Castleman disease

What it is

From: Orphanet

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.

Reported clinical features and what the terms mean

The following findings are associated with this condition in Orphanet. They are not a checklist for diagnosing yourself, and they do not all occur in every affected person. Some are examination, imaging or laboratory findings that cannot be recognised at home.

The frequency labels describe how often a finding was reported among people with the condition in the source. They do not give the chance that a person with that symptom has the condition. Definitions below reproduce HPO terminology; they explain the term, not the likely severity in an individual.

Lymphadenopathy · Very frequent (99-80%)

Enlargement (swelling) of a lymph node.

Abdominal pain · Frequent (79-30%)

An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) and perceived to originate in the abdomen.

Anemia · Frequent (79-30%)

A reduction in erythrocytes volume or hemoglobin concentration.

Constitutional symptom · Frequent (79-30%)

A symptom or manifestation indicating a systemic or general effect of a disease and that may affect the general well-being or status of an individual.

Elevated circulating C-reactive protein concentration · Frequent (79-30%)

The concentration of C-reactive protein in the blood circulation is above the upper limit of normal.

Elevated erythrocyte sedimentation rate · Frequent (79-30%)

An increased erythrocyte sedimentation rate (ESR). The ESR is a test that measures the distance that erythrocytes have fallen after one hour in a vertical column of anticoagulated blood under the influence of gravity. The ESR is a nonspecific finding. An elevation may indicate inflammation or may be caused by any condition that elevates fibrinogen.

Fatigue · Frequent (79-30%)

A subjective feeling of tiredness characterized by a lack of energy and motivation.

Follicular hyperplasia · Frequent (79-30%)

Lymphadenopathy (enlargement of lymph nodes) owing to hyperplasia of follicular (germinal) centers.

Increased circulating interleukin 6 concentration · Frequent (79-30%)

The concentration of interleukin-6 in the blood circulation is above the upper limit of normal.

Mediastinal lymphadenopathy · Frequent (79-30%)

Swelling of lymph nodes within the mediastinum, the central compartment of the thoracic cavities that contains the heart and the great vessels, the esophagus, and trachea and other structures including lymph nodes.

Weight loss · Frequent (79-30%)

Reduction of total body weight.

Abdominal distention · Occasional (29-5%)

Distention of the abdomen.

Abdominal mass · Occasional (29-5%)

An abnormal enlargement or swelling in the abdomen.

Abnormality of the gastrointestinal tract · Occasional (29-5%)

An abnormality of the gastrointestinal tract.

Other findings in the same source

From: Orphanet

Additional reported features include Cough (Occasional (29-5%)); Decreased mean corpuscular volume (Occasional (29-5%)); Flank pain (Occasional (29-5%)); Generalized lymphadenopathy (Occasional (29-5%)); Jaundice (Occasional (29-5%)); Nausea and vomiting (Occasional (29-5%)); Anasarca (Very rare (<4-1%)); Dyspnea (Very rare (<4-1%)); Hematuria (Very rare (<4-1%)); Intestinal obstruction (Very rare (<4-1%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

All ages

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Annual incidence: 1-9 / 100 000; United States; Class only.

Which doctor should you see?

The suggested department for discussing Castleman disease is Haematology, with a haematologist as the relevant type of clinician. General physician / Family Medicine; paediatrician for children. Referral depends on symptoms.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• Which blood-cell, marrow, bleeding or clotting finding matters most?
• Does the diagnosis need confirmation or a more precise subtype?
• Which symptoms or laboratory changes should trigger earlier review?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Castleman disease. That gap does not mean that treatment is unavailable. A clinician needs to establish the diagnosis and review current guidance before recommending medicines, procedures, rehabilitation or other support.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.