Castleman disease

Castleman disease is part of the Tumor like lesion with B-cell predominance lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B13)

See 9738/3 for Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease (HHV8 MCD).

UCD usually involves a single lymph node, or occasionally, one group of lymph nodes. The most common nodal stations are the mediastinum, neck, abdomen, and retroperitoneum.

iMCD occurs in lymph nodes in any site, most frequently the neck, mediastinum, axilla, and abdomen, and in the spleen.

KSHV/HHV8-MCD involves lymph nodes (most frequently axillary, abdominal, pelvic, mediastinal, and cervical) and the spleen.

Hyaline-vascular unicentric Castleman disease (HV-UCD

(KSHV/HHV8)-associated multicentric Castleman disease

Idiopathic multicentric Castleman disease (iMCD)

iMCD-TAFRO (thrombocytopenia, anasarca, fever / inflammatory symptoms, renal dysfunction / bone marrow reticulin fibrosis, organomegaly)

Mixed/plasmacytic unicentric Castleman disease

Multicentric Castleman disease

Unicentric Castleman disease

There are several types of Castleman disease

Unicentric Castleman disease (UCD) is a benign lymphoproliferative disorder with distinctive morphological features that involves a single lymph node or group of lymph nodes in one lymph node station. (WHO 5th ed).

Idiopathic multicentric Castleman disease (iMCD) is a lymphoproliferative disorder involving two or more lymph node sites, associated with systemic inflammatory symptoms and organ dysfunction related to hypercytokinemia. The diagnosis requires fulfilment of morphological, clinical, and laboratory criteria, and exclusion of other diseases, including HIV infection, KSHV/HHV8 infection, and other forms of Castleman disease. (WHO 5th ed).

Kaposi sarcoma–associated herpesvirus / human herpesvirus 8 (KSHV/HHV8)-associated multicentric Castleman disease (KSHV/HHV8-MCD) is a lymphoproliferative disorder featuring characteristic KSHV/HHV8-infected plasmablasts, idiopathic multicentric Castleman disease (iMCD)-like morphology, and systemic inflammatory symptoms due to proinflammatory hypercytokinemia. (WHO 5th ed)

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: B-cell lymphoid proliferations and lymphomas
Pages: Part B: 319-327