Key Points• Mixed, atypical, and warm immunoglobulin G plus C AIHA (;30% of cases) more frequently have a severe onset (Hb #6 g/dL) and require multiple therapy lines.• Infections, particularly after splenectomy, acute renal failure, Evans syndrome, and multitreatment, were predictors of fatal outcome.The clinical outcome, response to treatment, and occurrence of acute complications were retrospectively investigated in 308 primary autoimmune hemolytic anemia (AIHA) cases and correlated with serological characteristics and severity of anemia at onset. Patients had been followed up for a median of 33 months (range 12-372); 60% were warm AIHA, 27% cold hemagglutinin disease, 8% mixed, and 5% atypical (mostly direct antiglobulin test negative). The latter 2 categories more frequently showed a severe onset (hemoglobin [Hb] levels £6 g/dL) along with reticulocytopenia. The majority of warm AIHA patients received first-line steroid therapy only, whereas patients with mixed and atypical forms were more frequently treated with 2 or more therapy lines, including splenectomy, immunosuppressants, and rituximab. The cumulative incidence of relapse was increased in more severe cases (hazard ratio 3.08; 95% confidence interval, 1.44-6.57 for Hb £6 g/dL; P < .001). Thrombotic events were associated with Hb levels £6 g/dL at onset, intravascular hemolysis, and previous splenectomy. Predictors of a fatal outcome were severe infections, particularly in splenectomized cases, acute renal failure, Evans syndrome, and multitreatment (4 or more lines). The identification of severe and potentially fatal AIHA in a largely heterogeneous disease requires particular experienced attention by clinicians. (Blood. 2014;

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Summary In 1999, we implemented an automated platelet cross‐matching (XM) programme to select compatible platelets from the local inventory for patients refractory to random donor platelets. In this study, we evaluated platelet count increments in 40 consecutive refractory patients (8·3% of 480 consecutive platelet recipients) given 569 cross‐match‐negative platelets between April 1999 and December 2001. XM was performed automatically with a commercially available immunoadherence assay. Pre‐, 1‐ and 24‐h post‐transfusion platelet counts (mean ± SD) for the 569 XM‐negative platelet transfusions containing 302 ± 71 × 109 platelets were 7·7 ± 5·5, 32·0 ± 21·0 and 16·8 ± 15·5 × 109/l respectively. Increments were significantly higher (P < 0·05, t‐test) than those observed in the same patients given 303 random platelet pools (dose = 318 ± 52 × 109 platelets) during the month before refractoriness was detected, when pre‐, 1‐ and 24‐h post‐transfusion counts were 7·0 ± 8·6, 15·9 ± 16·1 and 9·6 ± 12·8 × 109/l respectively. The cost of the platelet XM disposable kit per transfusion to produce 1‐h post‐transfusion platelet count increments >10 × 109/l was euro 447. This programme enabled the rapid selection of effective platelets for refractory patients, from the local inventory.

The diagnosis of autoimmune hemolytic anemia (AIHA) is based on a positive direct antiglobulin test (DAT), which is performed using various methods with different sensitivities. Recently, mitogen-stimulated (MS)-DAT was suggested to be able to identify latent anti-erythrocyte autoimmunity. Traditional methods (tube, microcolumn, and solid phase) and MS-DAT were compared in 54 consecutive cases of suspected AIHA, 28 idiopathic AIHA in clinical remission, and 12 difficult-to-diagnose cases of DAT-negative AIHA, and the results (all cases) were correlated with hematologic and hemolytic parameters. DAT tube was confirmed as the gold standard to diagnose AIHA since almost all positive cases showed hemolytic anemia and positive eluates; 10 out of 26 tube-negative cases were positive on microcolumn and solid phase antiglobulin tests, and 22 out of 26 using MS-DAT, although only half of them showed clear signs of hemolysis. Mitogen stimulation increased the amount of IgG bound to red blood cells in all groups; moreover, MS-DAT was the only positive test in 10 cases of AIHA, and mitogen stimulation facilitated the identification of autoantibody specificity in culture supernatants. We conclude that a battery of tests rather than a single test is useful for the diagnosis of AIHA, including MS-DAT as an additional test for selected cases, although the results have to be cautiously interpreted based on the overall clinical context.

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