Zeb2 loss in adult mice results in a myeloproliferative disorder and is associated with altered JAK/STAT signaling pathway

J. Li, T. Riedt, S. Goossens, I. Guetgemann, D. Huylebroeck, J Haigh, P. Brossart, V. Janzen

Research output: Contribution to conferenceAbstract

Abstract

Introduction: Within the last 3 decades the knowledge of the history, demographics, immune pathology, clinical presentation and management dramatically have been expanded.

Methods: Prospecive registries, guidelines, the characteristics of the disturbed immune response by laboratory and clinical studies and new modalities of management are the main factors of improved knowledges.

Results: The prospective registries of immune thrombocytopenia led to new terminologies. Since only a part of patients with chronic ITP has symptoms of bleeding thrombocytopenic “Purpura” is no longer in use. The pathogenetic alterations of the innate and the adaptive immune system and the immune modulatory effects of new treatment modalities are the reasons to adapt the “i” from idiopathic to immune or autoimmune thrombocytopenia.In addition studies of the amount of platlet counts between 100-150 K showed that less than 7 percents of individuals developed ITP during further observation times. Follow up data of prospective registries with high numbers of patients revealed spontaneous recovery of up to 25% of patients between 6-12 months after diagnosis. Thus the terme “chronic ITP “ has been postponed to 12 months after diagnosis.

It was recognized that patients with no or mild bleeding management often do not need active treatment, but these patients have to be strictly followed for improvement of their quality of life.

Patients with acute and/or severe bleeding need intravenous immunoglobulins IVIG, high dose steroids and rarely platelet transfusions.

The guidelines of definitions and managements resulted in more individualized management including the quality of life of the patient.

Special considerations of management are needed in hyperactive children, in elderly persons with higher rates of comorbities, in pregnant womem, especially during labor an delivery, and in patients, who need preventive treatment (i.e befory surgery, acive sports etc.).

The management may be observation only in patient with no or mild bleeding. Active treatment modalities are now turning from the classic forms with high risk of adverse effects to acceptable side efffects (i.e. rituximab) and treatments with low adverse effects and higher rates of responses (i.e.in patients with bleeding. IVIG, in patients at risk of bleeding: thrombopoetin receptor agonists).

Conclusions: Registries may help to define special risk groups of patients with chronic ITP, which then should be evaluated by controlled studies.

Conference

ConferenceJahrestagung der Deutschen, Österreichischen und Schweizerischen Gesellschaften für Hämatologie und Medizinische Onkologie
CountrySwitzerland
CityBasel
Period9/10/1513/10/15
Internet address

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