Other hereditary hemolytic anemias
Chapter 3:Diseases of the blood and blood-forming organs
ICD-10 D58 is a billable code used to indicate a diagnosis of other hereditary hemolytic anemias.
Hereditary hemolytic anemias encompass a diverse group of genetic disorders characterized by the premature destruction of red blood cells (RBCs). These conditions can arise from various enzyme deficiencies, such as glucose-6-phosphate dehydrogenase (G6PD) deficiency, which leads to oxidative stress and hemolysis. Thalassemias, another subset of hereditary hemolytic anemias, involve defects in hemoglobin synthesis, resulting in ineffective erythropoiesis and hemolysis. Sickle cell disease, a well-known hereditary condition, is caused by a mutation in the beta-globin gene, leading to the production of abnormal hemoglobin (HbS) that distorts RBCs into a sickle shape, causing vaso-occlusive crises and hemolysis. Genetic factors play a crucial role in these disorders, with inheritance patterns varying from autosomal recessive to X-linked. Clinically, patients may present with symptoms such as fatigue, pallor, jaundice, and splenomegaly. Diagnosis typically involves a combination of clinical evaluation, laboratory tests including complete blood count (CBC), reticulocyte count, and specific hemoglobin electrophoresis or enzyme assays to identify the underlying cause of hemolysis.
Detailed family history, laboratory test results, and clinical findings.
Diagnosis and management of patients with hereditary hemolytic anemias, including G6PD deficiency and thalassemia.
Ensure accurate documentation of the specific type of hemolytic anemia and any associated complications.
Genetic testing results, family pedigree, and clinical evaluation.
Counseling patients with hereditary hemolytic anemias and their families regarding inheritance patterns and risks.
Documentation should include genetic counseling notes and recommendations for family testing.
Used to evaluate anemia and hemolysis in patients suspected of having hereditary hemolytic anemia.
Document the indication for the CBC and reticulocyte count.
Hematologists may require additional tests based on initial findings.
Hereditary hemolytic anemia is caused by genetic mutations affecting red blood cell stability, while acquired hemolytic anemia results from external factors such as infections, autoimmune diseases, or certain medications.
Document the specific type of hereditary hemolytic anemia, relevant family history, laboratory findings, and any associated symptoms to ensure accurate coding.
