Atypical hemolytic-uremic syndrome

Atypical hemolytic-uremic syndrome is a disease that primarily affects kidney function. This condition, which can occur at any age, causes abnormal blood clots (thrombi) to form in small blood vessels in the kidneys. These clots can cause serious medical problems if they restrict or block blood flow. Atypical hemolytic-uremic syndrome is characterized by three major features related to abnormal clotting: hemolytic anemia, thrombocytopenia, and kidney failure.Hemolytic anemia occurs when red blood cells break down (undergo hemolysis) prematurely. In atypical hemolytic-uremic syndrome, red blood cells can break apart as they squeeze past clots within small blood vessels. Anemia results if these cells are destroyed faster than the body can replace them. This condition can lead to unusually pale skin (pallor), yellowing of the eyes and skin (jaundice), fatigue, shortness of breath, and a rapid heart rate.Thrombocytopenia is a reduced level of circulating platelets, which are cell fragments that normally assist with blood clotting. In people with atypical hemolytic-uremic syndrome, fewer platelets are available in the bloodstream because a large number of platelets are used to make abnormal clots. Thrombocytopenia can cause easy bruising and abnormal bleeding.As a result of clot formation in small blood vessels, people with atypical hemolytic-uremic syndrome experience kidney damage and acute kidney failure that lead to end-stage renal disease (ESRD) in about half of all cases. These life-threatening complications prevent the kidneys from filtering fluids and waste products from the body effectively.Atypical hemolytic-uremic syndrome should be distinguished from a more common condition called typical hemolytic-uremic syndrome. The two disorders have different causes and different signs and symptoms. Unlike the atypical form, the typical form is caused by infection with certain strains of Escherichia coli bacteria that produce toxic substances called Shiga-like toxins. The typical form is characterized by severe diarrhea and most often affects children younger than 10. The typical form is less likely than the atypical form to involve recurrent attacks of kidney damage that lead to ESRD.

Different Conditions


Related ICD 10 COde
ICD 10 Code D59.3


Thrombotic microangiopathy, not elsewhere classified

A disease caused by acquired haematopoietic stem cell mutation defect of the PIGA gene in the X chromosome leading to the premature break down of red blood cells. This disease is characterised by haemolytic anaemia, thrombosis and. This disease may present with haemoglobinuria (blood in the urine will be most noticeable in the morning, and clear as the day progresses), pallor, fatigue, or shortness of breath. Confirmation is by identification of genetic mutation in the PIGA gene through genetic testing.

Signs And Symptoms

  • Anemia (disorder)
  • Organ Affected

    Blood (substance)

    Also Known As

  • Typical haemolytic uraemic syndrome
  • Gasser syndrome
  • Related ICD 10 COde
    ICD 10 Code D59.3

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