Positive autocontrol minor incompatibility in pure red cell aplasia patient: A case report

Abstract

Introduction: Pure red cell aplasia (PRCA) is a syndrome of normochromic normocytic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors in the bone marrow. The serum of PRCA patients often contains immunoglobulin G (IgG) antibodies that suppress the growth of erythroid progenitor cells in vitro. This report focused on the diagnosis and management of a PRCA case.

Case Description: A 5-year-old boy presented with complaints of pallor for two days and generalized weakness for five days. Medical history included a tuberculosis infection, for which he underwent treatment with anti-tuberculosis drugs, including rifampicin and isoniazid. He had a history of recurrent pallor and multiple hospitalizations due to decreased hemoglobin levels, requiring routine blood transfusions since 2018 at Wahidin Sudirohusodo Hospital. Laboratory investigations revealed normochromic normocytic anemia, severe reticulocytopenia, and bone marrow aspiration findings of normocellular marrow with significantly reduced erythropoiesis activity. Crossmatch testing showed minor incompatibility (1+) and autocontrol positivity (1+), with the degree of minor incompatibility matching the positive autocontrol results, indicative of erythrocytes coated with autoantibodies. Monoclonal Direct Coombs Test (DCT) findings included IgG positivity (1+) and C3d negativity. Packed red cell (PRC) transfusion was successfully administered under medical supervision.

Conclusion: This case highlights a 5-year-old boy diagnosed with pure red cell aplasia based on clinical presentation, physical examination findings, and laboratory investigations. The patient's treatment involved blood transfusion, which was administered with appropriate precautions to manage the underlying anemia effectively.