Lਅਔਔਅ ਔਏ ਔਈਅ Eਉਔਏ IgA autoimmune haemolytic anaemia in a pregnant woman Maria A. Villa 1, Norma N. Fantini 1, Nicoletta Revelli 1, Barbara Acaia 2, Cinzia Paccapelo 1, Maria C. Manera1, Maurizio Marconi1 1 Immunohaematology Reference Laboratory, Transfusion Centre; 2Obstetrics and Gynaecology Unit, IRCCS Ca' Granda Foundation, Maggiore Hospital, Milan, Italy
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column test (BIORAD, Cressier sur Morat, Swiss) was positive (score 2+) only with anti-IgA antiglobulin. The autoantibodies eluted from the patient's RBC (Elu-Kit II, Gamma) showed anti-e specificity. Irregular antibody screening and identification were performed by the indirect antiglobulin test in a microcolumn card (Ortho) and tube test with additive polyethylene glycol (PeG, Gamma) using anti-IgG and anti-IgA antiglobulin reagents. Free antibodies were not detected in the serum. The data led to the diagnosis of IgA-AIHA. An ultrasound of the foetus showed no abnormalities. The patient was treated with intravenous corticosteroids from day 10 to 113, with a starting daily dose of 80 mg/kg for 5 days, followed by tapering to 2.5 mg from day 83 to 113. Moreover, from day 17 to 21 the patient received 400 mg/kg/die of intravenous immunoglobulins to reduce the risk of complications due to the high steroid dosage in pregnancy (preterm premature rupture of the membranes, gestational diabetes and hypertension). The haemoglobin level began to rise after the administration of steroids and these treatments improved the patient's condition. The laboratory data are reported in Figures 1 and 2. One month after admission the patient was discharged. At the end of gestation she delivered a healthy male neonate. A maternal DAT with anti-IgA reagent was still weakly reactive with the gel column test (1+) and free antibodies were not detected in the serum with anti-IgG and anti-IgA antiglobulin reagents. AIHA can be a very severe disease, if not promptly detected and correctly treated. However, in pregnancy the presence of maternal autoantibodies may have little relevance for the foetus 1. Despite this, the correct identification of the presence of maternal autoantibodies is important for the differential diagnosis from several autoimmune conditions. For this reason, the Immunohaematology Laboratory must ensure that the techniques used for the patient's workup include several methods with appropriate sensitivity, such as a monocyte monolayer assay, eluate concentration, the direct Polybrene test, the direct polyethylene glycol test, solid-phase, gel column test, DAT using cold washes and the mitogen-stimulated DAT. In rare cases, warm AIHA can be associated with IgA or IgM autoantibodies without IgG being present. The presence of more than
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Dear Sir, Autoimmune haemolytic anaemia (AIHA) is a clinical condition caused by IgG, IgM or IgA antibodies to red blood cells. This condition affects 1-3 per 100,000 individuals per year1. Although IgA antibodies have been reported in 14% of cases of warm AIHA, they are mostly associated with IgG and/or IgM antibodies, while warm AIHA due exclusively to IgA antibodies is rare2. Several methods have been developed for the detection of these auto-antibodies. The direct antiglobulin test (DAT) by the conventional tube technique (CTT) is the gold standard. About 12% of AIHA patients show a negative CTT-DAT, possibly caused by the level of RBC-bound immunoglobulin being below the threshold, the presence of low-affinity IgG that washes off the RBC during the CTT-DAT washing phase and RBC-bound IgA or IgM not detected by routine antiglobulin reagents. In such cases, it is important to use additional tests to characterise the autoantibody and to confirm the diagnosis. We report an unusual case of IgA-AIHA in a pregnant woman not detected by CTT-DAT. A 32-year old woman in the 21st week of gestation of her second pregnancy was admitted to our hospital because of asthenia, headache and anaemia. The day before, the patient had received two units of RBC in another hospital. The patient's history was negative for chronic anaemia, medication use and infectious diseases. Laboratory results were as follows: haemoglobin 6.2 g/dL, reticulocytes 340×109/L, white blood cells 157×109/L, haptoglobin
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column test (BIORAD, Cressier sur Morat, Swiss) was positive (score 2+) only with anti-IgA antiglobulin. The autoantibodies eluted from the patient's RBC (Elu-Kit II, Gamma) showed anti-e specificity. Irregular antibody screening and identification were performed by the indirect antiglobulin test in a microcolumn card (Ortho) and tube test with additive polyethylene glycol (PeG, Gamma) using anti-IgG and anti-IgA antiglobulin reagents. Free antibodies were not detected in the serum. The data led to the diagnosis of IgA-AIHA. An ultrasound of the foetus showed no abnormalities. The patient was treated with intravenous corticosteroids from day 10 to 113, with a starting daily dose of 80 mg/kg for 5 days, followed by tapering to 2.5 mg from day 83 to 113. Moreover, from day 17 to 21 the patient received 400 mg/kg/die of intravenous immunoglobulins to reduce the risk of complications due to the high steroid dosage in pregnancy (preterm premature rupture of the membranes, gestational diabetes and hypertension). The haemoglobin level began to rise after the administration of steroids and these treatments improved the patient's condition. The laboratory data are reported in Figures 1 and 2. One month after admission the patient was discharged. At the end of gestation she delivered a healthy male neonate. A maternal DAT with anti-IgA reagent was still weakly reactive with the gel column test (1+) and free antibodies were not detected in the serum with anti-IgG and anti-IgA antiglobulin reagents. AIHA can be a very severe disease, if not promptly detected and correctly treated. However, in pregnancy the presence of maternal autoantibodies may have little relevance for the foetus 1. Despite this, the correct identification of the presence of maternal autoantibodies is important for the differential diagnosis from several autoimmune conditions. For this reason, the Immunohaematology Laboratory must ensure that the techniques used for the patient's workup include several methods with appropriate sensitivity, such as a monocyte monolayer assay, eluate concentration, the direct Polybrene test, the direct polyethylene glycol test, solid-phase, gel column test, DAT using cold washes and the mitogen-stimulated DAT. In rare cases, warm AIHA can be associated with IgA or IgM autoantibodies without IgG being present. The presence of more than
©
SI
M
TI S
er v
Dear Sir, Autoimmune haemolytic anaemia (AIHA) is a clinical condition caused by IgG, IgM or IgA antibodies to red blood cells. This condition affects 1-3 per 100,000 individuals per year1. Although IgA antibodies have been reported in 14% of cases of warm AIHA, they are mostly associated with IgG and/or IgM antibodies, while warm AIHA due exclusively to IgA antibodies is rare2. Several methods have been developed for the detection of these auto-antibodies. The direct antiglobulin test (DAT) by the conventional tube technique (CTT) is the gold standard. About 12% of AIHA patients show a negative CTT-DAT, possibly caused by the level of RBC-bound immunoglobulin being below the threshold, the presence of low-affinity IgG that washes off the RBC during the CTT-DAT washing phase and RBC-bound IgA or IgM not detected by routine antiglobulin reagents. In such cases, it is important to use additional tests to characterise the autoantibody and to confirm the diagnosis. We report an unusual case of IgA-AIHA in a pregnant woman not detected by CTT-DAT. A 32-year old woman in the 21st week of gestation of her second pregnancy was admitted to our hospital because of asthenia, headache and anaemia. The day before, the patient had received two units of RBC in another hospital. The patient's history was negative for chronic anaemia, medication use and infectious diseases. Laboratory results were as follows: haemoglobin 6.2 g/dL, reticulocytes 340×109/L, white blood cells 157×109/L, haptoglobin
