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D58.0 Hereditary Spherocytosis -- ICD-10 Explained
D58.0 is the ICD-10 code for hereditary spherocytosis, an inherited hemolytic anemia in which red blood cells take on a spherical shape and are prematurely destroyed.
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D58.0 is the ICD-10 code for hereditary spherocytosis, an inherited hemolytic anemia in which red blood cells take on a spherical shape and are prematurely destroyed.
What Does ICD-10 Code D58.0 Mean?
The ICD-10 code D58.0 refers to hereditary spherocytosis (also known as congenital spherocytic anemia or Minkowski-Chauffard syndrome). It is an inherited hemolytic anemia in which red blood cells (erythrocytes) have a spherical shape instead of the normal biconcave disc shape due to a genetic defect. These abnormal cells, called spherocytes, are less flexible and are prematurely removed from circulation by the spleen, leading to anemia.
Causes
Hereditary spherocytosis is caused by mutations in genes encoding proteins of the red blood cell membrane skeleton. The most commonly affected proteins include:
- Ankyrin (most frequent cause)
- Band 3 protein
- Spectrin (α or β)
- Protein 4.2
The condition is most commonly inherited in an autosomal dominant pattern, less often autosomal recessive. In approximately 25% of cases, the mutation arises de novo (new mutation with no family history).
Symptoms
Clinical severity varies widely, from asymptomatic to severe anemia. Common symptoms include:
- Anemia: fatigue, pallor, reduced physical performance
- Jaundice: elevated bilirubin levels due to accelerated red blood cell breakdown
- Splenomegaly (enlarged spleen): caused by increased destruction of spherocytes
- Gallstones (cholelithiasis): due to excess bilirubin production
- Hemolytic crises: acute worsening of anemia, often triggered by infections
- Aplastic crises: temporary failure of bone marrow red cell production, frequently caused by Parvovirus B19 infection
Diagnosis
Diagnosis is based on clinical findings, laboratory parameters, and specific diagnostic tests:
- Blood count and peripheral blood smear: identification of spherocytes, elevated mean corpuscular hemoglobin concentration (MCHC)
- Reticulocytosis: elevated reticulocyte count indicating compensatory increased red cell production
- Elevated indirect bilirubin and lactate dehydrogenase (LDH)
- Osmotic fragility test or eosin-5-maleimide (EMA) binding test: specific confirmatory tests
- Molecular genetic analysis: used in atypical or uncertain cases to identify the causative mutation
- Direct Coombs test: negative (to exclude autoimmune hemolytic anemia)
Treatment
Treatment depends on the severity of the disease:
Mild to Moderate Cases
- Regular medical follow-up and blood count monitoring
- Folic acid supplementation to support increased red blood cell production
Severe Cases
- Splenectomy (surgical removal of the spleen): significantly reduces spherocyte destruction and improves anemia; generally recommended after the age of 6 to minimize infection risk
- Post-splenectomy: lifelong vaccination (e.g., against pneumococci, meningococci, and Haemophilus influenzae type b) and, in some cases, antibiotic prophylaxis
- Blood transfusions: required during severe hemolytic or aplastic crises
Prognosis
With timely diagnosis and appropriate management, the prognosis is generally favorable. Many affected individuals lead a normal life. Following a successful splenectomy, symptoms of anemia typically resolve completely, although spherocytes remain in the blood. Regular follow-up is important to detect and manage potential complications such as gallstones.
References
- Eber S, Lux SE. Hereditary Spherocytosis -- Defects in Proteins That Connect the Membrane Skeleton to the Lipid Bilayer. Seminars in Hematology. 2004;41(2):118-141.
- Bolton-Maggs PHB, Langer JC, Iolascon A, et al. Guidelines for the diagnosis and management of hereditary spherocytosis -- 2011 update. British Journal of Haematology. 2012;156(1):37-49.
- World Health Organization. ICD-10 International Statistical Classification of Diseases and Related Health Problems, 10th Revision -- Code D58.0. WHO, Geneva.
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