Heavy chain disease

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Heavy chain disease

ORPHA:86864Disease

Also called HCD

What it is

Heavy-chain diseases (HCDs) are rare monoclonal lymphoplasma-cell proliferative disorders involving B cells and are characterized by the synthesis of truncated heavy chains without associated light chains.

Key facts

Prevalence
<1 / 1 000 000 (annual incidence, Europe)
Age of onset
Adult
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.

ICD-10 codes

C88.2ICD-10 uses a narrower term — shared with 2 other rare diseases
C88.3ICD-10 uses a narrower term — shared with 1 other rare disease

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.

Cross-references

MEDDRA 10019350MESH D006362MONDO 0019464UMLS C0018852

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.

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