≥1 swollen LNs 1 swollen LN: UCD >1 swollen LN: MCD Histopathological findings of LNs or other organs that is consistent with one of the following types of CD Hyaline vascular Plasma cell Mixed Diagnosis of iMCD is after exclusion of UCD and HHV-8-positive MCD	N/A	Malignant neoplasms Infectious diseases Autoimmune diseases Others (IgG4-RD, histiocytic necrotizing lymphadenitis, sarcoidosis, idiopathic portal hypertension, etc.)

Inclusion Criteria

Exclusion Criteria

Major Criteria

Minor Criteria

Fajgenbaum et al. (2017) ^a

(Mandatory)

Histopathologic features present in LN tissue
Enlarged LNs in more than one LN station

(need 2 or more of 11 with at least 1 laboratory criterion)
Laboratory

Elevated ESR or CRP
Anemia
Thrombocytopenia or thrombocytosis
Renal dysfunction or proteinuria
Polyclonal hypergammaglobulinemia
Hypoalbuminemia

Clinical

Constitutional symptoms
Splenomegaly and/or hepatomegaly
Fluid accumulation
Eruptive cherry angiomata or violaceous papules
Lymphocytic interstitial pneumonitis

Autoimmune/inflammatory disease
Infection-related disorders
Malignancy/lymphoproliferative disorders

Fujimoto et al. for CD (2018) ^b

(Mandatory)

≥1 swollen LNs
- 1 swollen LN: UCD
- >1 swollen LN: MCD
Histopathological findings of LNs or other organs that is consistent with one of the following types of CD
- Hyaline vascular
- Plasma cell
- Mixed
Diagnosis of iMCD is after exclusion of UCD and HHV-8-positive MCD

N/A

Malignant neoplasms
Infectious diseases
Autoimmune diseases
Others (IgG4-RD, histiocytic necrotizing lymphadenitis, sarcoidosis, idiopathic portal hypertension, etc.)

Abbreviations: CD, Castleman disease; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; HHV-8, human herpesvirus-8; HIV, human immunodeficiency virus; IgG4-RD, IgG4-related diseases; LN, lymph node; UCD, unicentric Castleman disease

International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease. Blood. 2017 Mar 23;129(12):1646-1657.

Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan. Mod Rheumatol 28(1): 161-167.