A life-threatening paediatric case of acute autoimmune haemolytic anaemia (AIHA) successfully cured by plasma-exchange and combined immunosuppressive treatment.

A 3-year-old boy present with a severe autoimmune haemolytic anaemia, triggered by IgG-class auto-antibodies, with hemoglobin levels decreased to 2, 1 gr/dL. A combined immunosuppressive regimen was begun together with multiple plasma-exchanges and transfusions which sustained the cardio-vascular balance until the specific therapy became effective.

A persistent severe autoimmune hemolytic anemia despite apparent direct antiglobulin test negativization.

BACKGROUND AND OBJECTIVE: Not all cases of autoimmune hemolytic anemia (AIHA) are diagnosed by the direct antiglobulin test (DAT). We present and discuss a simple method of enhancing the sensitivity of the standard DAT. DESIGN AND METHODS: We report the case of a five-month-old child diagnosed with a severe IgG-mediated AIHA, characterized by quick DAT negativization despite clinical worsening. Warm AIHA with negative DAT, possibly due to a low affinity autoantibody, unresponsive to conventional therapy, was hypothesized. RESULTS: The DAT resulted strongly positive with anti-IgG serum using a 4C saline for erythrocyte washing, to reduce the dissociation of the supposed low affinity autoantibody. Very intensive cytoreductive treatment was administered twice until clinical remission was obtained. INTERPRETATION AND CONCLUSIONS: The clinical course of AIHA can be dissociated by the DAT. Since autoantibody-mediated hemolysis with negative DAT rarely occurs, once other causes of high reticulocyte count anemia have been ruled out, the DAT after ice-cold saline washing could be a useful and easy means of corroborating the diagnosis of AHIA, when traditional methods fail.

Granulocyte serology findings in juvenile symptomatic idiopathic autoimmune neutropenia using a multiassay procedure. Report on 21 cases.

The serological findings on 21 children with idiopathic autoimmune neutropenia are reported. A multiassay procedure was adopted including agglutination, immunofluorescence and cytotoxicity tests. Beside a cause-effect correlation between granulocyte antibodies and clinical course, a serologic polymorphism was found. As the sensitivity of each assay seemed to be related to the characteristics of the involved antigens and antibodies, as well as to the intrinsic sensitivity of the tests, the performance of a multiassay procedure appears to be advisable for the diagnosis and the follow-up of autoimmune neutropenia.

Allo and auto-antibodies against platelet antigens: a new immunoenzymatic assay for their detection and quantitation.

A new immunoenzymatic assay for the detection of serum-free and platelet-associated (PA) antibodies is described. This method evidences the platelet bound IgG employing the biotin-avidin system. Following a common sensitization step, the PA-IgG can be directly detected on the cell membranes, and their amount can be evaluated by an anti-globulin consumption assay. In comparison with the platelet suspension immunofluorescence test (PSIFT), the ELISA assay is a little less specific but it is more sensitive: less than 1 ng/ml of IgG can be detected. The number of IgG molecules/platelet detected investigating normal and pathological sera is higher than that reported by others: such results are probably related to the nonhomogeneity of immunoglobulins implicated in the assay.

Neonatal thrombocytopenic purpura: case reports and methodological approach.

Six emblematic cases of neonatal thrombocytopenic purpura (NTP) due to different etiologic factors are described. Various methods for the detection of platelet and leukocyte antibodies are considered: platelet suspension and lymphocyte immunofluorescence tests (PSIFT and LIFT), solid-phase platelet antibody detection test (Capture-P), immune-enzymatic assay on platelet suspension (ELISA) and on solid-phase platelets (Capture-P-ELISA), lymphocytotoxicity (Lctx). Furthermore, chloroquine stripping of HLA antigens and monoclonal antibody (MoAb) treatment of target platelets specific antigens are described. The technical characteristics and advantages of each method are discussed. The authors stress that a step-by-step investigation employing an integrated system that includes various methods can correctly supply the pathogenetic information necessary for the physician to undertake the best therapeutical approach.