PMID: 8469220Jan 1, 1993Paper

Langerhans cell histiocytosis in childhood: results from the Italian Cooperative AIEOP-CNR-H.X '83 study

Medical and Pediatric Oncology
A CeciP Indolfi

Abstract

Ninety patients with biopsy-proven Langerhans cell histiocytosis (LCH) were enrolled from June, 1983, to December, 1988, in the multicenter AIEOP-CNR-H.X. '83 study. They were divided into two groups: poor prognosis (PP), comprising 11 children with organ dysfunction (OD), and good prognosis (GP), made up of 79 patients without OD. Eighty-four patients were evaluable for treatment results. Among GP patients, 16 with a single lesion received only local treatment, while 59 entered a clinical trial of immunotherapy and/or monochemotherapy with vinblastine (VBL). Nonresponders, sequentially received doxorubicin (ADM) and then etoposide (VP16). PP patients were treated with 4 week cycles of vincristine, ADM, cyclophosphamide, and prednisone for nine courses. The overall survival was 92.8% (100% for GP patients and 45.5% for PP patients) at 48 months. The complete response (CR) rates for immunotherapy, VBL, ADM, and VP16 were 10%, 62.9%, 42.8%, and 88.2%, respectively. Two of the 11 PP patients had a CR (18.2%), while six died and three are still alive with recurrent disease. The overall incidence of disease-related disabilities was 47.7%, while that of diabetes insipidus was 20%. Monochemotherapy is probably adequate in GP patients,...Continue Reading

References

Jun 1, 1977·Archives of Disease in Childhood·D G Sims
Oct 1, 1979·International Journal of Radiation Oncology, Biology, Physics·J S GreenbergerA C Crocker
Aug 1, 1975·The Journal of Pediatrics·E Lahey
Aug 1, 1975·The Journal of Pediatrics·M E Lahey
Jan 1, 1991·Medical and Pediatric Oncology·M B VianaV H Rios Leite
Dec 12, 1991·The New England Journal of Medicine·C H PuiJ T Sandlund
Mar 1, 1990·Archives of Disease in Childhood·J McLellandJ Pritchard
Jan 1, 1989·Medical and Pediatric Oncology·V BroadbentE Yeomans
Jan 1, 1986·Medical and Pediatric Oncology·P Grundy, R Ellis
May 1, 1987·Klinische Pädiatrie·H GadnerH J Spaar
Oct 15, 1986·Cancer·E C Miller, J A Miller
Jan 1, 1985·Pediatric Pathology·C Nezelof, S Barbey
Jan 1, 1980·Medical and Pediatric Oncology·D M KompS L George
Sep 1, 1981·Medicine·J S GreenbergerJ R Cassady
Jan 1, 1981·The American Journal of Pediatric Hematology/oncology·M P Richter, G J D'Angio

❮ Previous
Next ❯

Citations

Dec 10, 1999·Head & Neck·G SuricoN Rigillo
Oct 3, 1999·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·R J Arceci
Nov 27, 2009·Annals of Oncology : Official Journal of the European Society for Medical Oncology·H Gadner
Dec 14, 2011·Current Opinion in Rheumatology·Marta Wilejto, Oussama Abla
Jun 26, 2008·Pediatric Dermatology·Elizabeth K Satter, Whitney A High
Mar 21, 2008·Journal of Neurosurgery. Pediatrics·Laurence DavidsonMark D Krieger
Apr 17, 2013·Blood·Helmut GadnerUNKNOWN Histiocyte Society
Aug 1, 1995·European Journal of Pediatrics·J L Stéphan
Jun 24, 2008·Survey of Ophthalmology·Curtis E Margo, Darin R Goldman
Oct 9, 2009·Pediatric Blood & Cancer·Vasanta NanduriTony Sirimanna
Apr 30, 1998·Hematology/oncology Clinics of North America·N G GroisD Prayer
Dec 12, 2001·Growth Hormone & IGF Research : Official Journal of the Growth Hormone Research Society and the International IGF Research Society·P C SizonenkoZ Laron
Dec 3, 2014·The Journal of Pediatrics·Stephen J SimkoCarl E Allen
Jun 19, 2002·Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery·Arnd BartnickRainer Schmelzle
May 1, 1997·Human Pathology·V Wright-BrowneZ Estrov
Dec 8, 2015·Hematology·Oussama Abla, Sheila Weitzman
Nov 19, 2010·QJM : Monthly Journal of the Association of Physicians·B Ng-Cheng-HinJ Waxman
Jul 9, 2005·Journal of Oral and Maxillofacial Surgery : Official Journal of the American Association of Oral and Maxillofacial Surgeons·Shinichiro NakamuraRichard F Scott
Jul 6, 2014·Scottish Medical Journal·Allison MartinRobert Carachi
Mar 14, 2002·American Journal of Hematology·Carlos Rodriguez-GalindoWinfred Wang
Apr 1, 1997·Medical and Pediatric Oncology·V Broadbent, J Pritchard
Jul 28, 2005·Pediatric Blood & Cancer·N GroisUNKNOWN DALHX- and LCH I and II Study Committee
Dec 21, 2019·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Jaskaran Singh GosalDeepak Kumar Jha
Apr 13, 1999·American Journal of Clinical Oncology·P KusumakumaryM K Nair
Jul 29, 2000·Pediatric Hematology and Oncology·P Kusumakumary, F V James
Jul 1, 1995·Medical and Pediatric Oncology·M AricòG R Burgio
Nov 26, 2002·Hematology·Robert J ArceciPeter D Emanuel
Jun 24, 2008·Pediatric Hematology and Oncology·Begül YağciMünevver Büyükpamukçu
Nov 1, 2007·Expert Review of Endocrinology & Metabolism·Polyzois MakrasGregory A Kaltsas
Oct 3, 1999·Pediatric Hematology and Oncology·J BraierJ Goldberg

❮ 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.