Clinical advances in hematology & oncology : H&O
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Clin Adv Hematol Oncol · May 2009
ReviewThe diagnosis and management of polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the JAK2 V617F era.
The chronic myeloproliferative disorders, polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are clonal stem cell disorders that occur at a low frequency and mimic not only each other clinically, but also many benign and malignant hematopoietic disorders as well. The discovery that many patients with polycythemia vera, essential thrombocythemia, and primary myelofibrosis express a mutation in the Janus Kinase 2 gene (JAK2 V617F), a kinase essential for the normal development of erythrocytes, granulocytes, and platelets, provided a molecular explanation for the unregulated hematopoiesis typical of these disorders, a diagnostic test that distinguishes them from other types of myeloproliferative disorders, and an opportunity to develop targeted therapy that could potentially avoid the toxicities associated with the conventional chemotherapeutic agents currently employed in their treatment. In this review, we discuss the molecular basis of polycythemia vera, essential thrombocythemia, and primary myelofibrosis, their diagnosis and their management in the context of the JAK2 V617F mutation.
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Clin Adv Hematol Oncol · Feb 2003
ReviewPotential role of recombinant factor VIIa as a hemostatic agent.
Recombinant factor VIIa (rFVIIa) has been shown to induce hemostasis in hemophilia patients with inhibitors against factor VIII or factor IX independent of factor VIII/factor IX. Factor VIIa binds to tissue factor (TF) exposed at the site of injury and generates, through factor X activation on the TF-bearing cells, enough thrombin to activate factors VIII, V, and XI, as well as platelets. The thrombin-activated platelets provided a perfect template for binding of activated factors VIII, IX, and V, further activation of factor X, and thrombin generation. ⋯ This also maintains hemostasis in the absence of factor VIII or factor IX. Pharmacologic doses of rFVIIa may accordingly be of benefit in producing hemostasis in situations other than hemophilia characterized by profuse bleeding and impaired thrombin generation. There is now clinical experience indicating a hemostatic effect in patients with thrombocytopenia and functional platelet defects. rFVIIa has also been successfully used in acute trauma patients with profuse bleedings and in other bleeding situations.
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Clin Adv Hematol Oncol · Jul 2006
ReviewThe role of hematopoietic stem cell transplantation in non-Hodgkin lymphoma.
Hematopoietic stem cell transplantation has become an increasingly important treatment modality for hematologic malignancies. Autologous stem cell transplantation has become the standard of care in relapsed high-risk diffuse large B-cell lymphoma (DLBCL) patients who respond to reinduction therapy. ⋯ The treatment of mantle cell lymphoma is challenging and stem cell transplantation may play an important role, as our understanding of the biology and natural history of this disease improves. This review focuses on studies evaluating the role of stem cell transplantation in non-Hodgkin lymphoma.
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Clin Adv Hematol Oncol · May 2004
ReviewPalonosetron: a unique 5-HT3 receptor antagonist indicated for the prevention of acute and delayed chemotherapy-induced nausea and vomiting.
Despite the advance in supportive care that occurred with the introduction of selective serotonin subtype 3 (5-HT3) receptor antagonists, control of chemotherapy-induced nausea and vomiting (CINV) with first-generation agents (ondansetron, dolasetron, and granisetron) is less than ideal. Palonosetron is a unique 5-HT3 receptor antagonist whose distinctive pharmacologic characteristics (ie, high 5-HT3 receptor binding affinity, prolonged half-life) result in superior clinical benefit. Superiority of palonosetron over ondansetron and dolasetron in the prevention of both acute and delayed CINV has been observed in each phase III trial conducted. ⋯ Unlike other 5-HT3 receptor antagonists, palonosetron is also indicated for prevention of delayed nausea and vomiting associated with initial and repeat courses of moderately emetogenic cancer chemotherapy. Palonosetron exhibits an excellent tolerability profile, with frequency, severity, and duration of adverse reactions similar to that of comparator agents. Unlike older agents that are considered therapeutically interchangeable at equipotent doses, palonosetron should be considered a clinically distinct and superior treatment for the prevention of CINV.