Pure red cell aplasia in a patient with adult‐onset Still's disease

Recurrent transfusion-related acute lung injury after fresh frozen plasma in a patient with hereditary factor V deficiencyTo the Editor: Transfusion-related acute lung injury (TRALI) can be a serious complication resulting from the transfusion of plasma that contains antibodies against recipient white blood cells.There have been only four previously reported cases of recurrent TRALI [1][2][3].We describe a patient with recurrent TRALI, emphasizing potential strategies to prevent this disease.A 25-year-old man with hereditary factor V deficiency and a baseline FV level of <2% received five units of fresh frozen plasma after a spontaneous axillary hematoma and developed TRALI.The patient recovered fully and was discharged 48 hr later.Five months later, he received two units of plasma after a trauma-induced right foot hematoma and suffered a recurrent episode of TRALI.Donors associated with the initial and recurrent TRALI episodes were tested for anti-HLA class I, anti-HLA class II, and antigranulocyte antibodies.Sera from three female donors from the first transfusion episode and one female donor from the second transfusion episode showed antibodies against HLA class I, class II, or both (Table I).None showed antigranulocyte antibodies.The patient could not be HLA-typed because of relocation.Both TRALI episodes of our patient met American-European Consensus Conference criteria, including a new episode of acute lung injury occurring within 6 hr of transfusion, bilateral pulmonary infiltrates on chest X-ray, and no evidence of an alternative risk factor for acute lung injury nor of circulatory overload [4].Our patient was in good health, did not have any of the known associated antecedent ''first events'' such as major surgery, active infection, or massive transfusion, and the presence of anti-HLA antibodies in the transfused plasma appeared adequate, in itself, to cause the clinical entity.Our case study emphasizes the observation that the risk of recurrent TRALI may persist for longer periods (months, years) than previously thought, and raises the question of whether certain previously unrecognized populations (such as patients with FV deficiency) are vulnerable to TRALI.Our patient received 309 plasma units over a 13-year period, and the only registered transfusion reactions are the recurrent TRALI episodes reported herein.This amounts to an observed prevalence of $1 TRALI episode per 150 units, which is higher than the estimated risk for the general population (1:1,120 to 1:5,000).Strategies to reduce TRALI, such as the British experience using male gender plasma, suggest a positive impact of this strategy in decreasing the incidence of TRALI [5].On the other hand, it must be recognized that some recent observations do not support the disqualification of multiparous female donors, and such a policy may prove problematic for blood centers in which this population constitutes up to 30% of the donor pool [6].On account of this, our approach in this patient, and in Rhode Island, is be to screen donors at risk for alloimmunization to HLA antigens (previously pregnant or transfused) and exclude any donations from positive donors in the manufacture of high, plasma-volume components.Our report suggests that the susceptibility to recurrent TRALI merits consideration in ostensibly healthy persons and supports TRALI risk attenuated products such as male donor plasma or plasma from HLA antibody-screened females in patients with hereditary protein deficiencies requiring plasma for prophylaxis or treatment.

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