Learn About Sea-Blue Histiocytosis

What is the definition of Sea-Blue Histiocytosis?
Sea-Blue histiocytosis, also known as inherited lipemic splenomegaly, is an extremely rare condition characterized by elevated triglyceride levels (hypertriglyceridemia) and an enlarged spleen (splenomegaly). The disorder is so named because certain white blood cells, known as histiocytes, appear bright blue when stained and viewed under the microscope. Additional signs and symptoms may include a low platelet count (thrombocytopenia), liver function abnormalities, and heart disease. It is one of a group of related fat (lipid) disorders caused by certain changes in the APOE gene. The genetic change associated with this condition is inherited in an autosomal dominant manner though other factors, such as a patient's gender, the patient's lipid levels, and the genetic makeup of the other APOE gene may play a role in how the condition is expressed.
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What are the alternative names for Sea-Blue Histiocytosis?
  • Sea-Blue histiocytosis
  • Histiocytosis, sea-blue
  • Inherited Lipemic Splenomegaly
  • Sea-Blue histiocyte disease
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Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

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