High efficacy of Rituximab in indolent HCV-related lymphoproliferative disorders associated with systemic autoimmune diseases.

OBJECTIVE: The evidence of an increased frequency of B-non Hodgkin's lymphomas (NHL) in patients with HCV and systemic autoimmune diseases suggests a close relationship between infection, autoimmunity and cancer. Choosing the best therapy for patients affected either by HCV-related lymphoma or autoimmune disorders is not easy; in fact, some treatments may be accompanied by an excessive hepatic toxicity and may be followed by a reactivation of hepatitis. There is growing interest in the search for an ideal therapy for this kind of patient. Thanks to its mechanism of action and good toxicity profile, Rituximab could prove to be an attractive therapeutic option: it has been reported to be highly active in low-grade NHLs and has been proposed for the management of autoimmune diseases. RESULTS: In this paper we evaluate the role of anti-CD20 monoclonal antibody in mono-therapy in 10 patients with either indolent HCV-related lymphoma or autoimmune disease. A very high rate of response, of both NHL and of the associated autoimmune disease, was observed (100% of clinical response), with no significant hepatic and extra-hepatic toxicity. CONCLUSION: Thus, although the number of patients was small, our data strongly support the use of anti-CD20 in this patient setting.

[Methimazole-induced aplastic anemia]. / Anemia aplastica indotta da metimazolo.

The aplastic anemia methimazole induced is a rare event. We describe a case of a woman, affected by hyperthyroidism, who suffers from bone marrow aplasia during Tapazole treatment, and literature has been reviewed. Stopping methimazole and beginning a therapy with methylprednisolone in high dosage, intravenous IgG in high dosage, filgastrin and danatrol, medullary recover after 11 days of treatment is obtained. By analyzing our case and the 12 further cases described, we find that the prognosis of aplastic anemia during the treatment of antithyroid therapy, is rather good. Even if the pathogenetic mechanism of Tapazole induced bone marrow aplasia, it seems to be immuno-mediated in most cases, a direct toxicity in bone marrow cannot be excluded. In examined cases the antithyroid dosage was medium high (mean 40 mg/die). Therefore the use of Tapazole in low doses (15 mg/die) effective in almost all hyperthyroid patient must be considered, when possible, the best therapeutic choice, since it is safer in the incidence of important side effects such as bone marrow aplasia, especially in elderly patients or in patients with chronic renal failure.

[Chronic erythremic myelosis (or the chronic form of Di Guglielmo's disease). A case report]. / Mielosi eritremica cronica (o forma cronica della malattia di Di Guglielmo). Descrizione di un caso.

The chronic erythromyelosis (or chronic type of Di Guglielmo's disease) is a rare illness of uncertain classification. This illness shows characteristics that remember on the one hand the myeloproliferative and on the other hand the myelodysplastic diseases. The therapy is quite unsatisfactory. As a presence of erythrocytic antibodies has been detected, a therapy with steroids, danazol, splenectomy, immunosuppressive agents, plasmapheresis is advised. We have tried a cycle of therapy with high doses of intravenous gammaglobulin (0.4 g/kg/day for 4 days) also in the attempt of reduce, even if temporarily, the hemolytic component due to the reticuloendothelial system's hyperplasia. We conclude that this therapy, in our patient, has not yielded good outcomes immediately but carried out settled levels of haemoglobin as well as reduction of bilirubin and LDH after a month.

[Longevity in tetralogy of Fallot. The natural history of a 63-year-old man living without surgery]. / Longevità nella tetralogia di Fallot. Storia naturale di un 63enne vivente senza chirurgia.

We report the natural history, the clinical, radiological, echocardiographic and hemodynamic pattern of a living sixty-three year old man with tetralogy of Fallot and cyanosis since birth. We discuss the possible circulatory adaptations which allowed exceptional survival up to the seventh decade: it is the sixth case reported in the literature.