Haematologica
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Since optimal collection of peripheral blood progenitor cells (PBPC) remains crucial for high-dose therapy in patients with multiple myeloma (MM) in relapse phase or refractory to chemotherapy, we evaluated several variables that may influence mobilization. ⋯ In conclusion, patients who are potential candidates for high-dose therapy with PBPC support should not receive conventional alkylating therapy, even orally. Alternatively, progenitor cells should be collected early in the course of MM.
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Congenital afibrinogenemia is a rare coagulation disorder whose molecular basis is still poorly characterized. Most mutations have been identified in the fibrinogen Aalpha- and gamma-chain genes, whereas only two missense mutations have been reported in the Bbeta-chain gene. The aim of this work was to widen knowledge about the mutational spectrum of this disease by analyzing the molecular bases of congenital afibrinogenemia in three unrelated Iranian patients. ⋯ The identification of the first truncating mutation in the fibrinogen Bbeta-chain gene confirms the involvement of all three fibrinogen genes in the pathogenesis of congenital afibrinogenemia and widens the mutational spectrum of the disease. This knowledge is clinically essential in order to carry out prenatal diagnosis in families at risk.
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Therapy for relapsed/refractory lymphomas should be based only on drugs not included in the front-line chemotherapy regimens. We adopted the strategy of using salvage chemotherapy to debulk disease and simultaneously mobilize stem cells, using a regimen based on ifosfamide and etoposide, (drugs not usually used for front-line treatment). ⋯ Our results indicate the efficacy of the IEV regimen in inducing a good remission rate. IEV is a predictable and highly effective mobilization regimen in relapsed/refractory patients with aggressive NHL or HD.