3A51.3

ICD-11 MMS 2026-01

Compound heterozygous sickling disorders without crisis

Description

A disease caused by genetic inheritance of two heterozygous recessive alleles of the haemoglobin gene leading to abnormal formation of haemoglobin molecule. This disease is characterised by rigid, sickle shaped red blood cells. Confirmation is by identification of mutations through genetic testing.

Index terms

  • Compound heterozygous sickling disorders without crisis
  • double heterozygous sickling disorder
  • Sickle cell Hb-C disease without crisis
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  • Hb S Hb C disease
  • Hb SC disease
  • sickle-cell HbC disease
  • Sickle cell thalassaemia without crisis
  • double heterozygous for Hb S or beta thalassaemia
  • haemoglobin s/beta thalassaemia
  • microdrepanocytosis
  • sickle-cell beta thalassaemia
  • sickle-cell thalassaemia
  • thalassaemia with haemoglobin S disease
  • Compound HbS or HbC heterozygotes
  • Compound HbS, HbD-Punjab or Los Angeles heterozygotes
  • Hb-SD disease
  • Compound HbS or HbE heterozygotes
  • Hb SE disease
  • Compound HbS or O-Arab heterozygotes
  • Double heterozygous sickling disorders with retinopathy
  • Glomerular disorders in double heterozygous sickling disorders
  • Gouty arthropathy in double heterozygous sickling disorders
  • Compound HbS or beta thalassaemia heterozygotes
  • Compound HbS and beta thalassaemia heterozygotes including HbS/delta-beta-thal compounds
  • Hereditary persistence of fetal haemoglobin - sickle cell disease

Inclusions

  • Sickle cell Hb-C disease without crisis
  • Sickle cell thalassaemia without crisis

Exclusions from above levels

  • 04 – Diseases of the immune system
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth or the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations or chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes
  • http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
  • JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

  • 3A51.0 – Sickle cell trait
  • 3A51.1 – Sickle cell disease without crisis
  • 3A51.2 – Sickle cell disease with crisis
  • 3A51.4 – Compound heterozygous sickling disorders with crisis
  • 3A51.5 – Haemoglobin C disease
  • 3A51.6 – Haemoglobin D disease
  • 3A51.7 – High affinity haemoglobin
  • 3A51.8 – Low affinity haemoglobin
  • 3A51.9 – Haemoglobin O disease
  • 3A51.A – Haemoglobin E disease
  • 3A51.B – Haemoglobin C/beta thalassaemia compound heterozygosity
  • 3A51.Y – Other specified sickle cell disorders or other haemoglobinopathies
  • 3A51.Z – Sickle cell disorders or other haemoglobinopathies, unspecified

Foundation entity

Compound heterozygous sickling disorders without crisis – http://id.who.int/icd/entity/313446364

Allowed postcoordination axes

Part of: 3A51: Sickle cell disorders or other haemoglobinopathies › 03: Diseases of the blood or blood-forming organs