ICD-11 classes
03 Diseases of the blood or blood-forming organs
Anaemias or other erythrocyte disorders
3A51 — Sickle cell disorders or other haemoglobinopathies

ICD-11 3A51 — Sickle cell disorders or other haemoglobinopathies

Any disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may present with fatigue, shortness of breath, dizziness, headaches, pallor of skin or mucous membranes, and jaundice. This disorder is confirmed by identification of HbS mutation by genetic testing.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 3A51 contains 15 clarifying diagnoses:

  1. 3A51.0 — Sickle cell trait
  2. 3A51.1 — Sickle cell disease without crisis
  3. 3A51.2 — Sickle cell disease with crisis
  4. 3A51.3 — Compound heterozygous sickling disorders without crisis
  5. 3A51.4 — Compound heterozygous sickling disorders with crisis
  6. 3A51.5 — Haemoglobin C disease
  7. 3A51.6 — Haemoglobin D disease
  8. 3A51.7 — High affinity haemoglobin
  9. 3A51.8 — Low affinity haemoglobin
  10. 3A51.9 — Haemoglobin O disease
  11. 3A51.A — Haemoglobin E disease
  12. 3A51.B — Haemoglobin C/beta thalassaemia compound heterozygosity
  13. FB81.4 — Osteonecrosis due to haemoglobinopathy
  14. 3A51.Y — Other specified sickle cell disorders or other haemoglobinopathies
  15. 3A51.Z — Sickle cell disorders or other haemoglobinopathies, unspecified

The diagnosis is included in 2 other classes.

  • Osteonecrosis due to haemoglobinopathy (FB81.4)
  • Other sickle-cell disorders with retinopathy (9B71.Y)

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