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    Fanin, R

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    Plasma cell leukemia occuring in a patient with thrombocythemia treated with hyroxyurea and busulphan

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    Plasma cell leukemia (PCL) is a rare aggressive lymphoproliferative disease with a short median survival and a very poor prognosis. We report the case of a 63-year-old man who developed a PCL after 5 years of chemotherapy with hydroxyurea and busulphan for Essential thrombocythemia (ET). The karyotype showed a deletion of chromosome 7 and the plasma cells cytofluorimetric examination revealed a high expression of Multidrug Resistance related P-glycoprotein (PGP). After the second cycle of VAD chemotherapy the patient had a severe pneumonia and died with refractory PCL. This is a rare example of the coexistence of a chronic myeloproliferative and lymphoproliferative diseases in the same patient, and to the best of our knowledge, the first reported in the literature involving PCL and ET. Moreover, this case shows the possibility of secondary malignancies developing in patients treated with busulphan and hydroxyurea for chronic myeloproliferative disorders. © 2004 Taylor & Francis Ltd

    GEMTUZUMAB OZOGAMICIN COMBINED WITH INDUCTION CHEMOTHERAPY IN YOUNG ADULTS WITH ACUTE MYELOID LEUKEMIA: REVIEW AND PERSPECTIVES.

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    Progress in treatment of acute myeloid leukemia (AML) is slow. Many new agents have been tested, but few were approved. Gemtuzumab Ozogamicin (GO) is a new AML-targeted drug that is composed by a monoclonal antibody targeting a surface antigen of myeloid leukemic cells (CD33) combined with a potent cytotoxic (calicheamicin). We review here the studies of GO in AML, including an update of the Italian studies, and we trace back the story of a drug that was developed 15 years ago and, regrettably, is no longer available for the treatment of AML, with the exception of Japan. GO was approved by the US FDA for the second-line treatment of AML in the elderly, and was shown by several European large prospective and randomized studies to be active also in first line, both alone, but particularly in combination with standard chemotherapy. Regrettably, a registration study that was performed in US could not confirm the superiority of GO and chemotherapy on chemotherapy alone, and the drug was withdrawn. The differences among the US and the European studies are discussed. The profile of the AML patients who are expected to benefit more by the reintroduction of GO is proposed: first-line, less than 60 years old, CD33 expressed in more than 20% leukemic cells, low/intermediate cytogenetic risk, and low expression of the PGP multidrug resistance protein

    Management of immune thrombocytopenia in elderly patients

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    Despite the improvement in understanding its pathogenesis and the introduction of novel treatment options, the management of primary immune thrombocytopenia (ITP) still remains challenging. Considering its increased incidence with aging and prolonged life-expectancy, ITP is often diagnosed in elderly patients, a subset that deserves some special precautions. Ensure the diagnosis is a crucial step, and carefully attention must be given in excluding other causes of thrombocytopenia, especially among older people that frequently suffer from many comorbidities. When it comes to treatment decision, it is worth keeping into account that the elderly have an increased risk of bleeding, thrombosis and infections, that they often require many concomitant therapies, including antiplatelet or anticoagulant agents, and that treatment-related toxicities are often increased and sometimes more dangerous that the disease itself. There are not dedicated guidelines, and only few specific studies. Steroids with or without IVIG remain the first-line treatment. Splenectomy is less effective than in youngers and burdened by an increased thrombotic and infectious risk. Rituximab is a good option in non-immunocompromised patients, but long-term remissions are few. Eltrombopag and romiplostim have a good safety and efficacy profile, and have become a prominent drug in this subset, even if they are associated with a possible increased risk of thrombosis, and long-term toxicity is unknown. Other drugs, such as dapsone and danazol, have a well-known efficacy and safety profile, and still represent a valid option among elderly patients
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