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D57.0 – Sickle-Cell Disease with Crisis | ICD-10
D57.0 is the ICD-10 code for sickle-cell disease with haemolytic crisis. It describes a severe acute complication of sickle-cell anaemia requiring immediate medical attention.
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D57.0 is the ICD-10 code for sickle-cell disease with haemolytic crisis. It describes a severe acute complication of sickle-cell anaemia requiring immediate medical attention.
What is D57.0?
D57.0 is an ICD-10 diagnosis code that refers to sickle-cell disease with haemolytic crisis. This condition belongs to the group of haemoglobinopathies – disorders in which the structure of haemoglobin (the red blood cell protein that carries oxygen) is abnormal. In sickle-cell disease, a genetic mutation causes red blood cells to adopt a rigid, sickle-like shape under certain conditions, leading to blockages in blood vessels and accelerated cell destruction.
Causes
Sickle-cell disease is an autosomal recessive genetic disorder caused by a point mutation in the beta-globin gene, resulting in the production of abnormal haemoglobin S (HbS). When a person inherits two copies of the mutated gene (one from each parent), they develop sickle-cell disease (genotype HbSS).
- Genetic mutation in the beta-globin gene
- Autosomal recessive inheritance pattern
- Both parents must be carriers of the mutated gene
- Particularly prevalent in populations from sub-Saharan Africa, the Mediterranean region, and the Middle East
What is a haemolytic crisis?
In D57.0, the underlying chronic disease is accompanied by a haemolytic crisis – a sudden and accelerated destruction of red blood cells (haemolysis) that causes a rapid drop in haemoglobin levels. Common triggers include:
- Infections
- Oxygen deprivation
- Cold exposure or dehydration
- Physical overexertion
- Emotional stress
Symptoms
Symptoms of D57.0 include both the chronic manifestations of sickle-cell disease and the acute features of a crisis:
- Severe pain episodes (pain crises), commonly affecting bones, joints, the chest, or the abdomen
- Profound fatigue and weakness due to anaemia
- Yellowing of the skin and eyes (jaundice) due to elevated bilirubin levels
- Shortness of breath
- Enlarged spleen (splenomegaly)
- Increased susceptibility to infections
- In children: growth delays and developmental issues
Diagnosis
Diagnosis of sickle-cell disease and its acute crises involves several investigations:
- Full blood count: Reveals normocytic anaemia and elevated reticulocyte count
- Blood smear: Characteristic sickle-shaped red blood cells visible under microscopy
- Haemoglobin electrophoresis: Gold standard for identifying HbS
- Genetic testing: Confirms the beta-globin gene mutation
- Newborn screening: Routinely performed in many countries to enable early intervention
Treatment
Management of D57.0 addresses both the acute crisis and long-term disease control:
Acute Crisis Management
- Pain management: Analgesics including opioids for severe pain episodes
- Fluid therapy: Oral or intravenous hydration to support circulation
- Oxygen supplementation when blood oxygen levels are low
- Treatment of triggering factors (e.g., antibiotics for infections)
- Blood transfusion in cases of severe anaemia
Long-term Treatment
- Hydroxyurea (hydroxycarbamide): The primary disease-modifying medication that reduces the frequency of crises
- Penicillin prophylaxis: Especially important in young children to prevent bacterial infections
- Folic acid supplementation: Supports ongoing red blood cell production
- Stem cell transplantation: Currently the only potentially curative treatment
- Regular medical follow-up and vaccinations
References
- World Health Organization (WHO): Sickle-cell disease and other haemoglobin disorders. Fact sheet. Geneva, 2023. Available at: https://www.who.int/news-room/fact-sheets/detail/sickle-cell-disease
- Rees DC, Williams TN, Gladwin MT: Sickle-cell disease. The Lancet. 2010;376(9757):2018-2031. DOI: 10.1016/S0140-6736(10)61029-X
- National Heart, Lung, and Blood Institute (NHLBI): Evidence-Based Management of Sickle Cell Disease: Expert Panel Report. U.S. Department of Health and Human Services, 2014.
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