Effectiveness of bortezomib in cardiac Al amyloidosis: a report of two cases.

Cardiac involvement is a major prognostic determinant in patients with primary AL amyloidosis. The clinical results of standard therapeutic approaches are suboptimal. It has been recently shown that bortezomib, an inhibitor of the proteasome, can induce rapid favourable responses in AL amyloidosis improving cardiac function and survival. Herein we report on two patients with cardiac amyloidosis treated by bortezomib who experienced partial or total remission of hematologic disease and of cardiac involvement. However, death of one patient, suffering from chronic kidney disease stage 5, due to fulminant respiratory syndrome suggests the need for caution in bortezomib use if patients have this comorbid condition.

Survey on the prevention and incidence of haemolytic disease of the newborn in Italy.

BACKGROUND: In 2010, the Italian Society of Immunohaematology and Transfusion Medicine (SIMTI) carried out a survey of the incidence of haemolytic disease of the newborn (HDN) and the prevention of HDN caused by anti-Rh(D) in Italian Transfusion Structures (TS). MATERIALS AND METHODS: A questionnaire divided into the following five sections was administered: (i) types of services provided and maintenance of legally required registers, (ii) immunoprophylaxis (IP), (iii) red cell typing and searches for irregular antibodies, (iv) evaluation of foetal-maternal haemorrhage (FMH), and (v) incidence of HDN in 2010. Of the 280 TS sent the questionnaire, 176 (63%) replied. RESULTS: A HDN register was available in 55.5% of the TS (n =91). Immunoprophylaxis with a dose of anti-D IgG was given to all Rh(D) negative and Rh(D) variant puerpera with Rh(D) positive newborns: in more than 93% of cases the dose was between 1,500 IU (300 µg) and 1,250 IU (250 µg). Antenatal IP between the 25(th) and 28(th) week was proposed by 42 TS (26%). Seventy percent of the TS (n =115) did not make any evaluation of FMH. The number of births surveyed in 2010 was 203,384, the number of Rh(D) negative pregnancies was 13,569, while anti-D antibodies were present in 245 pregnancies. There were 111 cases of HDN due to anti Rh(D) incompatibility and in 40 of these, intrauterine transfusion (n =8) or exchange transfusion (n =32) was necessary. In 94 cases HDN was due to other irregular antibodies: in 4 of these cases intrauterine transfusion was needed and in 11 other recourse was made of exchange transfusion. Finally, there were 1,456 newborns with ABO HDN of whom 13 underwent exchange transfusion. DISCUSSION: The data collected give a picture of the incidence of HDN in Italy and of the methods of managing IP and could form the basis for an update of the SIMTI recommendations on the management and prevention of this disease.

The role of the direct antiglobulin test in pre-transfusion investigations and the approach to selecting blood for transfusion in autoimmune haemolytic anaemia: results of a regional survey.

INTRODUCTION: The aim of this study was to conduct a Regional survey to determine the policies and ways of performing the direct antiglobulin test in pre-transfusion screening, the approach used in cases giving positive results with this test and the technical and operative modalities for choosing blood for transfusion in cases of autoimmune haemolytic anaemia. MATERIALS AND METHODS: A questionnaire, containing ten multiple-choice questions, was sent to all the transfusion centres in the Region of Tuscany. RESULTS: The data from all 40 regional centres were analysed. Direct antiglobulin tests and autocontrols were not regularly used in pre-transfusion screening. The direct antiglobulin test was predominantly reserved for suspected cases of autoimmune haemolytic anaemia. Sixty percent of the laboratories characterised the specificity of samples that were positive for IgG and complement by the direct antiglobulin test, 45% that were positive for IgM, 35% also for IgA, and 13% also for subclasses of IgG. Elution studies were reserved (in 18% of laboratories) for those cases in which it was expected that transfusion therapy would be used. In cases of autoimmune haemolytic anaemia, autologous/allogeneic adsorption was carried out in 27% of the structures (the use of proteolytic enzymes is predominant, followed by the "ZZAP" reagent--a mixture of dithiothreitol and an enzyme) and the dilution method in 20%; transfusion of red blood cells with a phenotype extensively compatible (c, C, D, E, e, K, Jka, Jkb, Fya, Fyb, S, s) with that of the recipient is practised in 17% of the centres, while transfusion of units of "least incompatible" red blood cells was reported by 95% of the centres, but in 88% this is preceded by at least one of the above mentioned immunohaematological investigations. CONCLUSIONS: The organisation of a network of Services of Immunohaematology and Transfusion Medicine can be exploited to overcome some technical and operative limitations of peripheral, dependent Transfusion Sections. The results of this study reveal which immunohaematology laboratory is endowed with the greatest potential and which could, therefore, become the regional reference centre. This investigation could lay the basis for defining behavioural algorithms and recommendations on the issues considered.