Treatment Of Essential Thrombocythemia Research Articles

Successful treatment of essential thrombocythaemia and recurrent abortion with alpha interferon

A 27-year-old woman with essential thrombocythaemia (ET) had three miscarriages. She was treated with alpha interferon which was continued after she became pregnant again. The platelet count remained well controlled and at term she delivered a healthy baby with normal blood counts. The case demonstrates the effectiveness of IFN in preventing recurrent miscarriage associated with ET and the safety of IFN with respect to fertility and fetal development.

Recombinant Interferon Alpha-2b as Treatment of Essential Thrombocythaemia

We report the disease characteristics and therapeutic results for 25 patients suffering from essential thrombocythaemia (ET), treated with recombinant interferon-alpha-2b (IFN-alpha 2b). ET was diagnosed according to the criteria of the Polycythaemia Vera Study Group. All patients were programmed to receive a subcutaneous induction treatment consisting of 3 MU of IFN-alpha 2b daily for 6 months. In responding patients, treatment was continued for a further 6 months with 3 MU of IFN-alpha 2b three times a week. Complete response was achieved in 13 of 25 patients, partial response in 10 of 25. In 2 cases, therapy was unsuccessful. Side effects were usually mild, consisting of flu-like symptoms in most cases, and were easily controlled by paracetamol. After a median follow-up of 14 months after discontinuation of the treatment, most patients retained the therapeutic response in the absence of toxicity.

MCNU in the treatment of essential thrombocythemia--a pilot study.

The efficacy of a single dose of MCNU 150 mg was evaluated in nine symptomatic patients with essential thrombocythemia (ET). As the platelet counts increased during the following 4 weeks, an extra dose of 100 mg of MCNU was administered to three patients. All patients had thrombotic or hemorrhagic complications. Seven patients responded. The mean platelet count decreased to below 1.0 x 10(12)/l within 5 weeks; it reached its lowest level (0.35 x 10(12)/l) at 6 weeks and became stable at less than 1.0 x 10(12)/l without further therapy for 12 weeks in all patients. Thrombotic and hemorrhagic complications ameliorated within 4 weeks in all patients. Our study indicates that MCNU acts as a platelet-reducing agent in patients with ET.

α-Interferon in the Treatment of Essential Thrombocythemia

α-Interferon in the Treatment of Essential Thrombocythemia

Interferon alpha-2b in the long-term treatment of essential thrombocythemia

We treated 35 patients affected with essential thrombocythemia (ET) with interferon (IFN) alpha-2b. Our treatment scheme consisted of (a) a 6-month induction phase and (b) a continuous maintenance phase. During the induction phase, our results showed that using 21 million units (mu) of IFN weekly platelet counts fell below 600 x 10(9)/1 in about 90% of patients. These data demonstrate that well-tolerated doses of IFN can rapidly correct excessive thrombocytosis. During the continuous maintenance phase, 61% of patients required 3 mu three times a week, 15% once a week, and 24% daily. Thus the minimal IFN doses able to maintain platelet count below 600 x 10(9)/l varied between 3 and 21 mu per week. During long-term treatment, subjective side effects were tolerable, especially using 3 mu three times a week. We conclude that IFN alpha-2b is an effective drug in the long-term treatment of ET.

Myelodysplastic syndrome and secondary acute leukemia after treatment of essential thrombocythemia with hydroxyurea

American Journal of HematologyVolume 33, Issue 2 p. 152-152 Letter to the EditorFree Access Myelodysplastic syndrome and secondary acute leukemia after treatment of essential thrombocythemia with hydroxyurea P. J. Van den Anker-Lugtenburg, P. J. Van den Anker-Lugtenburg Department of Hematology Dr. Daniel den Hoed Cancer Center Groene Hilledgk 301 3075 EA Rotterdam The NetherlandsSearch for more papers by this authorW. Sizoo, W. Sizoo Department of Hematology Dr. Daniel den Hoed Cancer Center Groene Hilledgk 301 3075 EA Rotterdam The NetherlandsSearch for more papers by this author P. J. Van den Anker-Lugtenburg, P. J. Van den Anker-Lugtenburg Department of Hematology Dr. Daniel den Hoed Cancer Center Groene Hilledgk 301 3075 EA Rotterdam The NetherlandsSearch for more papers by this authorW. Sizoo, W. Sizoo Department of Hematology Dr. Daniel den Hoed Cancer Center Groene Hilledgk 301 3075 EA Rotterdam The NetherlandsSearch for more papers by this author First published: February 1990 https://doi.org/10.1002/ajh.2830330217Citations: 20AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume33, Issue2February 1990Pages 152-152 ReferencesRelatedInformation

9 Essential thrombocythaemia

Essential thrombocythaemia (ET) is a rare clonal myeloproliferative disorder characterized by a persistent increase in platelet count. The commonly used criteria for the diagnosis, except for the level of the increase in platelet count, are usually those fixed by the Polycythemia Vera Study Group. The average age of onset is around 60 years, both sexes being affected. The symptoms frequently present at diagnosis are related to microcirculatory disturbances (palms, soles, fingers). Neurological symptoms are often observed. Thrombotic complications of the large vessels are less frequent. Haemorrhagic problems are present in about 30% of patients. Bleeding time is normal in most cases, whereas platelet aggregation abnormalities are frequently found. Nil adrenaline aggregation is the most discriminative test. The clinical course is characterized by long intervals without any symptoms; thromboembolic or haemorrhagic episodes can, however, occur, mainly in uncontrolled ET. Development of terminal acute leukaemia has been reported in 34 cases. The expression of the influence of the treatments, 32P or alkylating agents, is very strong. The treatment of ET has to take in consideration the difficult compromise between balancing the necessity of preventing complications and the effects of drug toxicity. The use of recombinant α-interferon has recently been proposed and is under investigation. The pathogenesis of thrombocytosis in ET seems to involve an expansion in the megakaryocyte progenitor cell pool. Platelet membrane glycoprotein abnormalities and defective glycosylation of thrombospondin have been shown. Numerous other platelet abnormalities, including decreased α-adrenergic receptors, loss of PGD2 receptors and increased Fc receptors, have been reported. Arachidonic metabolism seems to be abnormal and lipoxygenase is defective. Most of the platelet abnormalities seem to be the result of intrinsic defects at the level of an abnormal clone of megakaryocytes. However, causal relationships between the platelet abnormalities and bleeding or thrombosis are not yet clearly demonstrated.

The treatment of essential thrombocythaemia with radioactive phosphorus

The major complications of essential thrombocythaemia include haemorrhage and thrombosis, events which are related to the level of the abnormal platelet count. We report here on the use of radioactive phosphorus (32P) in eight patients with essential thrombocythaemia. Two patients required multiple injections of 32P to control platelet count, and there was no reduction in the rate of fall of platelets with each injection. Three patients required chemotherapy for persistent thrombocythaemia despite multiple doses of 32P. No clinical or haematological characteristics could be identified for those patients whose disease was difficult to control. Radioactive phosphorus is a safe and effective method to control platelet counts in patients with essential thrombocythaemia.