Microangiopathic hemolytic anemia

Microangiopathic hemolytic anemia (MAHA) is a subtype of hemolytic anemia, a condition characterized by the premature destruction of red blood cells (RBCs) in the circulatory system. In MAHA, the destruction of RBCs is caused by their passage through small, damaged blood vessels.

Etiology

MAHA is often associated with a variety of conditions and diseases, including thrombotic microangiopathies such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), malignant hypertension, scleroderma, and disseminated intravascular coagulation (DIC).

Pathophysiology

In MAHA, the underlying disease or condition causes damage to the endothelial lining of the small blood vessels. As RBCs pass through these damaged vessels, they are subjected to shear stress and become fragmented. This fragmentation results in the formation of schistocytes, or fragmented RBCs, which are a characteristic feature of MAHA.

Clinical Features

Patients with MAHA often present with signs and symptoms of anemia, such as fatigue, pallor, and shortness of breath. In addition, they may also exhibit signs of the underlying disease or condition causing the MAHA. For example, patients with TTP may present with neurological symptoms, while those with HUS may have renal symptoms.

Diagnosis

The diagnosis of MAHA is typically based on the presence of anemia, thrombocytopenia, and schistocytes on a peripheral blood smear. Additional diagnostic tests may be performed to identify the underlying cause of the MAHA.

Treatment

The treatment of MAHA is primarily aimed at managing the underlying cause. This may involve the use of medications, plasmapheresis, or in some cases, surgical intervention.

See Also

• Anemia
• Hemolytic anemia
• Thrombotic microangiopathy
• Thrombotic thrombocytopenic purpura
• Hemolytic uremic syndrome