Serum immunoglobulin free light chains in severe forms of atopic dermatitis.

An increase in immunoglobulin free light chains (FLC) was recently described in several pathological conditions, including asthma. FLC pathology is classically associated with monoclonal gammopathies. Its association with allergic disorders is surprising and unexplained. We therefore tested a cohort of children with severe atopic dermatitis (SCORAD 50-80) to determine the serum levels of free kappa and lambda chains, and correlated the results with clinical status and relevant laboratory markers. Seventy-three patients with severe forms of AD, all children from 3 months to 3 years of age and ninety healthy age-matched controls were included in the study. Light chains in sera were tested using the Freelite assay (Binding Site, Birmingham, UK). There were highly significant differences in both kappa (mean: 7.05 and 3.22 mg/l) and lambda (mean: 10.99 and 9.8 mg/l) serum levels between patients and controls, respectively (P < 0.0001). The kappa/lambda ratio in patients with allergy (mean: 0.64) was significantly higher than in controls (0.33) (P < 0.0001). We further observed significantly increased levels of FLC and their ratio in the group of patients with severe forms of AD in comparison to the group of patients with a resting stage of the disease or healthy controls (P < 0.05 and P < 0.0001, respectively). On the other hand, we could not confirm any association of FLC levels with age or total IgE levels. In conclusion, an increase in FLC reflects disease activity in children with severe atopic dermatitis. FLC might thus represent an additional diagnostic marker independent of total IgE levels.

[Determination of soluble HLA class I molecules in children in bone marrow transplantation]. / Vyuzití vysetrení solubilních HLA molekul I. trídy u detí pri transplantaci kostní drene.

BACKGROUND: sHLA molecules are the soluble forms of their membrane bound counterparts. sHLA class I. were recently reported to be a useful marker in the prediction of graft versus host reaction (GVHR) in adults. To confirm these presumptions in children we measured sHLA class I. serum levels in children after allogeneic bone marrow transplantation (BMT). We also investigated the levels of sHLA in the supernatants of mixed lymphocyte cultures (MLC) as possible predictors of GVHR prior to BMT. METHODS AND RESULTS: Group of 6 investigated children included 1 child with severe combined immunodeficiency, 3 children with acute lymphoblastic leukemia, 1 with severe combined immunodeficiency, 3 children with acute lymphoblastic leukemia, 1 with severe aplastic anemia and 1 with non Hodgkin lymphoma. The period of follow up varied from 15 days to 21 months according to the course of the disease. In the prediction of GVHR the levels of sHLA were measured in 5 children with acute leukemia in supernatants of MLC and the results were compared with the grade of GVHR classified according Seattle criteria. Soluble HLA class I. molecules were evaluated by ELISA. Rise of the levels of sHLA preceded 1-2 days the clinical signs of GVHR, however, it could not be distinguished from the occasional rise of a different cause. The levels of sHLA found in the supernatants of MLC showed individual results, which did not correspond to the level of cytokines in the same culture, or to the grade of GVHR observed. However, twice higher levels of sHLA in the culture of donor lymphocytes correlated with the lethal outcome of GVHR despite the fact that the donors were HLA identical siblings. CONCLUSIONS: The usefulness of sHLA levels as the predictors of GVHR has to be interpreted with great caution, but they can be used as a part of the mosaic composed of the clinical image and other laboratory results indicating GVHR. The predictive value of sHLA in supernatants of MLC is still to be evaluated.

Successful pregnancy in transfusion-dependent thalassaemia.

Successful pregnancy in a transfusion-dependent thalassaemic patient receiving subcutaneous desferrixaomine is reported. This is the first such case to be described.

Correlation between computed tomographic values and liver iron content in thalassaemia major with iron overload.

Computer tomographic (CT) scans of the liver were obtained in six thalassaemic patients with iron overload confirmed by liver biopsy. Mean CT values for the liver in individual patients were linearly related to the iron content estimated by liver biopsy (correlation coefficient = 0.995).