Langerhans cell histiocytosis: central nervous system involvement treated successfully with 2-chlorodeoxyadenosine.

Abstract

A case of disseminated Langerhans cell histiocytosis with resistant central nervous system (CNS) disease in an adolescent is described. The child presented with visible cranial lesions, emesis, headaches, and short-term memory loss. Diagnostic evaluation revealed multiple osseous lesions in the cranium, ribs, vertebral bodies, and pelvis. The clinical course with complications and response to each therapy are sequentially reviewed. Remission, as evidenced clinically and by magnetic resonance imaging, was ultimately accomplished with 2-chlorodeoxyadenosine (2-CDA). The full course of 2-CDA was not tolerated due to bone marrow suppression. CNS histiocytosis is known to be resistant to therapy. Earlier introduction of 2-CDA for CNS disease might offer more successful treatment with less toxicity than seen in patient.

• Authors: J Watts, B Files
• Journal: Pediatric hematology and oncology (Pediatr Hematol Oncol) 2001 Apr-May Vol. 18 Issue 3 Pg. 199-204 ISSN: 0888-0018 [Print] England
• PMID: 11293288 (Publication Type: Case Reports, Journal Article)

Chemical References

• 2-Chloroadenosine

Topics

• 2-Chloroadenosine (therapeutic use)
• Adolescent
• Brain (pathology)
• Central Nervous System Diseases (diagnosis, drug therapy, etiology)
• Histiocytosis, Langerhans-Cell (diagnosis, drug therapy, physiopathology)
• Humans
• Magnetic Resonance Imaging
• Male
• Tomography, X-Ray Computed