2A84.1

ICD-11 MMS 2026-01

Gamma heavy chain disease

Description

A clonal disorder characterised by the secretion of a truncated gamma chain. In most cases, it is associated with morphologic changes also seen in lymphoplasmacytic lymphomas, but the clinical course is typically more aggressive than in lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia.

Index terms

  • Gamma heavy chain disease
  • Franklin disease

Inclusions

  • Franklin disease

Exclusions from above levels

  • 2E03 – Malignant neoplasm metastasis in bone or bone marrow
  • http://id.who.int/icd/entity/501778925 – Malignant neoplasm metastasis in lymph nodes
  • http://id.who.int/icd/entity/97145147 – Malignant neoplasm metastasis in thymus gland

Sibling codes

  • 2A84.0 – Alpha heavy chain disease
  • 2A84.2 – Mu heavy chain disease
  • 2A84.Y – Other specified malignant immunoproliferative diseases
  • 2A84.Z – Heavy chain diseases, unspecified

Foundation entity

Gamma heavy chain disease – http://id.who.int/icd/entity/705015637

Allowed postcoordination axes

Part of: 2A84: Heavy chain diseases or malignant immunoproliferative diseases › 02: Neoplasms