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Warm autoimmune hemolytic anemia

Warm Autoimmune Hemolytic Anemia (WAIHA)Warm autoimmune hemolytic anemia (WAIHA) is a type of autoimmune hemolytic anemia (AIHA), a condition where the body’s immune system mistakenly attacks and destroys its own red blood cells. In WAIHA, this destruction of red blood cells (hemolysis) occurs at body temperature (around 37°C or 98.6°F), hence the term "warm."

Warm Autoimmune Hemolytic Anemia (WAIHA)

Warm autoimmune hemolytic anemia (WAIHA) is a type of autoimmune hemolytic anemia (AIHA), a condition where the body’s immune system mistakenly attacks and destroys its own red blood cells. In WAIHA, this destruction of red blood cells (hemolysis) occurs at body temperature (around 37°C or 98.6°F), hence the term "warm."

Causes of WAIHA

WAIHA occurs when the body’s immune system produces antibodies that target red blood cells. These antibodies are typically IgG (immunoglobulin G), which are active at warm temperatures (body temperature). The exact cause of these antibodies is often unknown, but there are several factors and conditions associated with WAIHA:

  1. Primary (Idiopathic) WAIHA:
    • No obvious underlying cause, and the condition appears without a known trigger.
  2. Secondary WAIHA:
    • The anemia is caused by another underlying condition, including:
      • Autoimmune diseases: Such as lupus (SLE), rheumatoid arthritis, or other connective tissue disorders.
      • Infections: Like Epstein-Barr virus (EBV), HIV, or cytomegalovirus (CMV).
      • Cancer: Especially lymphomas or leukemias.
      • Medications: Some drugs, like penicillin or cephalosporins, can trigger the immune system to produce antibodies that attack red blood cells.

Symptoms of WAIHA

Symptoms of warm autoimmune hemolytic anemia are related to the destruction of red blood cells, which leads to reduced oxygen-carrying capacity of the blood. Common symptoms include:

  • Fatigue: Due to decreased red blood cell count.
  • Paleness (Pallor): A common sign of anemia.
  • Shortness of breath: As the body struggles to oxygenate tissues.
  • Jaundice: Yellowing of the skin and eyes due to the increased breakdown of hemoglobin from destroyed red blood cells.
  • Dark-colored urine: Often caused by the release of hemoglobin into the bloodstream, which is then filtered by the kidneys.
  • Enlarged spleen (splenomegaly): The spleen works harder to remove damaged red blood cells.
  • Heart palpitations: Resulting from the body’s effort to compensate for low red blood cell counts.

Diagnosis of WAIHA

To diagnose WAIHA, a combination of tests are typically used:

  1. Blood Tests:
    • Complete Blood Count (CBC): To check for anemia (low red blood cells, hemoglobin, or hematocrit).
    • Reticulocyte Count: Elevated in response to anemia, as the bone marrow tries to compensate for red blood cell loss.
    • Peripheral Blood Smear: To look for signs of hemolysis and abnormal red blood cells.
    • Coombs' Test (Direct Antiglobulin Test): This is the primary diagnostic test for autoimmune hemolytic anemia. A positive result indicates that antibodies are attached to the red blood cells.
  2. Other Tests:
    • Liver Function Tests and Bilirubin Levels: Elevated indirect bilirubin levels may indicate increased red blood cell destruction.
    • Kidney Function Tests: To assess for damage due to hemoglobin in the urine.

Treatment of WAIHA

Treatment focuses on controlling the immune system’s attack on red blood cells and managing anemia symptoms.

  1. Corticosteroids (Prednisone):
    • The first line of treatment for most people with WAIHA. Steroids help suppress the immune response and decrease the destruction of red blood cells.
  2. Immunosuppressive Drugs:
    • If corticosteroids alone are not effective, other immunosuppressive drugs (e.g., azathioprine, cyclophosphamide) may be used to further suppress the immune system.
  3. Rituximab:
    • A monoclonal antibody that targets and destroys B cells, which are responsible for producing antibodies. It is often used in refractory cases.
  4. Splenectomy:
    • Removal of the spleen may be considered in severe cases, as the spleen is the main site for red blood cell destruction. This can help reduce symptoms but is typically considered when other treatments fail.
  5. Blood Transfusions:
    • In cases of severe anemia, blood transfusions may be necessary to stabilize the patient and increase red blood cell count.
  6. Other Therapies:
    • Eculizumab: In rare cases, a monoclonal antibody used for autoimmune hemolytic anemia that targets the complement system.

Prognosis

The prognosis for WAIHA varies depending on the severity of the condition and the response to treatment. Some patients may experience long periods of remission with proper management, while others may have recurrent episodes of hemolysis. In secondary cases, addressing the underlying cause (such as treating an autoimmune disease or infection) can improve outcomes.

In general, with appropriate treatment, most people with WAIHA can manage their symptoms effectively, although long-term monitoring may be required due to the potential for relapse or complications related to treatments.

Complications

Without proper treatment, WAIHA can lead to:

  • Severe anemia: Which may lead to organ damage and heart failure if not treated.
  • Infections: Due to immunosuppressive treatments.
  • Gallstones: Resulting from the increased breakdown of red blood cells and the resulting elevated bilirubin levels.

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