bubble_chart Overview

This disease occurs due to immune dysfunction, which produces autoantibodies that bind to the surface of red blood cells or circulate freely in the serum. These antibodies sensitize the red blood cells or activate complement, leading to premature destruction of red blood cells and resulting in hemolytic anemia.

bubble_chart Diagnosis

(1) Medical History and Symptoms

(1) History Inquiry: Note: ① Presence of jaundice, history of blood transfusion; ② Whether there is painful acrocyanosis; ③ Whether suffering from lymphatic system tumors, myeloma, gastric adenocarcinoma, autoimmune diseases (systemic lupus erythematosus, rheumatoid arthritis, etc.), mycoplasma, viral infections, and syphilis, among others. ④ Use of medications such as penicillin, quinidine, quinine, methyldopa, etc.
(2) Clinical Symptoms: Dizziness, lack of strength, shortness of breath, palpitation; shivering, fever, lumbago, chest tightness (acute hemolysis).

(2) Physical Examination Findings

Anemic appearance, jaundice of the skin and sclera, grade I splenomegaly, some patients exhibit acrocyanosis (cold antibody type).

(3) Auxiliary Examinations

Blood Test: Decreased hemoglobin, presenting as normocytic normochromic anemia; normal white blood cells and platelets; increased reticulocytes, presence of microspherocytes, and observable erythroblasts.

Bone Marrow Examination: Hyperactive proliferation, notably of erythroblasts. Granulocytic and megakaryocytic series are normal.
Elevated indirect bilirubin in blood, reduced or absent serum haptoglobin, increased plasma free hemoglobin. Urine hemosiderin negative.

Direct Coombs test positive, indirect Coombs test positive or negative (warm antibody autoimmune hemolytic anemia).

Cold agglutinin test positive (cold agglutinin syndrome).

Donath-Landsteiner test positive, urine hemosiderin test positive (paroxysmal cold hemoglobinuria).

(4) Differential Diagnosis

1. Hereditary Spherocytosis: Family history; peripheral blood microspherocytes >10%; positive osmotic fragility test; autohemolysis test: hemolysis >5%.

2. Paroxysmal Nocturnal Hemoglobinuria: Positive acid hemolysis test, snake venom factor hemolysis test, and urine hemosiderin test.

3. Glucose-6-Phosphate Dehydrogenase Deficiency: Positive methemoglobin reduction test; fluorescent spot test and nitroblue tetrazolium paper method: decreased G6PD activity.

bubble_chart Treatment Measures

(1) Warm Antibody Autoimmune Hemolytic Anemia

For cases with a clear disease cause, actively treat the primary disease.

Glucocorticoids: Hydrocortisone: 400–600 mg/d, intravenous infusion, switch to prednisone: 1 mg/kg·d, orally, after 3–5 days. If the condition improves within 7–10 days and hemoglobin approaches normal, gradually reduce the prednisone dose by 10–15 mg per week until reaching 20 mg/d. Monitor hemoglobin and reticulocyte count every 2–3 weeks. If stable, reduce prednisone by 2.5 mg weekly to 5–10 mg/d, or maintain treatment with prednisone 10–20 mg every other day for 6 months.

Splenectomy: Consider if there is no improvement in hemolysis and anemia after 2 weeks of high-dose glucocorticoid therapy; if a high daily dose of prednisone (>15 mg) is required to maintain hematologic improvement; or if the patient cannot tolerate prednisone or immunosuppressive therapy or has contraindications. Before splenectomy, it is best to perform a 51Cr red cell lifespan and sequestration test to evaluate efficacy.

Immunosuppressants: For patients who do not achieve remission with glucocorticoids or splenectomy, have contraindications for splenectomy, or require a high daily dose of prednisone (>15 mg) to maintain hematologic improvement. Use azathioprine: 50–200 mg/d; or cyclophosphamide: 50–150 mg/d. After hematologic remission, first reduce the glucocorticoid dose, then taper the immunosuppressant to a maintenance dose, continuing treatment for 3–6 months. Monitor for side effects such as bone marrow suppression during treatment.

Others: ⑴ High-dose intravenous immunoglobulin: 0.4–1.0 g/kg for 5 consecutive days, which may be effective for a small subset of IgG-mediated immune hemolytic anemia, though the effect is short-lived. ⑵ Plasma exchange: Suitable for patients with high antibody titers and poor response to glucocorticoid therapy. ⑶ Blood transfusion: Whole blood or washed red blood cells may be transfused in cases of hemolytic crisis or severe anemia.

(2) Cold Antibody Autoimmune Hemolytic Anemia

Identify and treat any underlying disease. Avoid cold exposure and keep warm.

Immunosuppressants: For patients unresponsive to warmth and supportive therapy, use chlorambucil: 2–4 mg/d for 3–6 months; or cyclophosphamide: 100–150 mg/d for >3 months.