Abstract
Autoimmune hemolytic anemia (AIHA) is diagnosed in the presence of anemia, hemolysis, and direct antiglobulin test (DAT) positivity with monospecific antisera. Many confounders of anemia and hemolytic markers should be included in the initial workup (i.e., nutrients deficiencies, chronic liver or kidney diseases, infections, and cancers). Besides classical presentation, there are difficult cases that may challenge the treating physician. These include DAT negative AIHA, diagnosed after the exclusion of other causes of hemolysis, and supported by the response to steroids, and secondary cases (infections, drugs, lymphoproliferative disorders, immunodeficiencies, etc.) that should be suspected and investigated through careful anamnesis physical examination, and specific tests in selected cases. The latter include autoantibody screening in patients with signs/symptoms of systemic autoimmune diseases, immunoglobulins (Ig) levels in case of frequent infections or suspected immunodeficiency, and ultrasound/ computed tomography (CT) studies and bone marrow evaluation to exclude hematologic diseases. AIHA occurring in pregnancy is a specific situation, usually manageable with steroids and intravenous (iv) Ig, although refractory cases have been described. Finally, AIHA may complicate specific clinical settings, including intensive care unit (ICU) admission, reticulocytopenia, treatment with novel anti-cancer drugs, and transplant. These cases are often severe, more frequently DAT negative, and require multiple treatments in a short time.
Highlights
Autoimmune hemolytic anemia (AIHA) is a rare disease caused by an autoimmune attack against red blood cells
Among host-related secondary causes of AIHA, various immunodeficiencies have been identified as predisposing conditions, including common variable immunodeficiency (CVID) [16], IgA deficiency, and autoimmune lymphoproliferative syndromes (ALPS) [17]
The correct diagnosis is important since the patient might be handled with iv Ig cycles, as in the cited example, and other immunosuppressive treatments may lead to severe infections and be contraindicated
Summary
Autoimmune hemolytic anemia (AIHA) is a rare disease caused by an autoimmune attack against red blood cells. The disease is classified in warm (wAIHA, 48–70% of cases) and cold forms (cAIHA, 15–25% of cases) basing on the optimal temperature of activity of the autoantibodies and their isotypes. AIHA may be secondary to a variety of conditions including systemic autoimmune diseases (i.e., 10% of cases with systemic lupus erythematosus), and lymphoproliferative syndromes, chronic lymphocytic leukemia (5–10%) [5,6,7,8]. All types of AIHA can be acute and transient, or chronic with multiple relapses and therapy lines. At onset and in the acute setting, AIHA may present to the emergency room and is usually admitted to the general ward becoming a challenge for the internal medicine specialist. AIHA presentation and diagnosis and will focus on the difficult cases from both a diagnostic and therapeutic point of view
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