What is the diagnosis and treatment for Hemolytic (breakdown of red blood cells) anemia?

From the Guidelines

Hemolytic anemia is a condition where red blood cells are destroyed faster than they can be produced, and treatment depends on the underlying cause, with the goal of reducing morbidity, mortality, and improving quality of life. The diagnosis of hemolytic anemia involves identifying the underlying cause, which may be inherited, such as pyruvate kinase deficiency, or acquired, such as autoimmune hemolytic anemia.

• The clinical manifestations of pyruvate kinase deficiency, a common cause of chronic hereditary nonspherocytic hemolytic anemia, comprise the usual hallmarks of lifelong chronic hemolysis, including anemia, jaundice, and splenomegaly 1.
• The degree of anemia varies widely, ranging from very mild anemia or fully compensated hemolysis to nonimmune hydrops fetalis or life-threatening neonatal anemia, and patients may present any time from very early in life to in young adulthood 1.
• Treatment for pyruvate kinase deficiency may include splenectomy, which can increase hemoglobin levels and reduce transfusion requirements, as well as supportive care, such as blood transfusions and folic acid supplementation 1.
• For autoimmune hemolytic anemia, first-line treatment typically includes prednisone at 1-2 mg/kg/day for 3-4 weeks, followed by a slow taper, and in severe cases, intravenous immunoglobulin (IVIG) at 1 g/kg for 2 days may be necessary 1.
• For patients with hemolytic anemia, particularly those with pyruvate kinase deficiency or autoimmune hemolytic anemia, prompt treatment is crucial to prevent long-term complications, such as gallstones, pulmonary hypertension, and thrombosis, and to improve quality of life, with the goal of reducing morbidity and mortality 1.
• Supportive care includes blood transfusions for severe anemia (hemoglobin <7 g/dL), folic acid 1 mg daily to support increased red cell production, and treating underlying conditions, and patients should be monitored with regular complete blood counts, reticulocyte counts, and liver function tests 1.
• In cases of delayed hemolytic transfusion reactions (DHTRs), immunosuppressive therapy, such as IVIg, high-dose steroids, and eculizumab, may be necessary to prevent further hemolysis and improve outcomes 1.

From the FDA Drug Label

8 Hematologic Disorders Idiopathic thrombocytopenic purpura in adults Secondary thrombocytopenia in adults Acquired (autoimmune) hemolytic anemia Erythroblastopenia (RBC anemia) Congenital (erythroid) hypoplastic anemia

The diagnosis of Hemolytic anemia is Acquired (autoimmune) hemolytic anemia. The treatment for Hemolytic anemia is prednisone (PO), as it is indicated for acquired (autoimmune) hemolytic anemia 2. Key points:

• The diagnosis is based on the presence of autoimmune hemolytic anemia.
• The treatment involves the use of prednisone (PO) to manage the condition.

From the Research

Diagnosis of Hemolytic Anemia

• Hemolytic anemia is a disease caused by autoantibodies, resulting in various complaints and clinical symptoms 3
• The diagnosis of autoimmune hemolytic anemia (AIHA) involves a monospecific direct antiglobulin test, which is considered mandatory in the diagnostic workup 4
• Exact diagnostic workup is important because symptoms, course of disease, and therapeutic management relate to the type of antibody involved 4

Treatment Options for Hemolytic Anemia

• Corticosteroids are the first-line treatment option for warm autoantibody-related hemolytic anemia 3, 4
• In patients who develop steroid side effects or do not respond adequately, other immunosuppressives may be preferred 3
• Rituximab may be used instead of splenectomy in therapy-refractory patients 5, 6
• Plasma exchange (PE) may be utilized in severe and refractory anemia, especially in the case of cardiovascular imbalance in fulminant hemolysis 3
• Blood transfusion is the most rapidly effective measure for patients who develop or may develop hypoxic anemia 5

Novel Therapies for Hemolytic Anemia

• Novel therapies, including B-cell-directed therapy, phagocytosis inhibition, plasma cell-directed therapy, and complement inhibition, have become promising for AIHA patients 7
• Rituximab, an anti-CD20 chimeric monoclonal antibody, can effectively deplete B-cells and is commonly used in B-cell non-Hodgkin lymphoma and autoimmune disorders 6