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Castleman Disease

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Castleman Disease

  • Disease Overview
  • Treatment Overview

Castleman disease (CD) describes a rare group of lymphoproliferative disorders that share
morphological features but have a wide range of different aetiologies, presentations and
outcomes.1–4

CD can affect lymph nodes in any body area, including the neck, chest, abdomen, and pelvis,
with the lesions imitating both benign and malignant malformations.4 CD can be divided into
the more common unicentric CD (UCD), which is a localised condition involving a single
lymph node, and the less common multicentric CD (MCD), a systemic, progressive, and
potentially fatal condition with lymphadenopathy in multiple nodes across the body.3,4 

Incidence

The estimated incidence of all forms of CD is 21–25 individuals per million person-years (based on insurance registries in the US).2

The incidence of UCD is ~16–19 per million patient-years, while the incidence of MCD is ~5 per million patient-years (US data).3–5

The incidence of idiopathic MCD (iMCD) is ~3.1–3.4 per million patient-years (US data).6

Cause

The exact pathophysiology of CD is not well defined, but there is evidence suggesting that this condition may be due to impaired immunoregulation, leading to excessive proliferation of B lymphocytes and plasma cells in lymphoid organs.4  

Human herpes virus-8 (HHV8), in the presence or absence of HIV infection, has been associated with a proportion of cases of MCD by mediating dysregulation of inflammatory mediators such as CD20 and interleukin-6 (IL-6).1,2,4

In addition, IL-6 is a key disease driver in some patients with iMCD.1

Clinical forms of CD

UCD

This is the most common form, referring to a localised disease state in which a single lymph node is affected.1,2,4 Systemic symptoms are uncommon in UCD, with fever observed in <10% of cases and inflammatory markers and organ function tests being generally normal.2

HHV8-MCD

This refers to cases of MCD that have been attributed to HHV8 infection. Generally, infection with HHV8 occurs in the context of immunodeficiency, mainly due to HIV infection.1,2 Patients frequently present with fever, splenomegaly, and effusions, while blood test results may show high ferritin levels, direct anti-globulin test positive haemolysis, and monoclonal gammopathy.2

POEMS-associated MCD

This refers to patients with MCD who are HHV8-infection negative, that are simultaneously diagnosed with peripheral sensorimotor neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesions (POEMS) syndrome.1 Patients generally present with fever, splenomegaly, hepatomegaly, skin
changes, and polyneuropathy.1–3

iMCD

This refers to cases of MCD without a known cause, in which other conditions and types of MCD have been previously ruled out.2,4,7 iMCD can lead to a wide range of symptoms and complications, including fever, drenching sweats, weight loss, splenomegaly, hepatomegaly, liver and kidney dysfunction, and multi-organ failure.2,3,7

Patients with iMCD have a significantly higher risk of developing malignancy (such as lung cancer, thyroid cancer, and colon cancer) compared to non-iMCD matched patients.8

iMCD affects multiple systems and organs, with ~51% of patients experiencing organ dysfunction, compared to ~13% of non-iMCD matched patients.8

Organ dysfunction is the most prevalent of all morbidities in patients with iMCD, with the kidneys and lungs being the most likely affected organs (affecting ~25% of iMCD patients vs ~5% of non-iMCD matched patients).8

Survival

5-year overall survival rates for UCD and MCD have been estimated at 91% and 65%, respectively2

Symptoms of MCD include:

Fatigue2

Fever2

Night sweats2

Anasarca2

Weight loss2,3,7

Complications of MCD include:

Kidney dysfunction3

Liver dysfunction3

Impaired lung function7

Polyneuropathy3

Anaemia2,3

Diagnosis

To learn more about diagnosis and other information, please explore our resources by clicking on the link below.

EXPLORE RESOURCES

CD, Castleman disease; CD20, cluster of differentiation 20; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; HHV8, human herpes virus-8, HIV, human immunodeficiency virus; IL, interleukin; iMCD, idiopathic multicentric Castleman disease; MCD, multicentric Castleman disease; POEMS, peripheral sensorimotor neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesions; UCD, unicentric Castleman disease; UK, United Kingdom; US, United States.

References:

  1. Dispenzieri A, Fajgenbaum DC. Blood. 2020;135(16):1353–1364.
  2. Lomas OC et al. Br J Haematol. 2021;195(3):328–337.
  3. Carbone A et al. Nat Rev Dis Primers. 2021;7(1)84.
  4. Ehsan N, Zahra F. Treasure Island (FL): StatPearls Publishing. 2024.
  5. van Rhee F et al. 2020;4(23):6039–6050.
  6. Mukherjee S et al. Blood Adv. 2022;6(2):359–367.
  7. NHS. Available at: https://www.england.nhs.uk/wp-content/uploads/2023/11/2124-cc-policy-siltuximab-castleman-disease-updated.pdf. Date accessed: November 2025.
  8. Mukherjee S et al. 2022. Poster presented at EHA; Vienna, Austria; 9–17 June, 2022. Abstract: P1733.

To learn more about treatment for iMCD, and in particular information for Sylvant▼ (siltuximab), please visit our resources by clicking the link below.

EXPLORE RESOURCES

IE-NPS-0113 | June 2026