Interferon-α Therapy in Polycythemia Vera and Essential Thrombocythemia

Abstract

Essential thrombocythemia (ET) and polycythemia vera (PV) are chronic clonal myeloid disorders that originate from the multipotential hematopoietic stem cell. They are characterized, respectively, by excessive thrombocytosis and erythrocytosis, a high incidence of thrombohemorrhagic events, vasomotor symptoms, and an inherent tendency to undergo leukemic transformation. Current standard therapies to control the excess accumulation of myeloid cells and to provide symptomatic relief carry either a persistent risk of thrombosis, as in the case of phlebotomy, or, in the case of hydroxyurea, the potential for inducing leukemia. None alter the natural history of these diseases. Interferon-alpha has been shown to have potent antiproliferative effects on the hematopoietic stem cells and bone marrow fibroblasts and, as a result, has received much attention as a therapeutic agent for chronic myeloproliferative disorders. The ability of interferon-alpha to induce hematologic and cytogenetic remission in chronic phase chronic granulocytic leukemia has further increased interest in this agent. Interferon-alpha has shown therapeutic activity in PV and ET, as demonstrated in multiple small studies and single-arm trials reviewed in this article. Reported beneficial effects include the ability to control excessive erythrocytosis and thrombocytosis and such disease-related features as vasomotor symptoms, pruritus, and splenomegaly. Recent reports of cytogenetic remission and reversal of bone marrow fibrosis after interferon therapy are of interest. Advantages over current therapeutic standards include lack of known leukemogenic and teratogenic effects and the potential to alter the underlying course of disease. Nevertheless, none of the information available allows definite therapeutic recommendations for the use of interferon-alpha in PV or ET. The available data support the need for randomized controlled trials comparing interferon-alpha with standard therapy.

Aquagenic pruritus in polycythemia vera: Characteristics and influence on quality of life in 441 patients

“…The exact reason for this is unknown. However, it can be speculated that patients with persistent AP are switched to an Interferon-alpha treatment [11,12] to alleviate pruritus symptoms. Although we could not find a statistical difference between hydroxyurea, aspirin, anagrelide, phenprocoumon, or interferon-alpha for treatment of pruritus (Table IX), patients could potentially benefit from a switch of PV therapy.…”

Interferon-α for Treatment of Essential Thrombocythemia during Pregnancy: Case Report and Review of the Literature

“…Moreover, INF-␣ is nonleukemogenic, nonteratogenic [7] , not detected in the amniotic fluid or in fetal blood at the doses used [8] . These data suggest that INF-␣ is probably the optimal option in the management of pregnant women with ET who are candidates for platelet-lowering therapy (grade B), i.e.…”

Was ist bei einem Glottiskrampf zu tun?

“…Hier sind neben der rein HNO-ärztlichen Diagnostik (Panendoskopie) entsprechende gastroenterologische Untersuchungen (Refluxdiagnostik, 24hpH-Metrie des Ösophagus/Hypopharynx, Ösophagusmanometrie etc.) gefordert, die dann die adäquate Therapie nach sich ziehen (2,3,5). In den meisten Fällen lässt sich durch eine kausale Therapie der zugrundeliegenden Grunderkrankungen eine deutliche Verbesserung der Symptomatik erzielen.…”