Interest of interferon alpha in systemic mastocytosis. The French experience and review of the literature

Pathologie-biologie
J SimonGroup AFIRMM (Association française pour les initiatives de recherche sur le mastocyte et les mastocytoses)

Abstract

Systemic mastocytosis (SM) are defined by an abnormal growth and accumulation of mast cells in bone marrow and/or other extracutaneous organs. There is currently no cure for this disease. Because of similarities and/or association of mastocytosis with myeloproliferative disorders, interferon alpha has been tested but with contradictory reported results. A first prospective multicenter phase II trial was then started in France. From 1994 to 1997, 20 adult patients with confirmed bone marrow involvement received interferon alpha-2b for at least 6 months, (from 1 million U per day up to 5 million U/m(2)/day). Thirteen patients who presented systemic and/or specific cutaneous manifestations, demonstrated objective responses: seven (35%) were partial, six (30%) minor but no complete response could be observed at the time of analysis. The bone marrow remained unchanged in 12/13. Thus, interferon should be offered to patients with severe systemic manifestations, who have not responded to symptomatic therapies, even in case of non-aggressive mastocytosis, with or without corticosteroids the first weeks. Long-term therapy should be offered to patients with initial positive response. To control more aggressive SM or mastocytosis associat...Continue Reading

References

Feb 27, 1992·The New England Journal of Medicine·H C Kluin-NelemansR Willemze
Aug 1, 1996·Leukemia & Lymphoma·A S WorobecD D Metcalfe
May 15, 1998·The British Journal of Dermatology·J H Butterfield
Oct 31, 1998·Annals of Hematology·A Reinacher-SchickW Schmiegel
Feb 17, 1999·Proceedings of the National Academy of Sciences of the United States of America·B J LongleyY Ma
May 30, 2001·Leukemia Research·P ValentJ M Bennett
Mar 29, 2002·International Archives of Allergy and Immunology·Alexandra S Worobec, Dean D Metcalfe
Aug 22, 2003·British Journal of Haematology·Peter ValentDean D Metcalfe
Aug 23, 2003·Blood·Hanneke C Kluin-NelemansRob Fijnheer
Dec 5, 2003·Leukemia Research·A PardananiA Tefferi

❮ Previous
Next ❯

Citations

Mar 23, 2011·Journal of Hematology & Oncology·Gerhard J MolderingsLawrence B Afrin
Nov 27, 2008·International Journal of Hematology·Livio PaganoPellegrino Musto
Dec 3, 2014·Annales de dermatologie et de vénéréologie·S Barete
Sep 27, 2012·Hematology/oncology Clinics of North America·Animesh Pardanani
Aug 26, 2006·Immunology and Allergy Clinics of North America·Todd M WilsonJamie Robyn
May 31, 2013·American Journal of Hematology·Animesh Pardanani
Mar 29, 2011·American Journal of Hematology·Animesh Pardanani
Feb 18, 2015·American Journal of Hematology·Animesh Pardanani
Sep 21, 2013·Expert Opinion on Pharmacotherapy·Juan Carlos CardetMin Jung Lee
Sep 11, 2013·Orvosi hetilap·Noémi MihalikSarolta Kárpáti
May 2, 2016·Naunyn-Schmiedeberg's Archives of Pharmacology·Gerhard J MolderingsLawrence B Afrin
Oct 21, 2016·American Journal of Hematology·Animesh Pardanani
Jun 21, 2008·Acta Haematologica·Ken-Hong LimAyalew Tefferi
Jun 7, 2007·Journal of Pediatric Hematology/oncology·Jae Wook LeeDae Chul Jeong
Aug 12, 2004·Blood·Amie S CorbinMichael W N Deininger
Mar 14, 2012·American Journal of Hematology·Animesh Pardanani

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.