Long-term clinical outcome of spinal Langerhans cell histiocytosis in children

International Journal of Hematology
Seong Wook LeeJong Jin Seo

Abstract

Spinal involvement of Langerhans cell histiocytosis (LCH) affects morbidity, but outcomes are not well understood. We analyzed long-term outcomes following uniform treatment at a single institution. Clinical characteristics and outcomes of spinal LCH patients were retrospectively analyzed. Height ratios were calculated using the anterior height of the involved vertebral body on magnetic resonance imaging (MRI) and the expected normal vertebral height. Twenty-two (22.4%) of 98 patients diagnosed with LCH had spinal involvement. The median age at diagnosis was 4.1 (range 0.6-12.3) years. Thirty-one spinal lesions were identified in 22 patients; the thoracic spine (n = 17) was most commonly affected. Eight lesions with minimal collapse, which appeared normal on plain radiography, were detected with MRI. All patients received vinblastine-based chemotherapy. Fourteen (70%) of 20 evaluable vertebral body collapses, including eight severe lesions, showed improvement in vertebral body height at a median follow-up of 6.0 (range 2.8-12.0) years. All traceable patients were alive without disease. Long-term follow-up of vertebral body collapse revealed vertebral height improvement in approximately 70% of spinal LCH patients, even in severe...Continue Reading

References

Feb 1, 1976·Acta Orthopaedica Scandinavica·J O Poulsen, P Thommesen
Jul 1, 1991·Journal of Pediatric Orthopedics·G BolliniJ M Bouyala
Oct 1, 1969·The Journal of Bone and Joint Surgery. American Volume·M E NesbitG J D'Angio
Oct 31, 1997·Journal of Pediatric Orthopedics. Part B·Y FlomanN Ramu
Apr 30, 1998·Hematology/oncology Clinics of North America·J S Meyer, B De Camargo
May 9, 2001·The Journal of Pediatrics·H GadnerUNKNOWN Histiocyte Society
Aug 5, 2004·The Journal of Bone and Joint Surgery. American Volume·Sumeet GargJohn P Dormans
Apr 9, 2009·Journal of Pediatric Orthopedics·Alexandre ArkaderJohn P Dormans
May 11, 2012·The New England Journal of Medicine·Pei-Yu Tsai, Wen-Sheng Tzeng
Aug 22, 2012·Annual Review of Pathology·Gayane Badalian-VeryBarrett J Rollins
Mar 8, 2013·American Journal of Clinical Oncology·Rupesh KotechaKenneth Wong
Apr 17, 2013·Blood·Helmut GadnerUNKNOWN Histiocyte Society
Apr 23, 2013·The Spine Journal : Official Journal of the North American Spine Society·Wending D HuangNingyang Y Jia
Nov 28, 2013·Journal of Pediatric Hematology/oncology·Bo Eun KimUNKNOWN Korea Histiocytosis Working Party
Nov 29, 2014·British Journal of Haematology·Marie-Luise BerresCarl E Allen

❮ Previous
Next ❯

Citations


❮ 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.