The Italian SIdEM registry for apheresis: an overview of the 2005 statistics.

Data collection on apheresis activities in Italy throughout 2005 including techniques, types of blood cell separators, clinical indications and adverse effects was performed by means of a standardized questionnaire. These data provided by 83 Apheresis Units from 16 Italian regions, albeit rough, are sufficiently informative, mainly in comparison with previous surveys on these statistics (1997 and 2000). In 2005 a total number of 204,746 apheresis procedures were carried out, with a clear-cut prevalence of apheresis production (87.7%), performed by 66 out of 83 Apheresis Units (79.5). Lombardy, Veneto and Tuscany were the most active regions for therapeutic apheresis (51.1% of the total national procedures). An increasing number in extracorporeal photochemotherapy as compared to the 2000 national survey (3,386 vs. 704 procedures) is the most striking observation to emerge from the 2005 data collection on therapeutic apheresis in Italy. Adverse effects, predominantly mild ones (i.e., paresthesia due to citrate-induced hypocalcemia), occurred in 0.12% of apheresis production and 6.04 of therapeutic sessions, particularly in the course of peripheral blood stem cell collection (20.79%), as already reported in the 2000 national survey.

[Malar rash in a painting by Jean-Baptiste Siméon Chardin (1699-1779)] / Rash malare in un dipinto di Jean-Baptiste Siméon Chardin (1699-1779).

In the present report we describe a typical malar rash, as painted by Jean-Baptiste Siméon Chardin (1699-1779), French painter, one of the greatest of the 18th century, on the face of a child in "Le Bénédicité" (Hermitage, St. Petersburg). Three versions of this theme were more completed by Chardin, in various times of his life, but the malar eruption can be seen solely on the painting at the Hermitage. In the attempt to clarify, on a diagnostic ground, such a cutaneous abnormality, the relationships between systemic lupus erythematosus and parvovirus B19 infection, i.e. the causative agent of fifth disease, are briefly discussed.

A preliminary survey of the apheresis activity in Italy: a perspective for the Italian Registry of apheresis.

Data collection on the apheresis activity in Italy throughout 1997 was performed by means of a standardized questionnaire. These data, provided by 96 Apheresis Units from 13 Italian regions, albeit rough, are sufficiently informative. In 1997, a total number of 170,373 apheresis sessions was carried out, with a clear-cut prevalence of productive apheresis (92%) that was performed by all Apheresis Units. Lombardy, Venetia and Latium were the most active regions for therapeutic apheresis (56% of total activity).

[Serotypes of hepatitis C virus in mixed cryoglobulinaemia]. / Caratterizzazione sierotipica del virus dell'epatite C nella crioglobulinemia mista.

BACKGROUND: Several authors have studied the genotypic distribution of hepatitis C virus (HCV) in mixed cryoglobulinaemia (MC), which represents the most typical extra-hepatic manifestation of the HCV infection. On the other hand, at present no data are available on the HCV serotypic characterization of MC patients. METHODS: Thus, 28 serum specimens from HCV positive patients affected by MC (7 males and 21 females; mean age of 64 yrs, range 35-80) have been evaluated by a serotyping EIA method (Murex HCV Serotyping 1-6 Assay, Murex Diagnostics, Pomezia, Italy). RESULTS: The HCV serotype 1 was found in 13 patients (46.4%), serotype 2 in 5 patients (17.9%) and mixed serotypes in 3 patients (10.7%), showing, respectively, serotypes 1+2, 1+4, and 1+4+5. Moreover, the HCV serotype was not detectable in 7 patients (25.0%). CONCLUSIONS: These results, obtained by using a simple, serologic technique, agree with some literature data concerning MC patients from the same geographic region as ours (Venetian area). In addition, our findings appears to reflect merely the genotypic distribution of the HCV infection in the North Italy.

Anti-hepatitis C virus serology in patients affected with congenital coagulation defects: a comparative study using three second generation ELISA tests.

We determined the prevalence of anti-hepatitis C virus (HCV) antibodies in 34 patients affected with congenital coagulation disorders attending the Haemophilia Centre of Padua, Italy. Serological tests were carried out by three second generation enzyme linked immunosorbent assays (ELISA), two based on recombinant proteins (Ortho and Abbott) and one based on synthetic peptides (Behring) as antigenic substrate. The repeatedly reactive specimens were further assayed by the supplemental 4-antigen recombinant immunoblot assay (RIBA) (Chiron and Ortho). Moreover, we performed the dot-blot Matrix test (Abbott) on the samples showing discrepant results by the three ELISA tests. Twenty-six patients (76.5%) were anti-HCV positive using all three ELISA tests; 25 were confirmed by the supplemental RIBA test, the other one was indeterminate. Two samples were in a gray-zone only using the anti-HCV ELISA Abbott. These were positive by the RIBA; in contrast, such samples showed no reactivity with the Matrix test. In accordance with the current literature, these data show an equivalence between the 2nd generation screening tests (ELISA), at least when applied to a high risk population as in the present study. Further, these screening tests demonstrated a reliable specificity, since most of the ELISA-reactive specimens were confirmed by the supplemental RIBA test. In contrast, combined use of the anti-HCV tests could be useful when high sensitivity is requested, as in the case of blood donor pretransfusion screening.

[Prevalence of antibodies against hepatitis C virus in rheumatoid arthritis. Study using second-generation tests]. / Prevalenza degli anticorpi anti-virus C dell'epatite nell'artrite reumatoide. Studio mediante i test di seconda generazione.

In order to evaluate the seroprevalence of anti-hepatitis C virus (HCV) antibody in rheumatoid arthritis (RA), where a high prevalence of false-positive anti-HCV reactions is reported, we studied 79 patients affected with RA. In these subjects we recorded some clinical and anamnestic data (history of blood transfusion, risk factors of liver disease, therapy) and determined, besides a few routine laboratory parameters including rheumatoid factor (RF), AST and ALT, the anti-HCV serology using the 1st (EIA, Ortho and Abbott; Neutralization test, Abbott; RIBA, Chiron-Ortho) and the 2nd generation tests (EIA, Ortho; RIBA, Chiron-Ortho). Four patients (of whom three were RF seronegative) were anti-HCV reactive by the 1st generation EIA tests (5.1%). According to the results of the confirmatory tests, and particularly of the 2nd generation, two patients resulted infected by HCV. These results do not confirm the previously reported high prevalence of false-positive anti-HCV reactions in RA, and demonstrated the usefulness of the 2nd generation tests in diagnosing the HCV infection.

First case in Italy of fatal AIDS in a hemophiliac.

The first fatal case of AIDS in an hemophiliac observed in Italy is reported. The propositus is a 53-year-old hemophilia A patient who died on the 8th December, 1984. AIDS was documented clinically and in the laboratory by serum antibodies to HTLV-III detected by ELISA and Western blot assays. A progressive intellectual worsening of the patient due to diffuse cerebral atrophy was followed by CT scan, EEG and by evaluation of proper neurological signs and symptoms.

Greater decrease of fibrinogen as compared to antithrombin III in L-asparaginase treated leukemia patients.

Fibrinogen and Antithrombin III (AT III) were studied sequentially during remission induction with L-asparaginase, prednisone and vincristine in 20 children with acute lymphoblastic leukemia (ALL). The first 2 weeks of therapy were characterized by significant decreases in plasma concentration of fibrinogen (p less than 0.05 or p less than 0.01). AT III (antigen and activity) decreased between the first and second week of therapy, but on an average, they remained a little above the lower limits of normal. However, at the second week, the difference was significant from basic values (p less than 0.05). All parameters gradually returned to normal after completion of L-asparaginase therapy.

Associated von Willebrand disease as a possible cause of lack of thrombosis in an AT III abnormality (AT III Trento).

In a family with a known antithrombin III abnormality (AT III Trento) an associated von Willebrand defect (Type I) was found. The two defects seem to segregate independently. In fact four types of individuals were present, namely: subjects with isolated AT III abnormality, subjects with isolated von Willebrand defect, patients with double defect and normal subjects. Only one of the two patients with isolated AT III abnormality showed a thrombotic tendency. None of the patients with double defect showed thrombotic disease, indicating a possible protective action of the von Willebrand defect against thrombotic manifestations. Patients with isolated von Willebrand defect showed neither thrombotic nor bleeding manifestations. The study emphasizes the need for a careful evaluation of the hemostatic balance of patients with AT III abnormalities before concluding that they are symptomatic or asymptomatic.

Behaviour of antithrombin III abnormalities in the crossed immunoelectrophoresis system.

Antithrombin III (AT III) abnormalities can be characterized by means of crossed immunoelectrophoresis. In the past, it was thought that the abnormalities could be demonstrated only if heparin is present in the system. Now some conditions (AT III Trento, for example) are known to show an abnormal pattern only in the absence of heparin. This indicates that some of the changes are heparin-independent. Furthermore, it could be demonstrated that in some cases the abnormality is present only in serum (AT III Vicenza, for example). Therefore, the test should be carried out as a screening procedure both in plasma and serum and in the presence or absence of heparin in every case of suspected AT III abnormality.

Antithrombin III deficiency: a report of 14 cases belonging to three different kindreds.

A hereditary deficiency of AT III is described in 14 subjects belonging to three different kindreds. There is no consanguineity in any of the families investigated. The pattern of inheritance of defect appears autosomal dominant. Seven of the affected subjects presented thrombotic episodes (deep vein thrombosis, splanchnic thrombosis, pulmonary embolization). The main laboratory features were: normal routine clotting tests, decreased AT III activity in all assay systems and concomitantly reduced AT III antigen levels. Crossed immunoelectrophoresis showed only reduced peaks with respect to normal in both plasma and serum. No correlation was found between age of patients and AT III levels.

Excess of factor X antigen versus clotting counterpart in coumarin treated patients as determined by an Elisa method.

Factor X antigen was assayed by means of an Elisa (sandwich) method in 36 patients on long-term anticoagulant therapy. The average value observed was 31.4% +/- 12.2. In almost every instance the antigen level was higher than the clotting counterpart (14.4% +/- 4.5). In a few instances no major difference was noted between factor X antigen and factor X activity. The method correlated fairly well with other immunological methods (electroimmunoassay and Laser Nephelometer). Therefore, factor X Elisa method appears to be a suitable method for factor X antigen evaluation.

Factor X Padua: a 'new' congenital factor X abnormality with a defect only in the extrinsic system.

A family with a new factor X defect is reported. The proposita is a 56-year-old female. She is asymptomatic and no consanguinity is present between the parents. The main features of the defect are: prolongation of prothrombin time and derivative tests but normal partial thromboplastin time. Factor X was found to be low (about 25-30% of normal) only if tissue thromboplastins were used in the assay system. Chromogenic substrate S-2222 also yielded decreased factor X levels. However, factor X activity was normal with cephalin and cephalin-RVV mixture. Factor X antigen was normal in three immunological systems (electroimmunoassay, an Elisa method and laser nephelometry). Crossed immunoelectrophoresis and antigen-antibody kinetics recorded in a laser nephelometer failed to show major differences from normal factor X. Both sons of the proposita, the father and other family members showed slightly decreased factor X levels and normal factor X antigen and were considered heterozygous for the abnormality. The toponym factor X Padua is proposed to indicate this peculiar abnormality.

Antithrombin III Trento. A 'new' congenital AT III abnormality with a peculiar crossed-immunoelectrophoretic pattern in the absence of heparin.

A family with a new congenital abnormality of antithrombin III (AT III) is presented. 5 members, all females, were affected. The proposita has had several thrombotic manifestations. The other patients, so far, are asymptomatic. Antithrombin activities were all decreased regardless of the method used (chromogenic or clotting) and regardless of the presence or absence of heparin in the assay system. AT III antigen, on the contrary, was normal in all patients regardless of the method used (electroimmunoassay, radial immunodiffusion or Laser nephelometer). The crossed immunoelectrophoresis without heparin showed in plasma the presence of an abnormal peak which was more anodal than the normal counterpart. The same pattern was seen in serum. In the heparin-modified cross-immunoelectrophoresis a normal pattern was seen in plasma and an abnormal one in serum. In the latter the anodal peak was in fact larger than the normal counterpart. Chromatographic studies using Heparin-Sepharose column failed to show changes in heparin affinity, and indicated that both the normal and the abnormal proposed to describe this abnormality. These studies further emphasize the great heterogeneity of AT III defects. This is the first AT III abnormality to show an abnormal crossed-immunoelectrophoresis in the absence of heparin.