Caring for someone with this diagnosis? Eleplan keeps diagnoses, medications, documents and every specialist in one plan.
Start free with EleplanCastleman disease
ORPHA:160Disease
Also called Angiofollicular ganglionic hyperplasia · Angiofollicular lymph hyperplasia
What it is
A rare lymphoid hemopathy characterized by involvement of lymph nodes in any part of the body, most frequently the mediastinum, abdomen, neck, or spleen, and occurring as unicentric, idiopathic multicentric, or KSHV/HHV8-associated multicentric Castleman disease. Depending on the type, patients are most commonly asymptomatic or typically present with systemic symptoms.
Key facts
- Prevalence
- 1-9 / 100 000 (annual incidence, United States)
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
10Sometimes5–29%
9- Abdominal distention
- Abdominal mass
- Abnormality of the gastrointestinal tract
- Cough
- Decreased mean corpuscular volume
- Flank pain
- Generalized lymphadenopathy
- Jaundice
and 1 more in this range
Rare1–4%
9- Anasarca
- Dyspnea
- Hematuria
- Intestinal obstruction
- Myelofibrosis
- Renal insufficiency
- Restrictive cardiomyopathy
- Thrombocytopenia
and 1 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
Powered by Eleplan
A rare diagnosis is just the start. Eleplan keeps the whole care plan in one place.
Diagnoses, medications, documents, appointments, and the whole care team — organized and always in sync, so you are not retelling the same story to every new specialist. With Ellie, your AI care assistant, on top of it. Free to start.