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Biomedical subjects

Massimo Franchini

Publications and source records attributed to Massimo Franchini.

At least 55 records · Page 3Linked to original sources
PubMed2006

The efficacy of rituximab in the treatment of inhibitor-associated hemostatic disorders.

Rituximab is a chimeric anti-CD20 monoclonal antibody active against normal and malignant B cells which has proven to be effective in the therapy of CD-20 positive lymphomas. Its B-cell cytotoxic action has also been exploited in many non-malignant autoimmune disorders in which it has been used with the aim of interfering with the production of pathologic antibodies. The present knowledge regarding the use of rituximab in antibody-associated disorders of hemostasis (i.e. idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, acquired hemophilia A, congenital hemophilia with inhibitors, acquired inhibitors against coagulation factors) is presented briefly in this review. The results suggest that rituximab can be useful in the treatment of disorders of hemostasis associated with inhibitor formation. Although collectively the number of patients treated is now quite substantial, most of the data are drawn from isolated case reports or descriptions of small, uncontrolled series. Large, prospective, randomized trials are, therefore, needed to confirm the positive, preliminary results.

Adolescent↗
PubMed2005

Heparin-induced thrombocytopenia: an update.

Heparin-induced thrombocytopenia (HIT) is the most important and most frequent drug-induced, immune-mediated type of thrombocytopenia. It is associated with significant morbidity and mortality if unrecognized. In this review, we briefly discuss the main features of heparin-induced thrombocytopenia, particularly analyzing the most recent advances in the pathophysiology, diagnosis and treatment of this syndrome.

Journal Article↗
PubMed2005

Advances in the pathogenesis, diagnosis and treatment of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.

The thrombotic microangiopathies are microvascular occlusive disorders characterized by hemolytic anemia caused by fragmentation of erythrocytes and thrombocytopenia due to increased platelet aggregation and thrombus formation, eventually leading to disturbed microcirculation with reduced organ perfusion. Depending on whether brain or renal lesions prevail, two different entities have been described: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). However, not rarely the clinical distinctions between these two conditions remain questionable. Recent studies have contributed greatly to our current understanding of the molecular mechanisms leading to TTP and HUS. In this review, we briefly focus on the most important advances in the pathophysiology, diagnosis and treatment of these two thrombotic microangiopathies.

Hemolytic-Uremic Syndrome↗
PubMed2005

Recent acquisitions in the management of iron overload.

Chronically transfused patients develop iron overload, which leads to organ damage and ultimately to death. The introduction of the iron-chelating agent desferrioxamine mesylate dramatically improved the life expectancy of these patients. However, the very demanding nature of this treatment (subcutaneous, continuous infusion via a battery-operated portable pump) has been the motivation for attempts to develop alternative forms of treatment that would facilitate the patients' compliance. In this review, we describe the most important advances in iron-chelation therapy.

Deferoxamine↗
PubMed2005

Recent advances in hereditary hemochromatosis.

Hereditary hemochromatosis, a very common genetic defect in the Caucasian population, is characterized by progressive tissue iron overload which leads to irreversible organ damage if it is not treated in a timely manner. Recent developments in the field of molecular medicine have radically improved the understanding of the physiopathology and diagnosis of this disease. However, transferrin saturation and serum ferritin are still the most reliable tests for identifying subjects with hereditary hemochromatosis. Therapeutic phlebotomy is the mainstay of the treatment of this disease and the life expectancy of these patients is similar to that of the normal population if phlebotomy is started before the onset of irreversible organ damage. In this review we discuss the genetics, pathophysiology, diagnosis, clinical features, and management of hereditary hemochromatosis.

Age of Onset↗
PubMed2005

Analysis of HFE and TFR2 gene mutations in patients with acute leukemia.

There are increasing evidences regarding the association between iron overload and extra-hepatic malignancies. We studied the prevalence of 12 hereditary hemochromatosis (HH) gene mutations (C282Y, V53M, V59M, H63D, H63H, S56C, Q127H, E168Q, E168X, W169X and Q283P in the HFE gene and Y250X in the TFR2 gene) and its correlation with the iron status in 82 adult patients with acute leukemia (AL); 48 patients (58.5%) were affected by acute myeloid leukemia (AML) and 34 patients (41.5%) by acute lymphoblastic leukemia (ALL); 27 patients (32.9%) had at least one HH gene mutation (6 heterozygous for C282Y, 6 homozygous for H63D, 13 heterozygous for H63D and 2 heterozygous for S56C). Mean serum ferritin levels at diagnosis were increased (822.5+/-811.4 microg/L). However, there was no difference between patients positive or negative for the HH gene mutations. Similarly, we did not observe any statistically significant difference as far as iron status between AML and ALL patients. Our study does not support the evidence of an association between hemochromatosis gene mutations and iron overload in AL patients.

Adolescent↗
PubMed2005

Acquired hemophilia A: a concise review.

Acquired hemophilia A is a rare but severe autoimmune bleeding disorder. It is more frequent in the elderly and results from the presence of autoantibodies directed against clotting factor VIII. In this review, we briefly report on the present state of knowledge regarding acquired hemophilia A, analyzing its epidemiology, pathogenesis, diagnostic, and clinical features. We also describe the main characteristics of this disorder according to its association with different conditions and the most important advances in the treatment of bleeding episodes and the eradication of the autoantibody.

Aged↗
PubMed2005

Stability of blood cell counts, hematologic parameters and reticulocytes indexes on the Advia A120 hematologic analyzer.

Delayed sample analysis is not a rare circumstance in clinical and laboratory practice, especially when blood samples are shipped to distant centralized laboratories, when the analysis can not be readily performed, or when retesting is appropriate. In this study we sought to evaluate the stability of conventional and new hematologic parameters in blood specimens stored for as long as 24 hours at 4 degrees C. Of the 21 hematologic parameters tested with the use of the Advia 120 hematologic analyzer (Bayer Diagnostics), means for paired samples of specimens differed significantly over the 24-hour storage period for hematocrit, main corpuscular volume, percentage of macrocytes, platelet count, main platelet volume, reticulocyte count and percentage, and reticulocyte hemoglobin content (all P < .01). We noted no significant changes in the other parameters tested or in the white blood cell differential. The overall distribution of the immature reticulocytes fractions remained substantially unchanged, though the high staining-intensity fraction showed a considerable shift from the baseline measure. Bland-Altman plots and limits-of-agreement analysis showed mean biases between -4.8% and 37.2% and relative coefficients of variations ranging from 0.4% to 32.7%. The 95% agreement interval in the set of differences was satisfactory and almost within the current analytic-quality specifications for desirable bias. The results of this investigation suggest that, within certain limitations for parameters derived or calculated from cellular volumes, blood specimens stored for as long as 24 hours at 4 degrees C may be suitable for hematologic testing.

Automation↗
PubMed2005

High prevalence of sustained remission of idiopathic thrombocytopenic purpura after Helicobacter pylori eradication: a long-term follow-up study.

We studied the effect of Helicobacter pylori (H. pylori) eradication in 43 consecutive H. pylori-infected patients with idiopathic thrombocytopenic purpura. H. pylori was eradicated with antibiotics in 41 of them (95.3%). The difference between the mean platelet count before and after therapy was statistically significant (54.3 x 10(9)/l vs. 119.1 x 10(9)/l; P < 0.001). A sustained remission was observed in 20 patients (48.8%), after a median follow-up of 31.2 months. None of the patients still infected by H. pylori after therapy reached normal platelet values. The long-term follow-up confirms the efficacy of H. pylori eradication in H. pylori-infected ITP patients.

Adult↗
PubMed2005

Analysis of B- and T-cell clonality and HLA class II alleles in patients with idiopathic thrombocytopenic purpura: correlation with Helicobacter pylori infection and response to eradication treatment.

Many authors have recently found a positive correlation between Helicobacter pylori infection and idiopathic thrombocytopenic purpura (ITP), the most common autoimmune hematological disorder. In order to clarify the pathogenic mechanism of H. pylori-associated ITP, we have investigated 52 consecutive ITP adult patients for Helicobacter pylori infection, B- and T-cell clonality and HLA class II alleles. Thirty-four ITP patients (65.4%) were infected by H. pylori and bacterium eradication was accompanied by a long-term platelet response in 17 (53.1%) of them. A B-cell clonality was found in three patients (5.8%, two patients H. pylori-negative and one patient H. pylori-positive). The ITP patients with H. pylori infection showed a HLA-DRB1*11, *14 and -DQB1*03 frequencies significantly higher and a -DRB1*03 frequency significantly lower than in H. pylori-negative patients. Moreover, an HLA-DQB1*03 pattern was associated with a higher probability of platelet response to eradication treatment. If our study documents the efficacy of eradication treatment in H. pylori-infected ITP patients, it may also help to identify different subgroups of ITP patients with probably different pathogeneses of thrombocytopenia and, finally, different responses to eradication treatment.

Adult↗
PubMed2005

The platelet-function analyzer (PFA-100) for evaluating primary hemostasis.

The platelet-function analyzer, PFA-100, is a relatively novel method for rapid in vitro global evaluation of primary hemostasis. The aim of this review is to summarize the published studies reporting on the utility of the PFA-100 device as a screening tool for primary hemostasis. Data were identified by searches of the published literature, including PubMed, references from reviews and abstracts from the most important meetings on this topic. The literature data support the use of the PFA-100 as a useful screening tool for the investigation of von Willebrand's disease and other acquired and congenital intrinsic platelet function disorders. Moreover, it is useful for evaluating primary hemostasis before surgical procedures and for monitoring desmopressin therapy in both von Willebrand's disease and platelet function disorders. Given its high sensitivity, speed and simplicity of use, we conclude that the PFA-100 could replace the in vivo bleeding time as a screening test for primary hemostasis in routine clinical practice.

Blood Platelet Disorders↗
PubMed2005

Hereditary hemochromatosis.

Hereditary hemochromatosis (HH) is a disorder of iron metabolism characterized by a progressive tissue iron overload which leads to an irreversible organ damage if it is not treated timely. The recent developments in the field of molecular medicine have radically changed the physiopathology and the diagnosis of this disease. However, transferrin saturation and serum ferritin are still the most reliable tests for the detection of subjects with HH. Therapeutic phlebotomy is the mainstay of the treatment of HH. If phlebotomy is started before the onset of irreversible organ damages, the life expectancy of these patients is similar to that of normal population.

Ferritins↗
PubMed2005

The antiphospholipid syndrome.

Antiphospholipid syndrome is an autoimmune disorder characterized by the association between antiphospholipid antibodies and venous or arterial thrombosis or obstetric complications. In spite of the recent progresses, many aspects of this disease remain unclear. In this review, we briefly focus on the most important advances in the pathophysiology, diagnosis and treatment of this condition.

Antiphospholipid Syndrome↗
PubMed2005

Acquired coagulation inhibitor-associated bleeding disorders: an update.

Acquired blood coagulation inhibitors are circulating immunoglobulins that neutralize the activity of a specific coagulation protein or accelerate its clearance from the plasma, thus causing a bleeding tendency. In this review, we focus on the nonhemophilic inhibitors of coagulation, i.e. the autoantibodies occurring in individuals without a pre-existent coagulation defect, reporting the most recent advances in the pathophysiology, diagnosis and treatment of these rare acquired bleeding disorders.

Autoantibodies↗
PubMed2005

Serum ferritin as a marker of potential biochemical iron overload in athletes.

OBJECTIVES: Beyond hematological manipulation, iron supplementation therapy is commonplace in athletes to counterbalance physiological or pathologic anemia and to prevent physiologic dysfunction. However, misuse of iron therapy, occasionally resulting in iron overload, is not free from metabolic risks. DESIGN: We planned to measure baseline serum ferritin concentration in sedentary individual and athletes. SETTING: The Institute of Clinical Biochemistry of the Verona University. PARTICIPANTS Serum ferritin was measured in 60 male healthy sedentary controls, 80 amateur road cyclists, 42 male professional cross-country skiers, and 88 professional male road cyclists. ASSESSMENT OF RISK FACTORS: The biochemical iron overload was ascertained by measuring baseline serum ferritin concentration as a reliable approach that mirrors the total body iron content. MAIN OUTCOME MEASUREMENTS: The concentration of serum ferritin in healthy controls was 112 +/- 78 ng/mL, whereas that of amateur cyclists, professional skiers, and professional cyclists was 127 +/- 76 ng/mL (P = 0.185), 183 +/- 130 ng/mL (P = 0.001), and 332 +/- 218 ng/mL (P < 0.001), respectively. RESULTS: Both categories of professional athletes showed significantly increased concentrations of serum ferritin, whereas the concentration of amateur cyclists was comparable to that of healthy sedentary controls. CONCLUSIONS: Professional endurance athletes have serum ferritin concentrations that are 2-fold to 3-fold higher than those of matched sedentary individuals and amateur athletes, exceeding the threshold for the diagnosis of biochemical iron overload and unveiling potential metabolic risks.

Bicycling↗