Clinicopathological and molecular biological studies in a patient with neurolymphomatosis

Détails

ID Serval
serval:BIB_D942DB7B5A0E
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Institution
Titre
Clinicopathological and molecular biological studies in a patient with neurolymphomatosis
Périodique
Muscle and Nerve
Auteur(s)
Kuntzer  T., Lobrinus  J. A., Janzer  R. C., Ghika  J., Bogousslavsky  J.
ISSN
0148-639X (Print)
Statut éditorial
Publié
Date de publication
10/2000
Peer-reviewed
Oui
Volume
23
Numéro
10
Pages
1604-9
Notes
Case Reports Journal Article --- Old month value: Oct
Résumé
We describe a patient with a clinical disorder that resembled vasculitic neuropathy in which peripheral nerves were successively affected over several months, but without systemic involvement. An initial muscle biopsy near the involved nerves showed signs of nonspecific inflammation around the muscle and nerve fibers. Immunosuppressive treatment resulted in a dramatic reduction in pain, but relapses of the disease eventually occurred, and the patient died 22 months after onset of the first symptoms. Pathologically, a malignant non-Hodgkin's B-cell lymphoma, restricted to the intra- and extradural peripheral nervous system, was found. The demonstration by Southern blotting of immunoglobulin heavy chain gene rearrangement confirmed the monoclonal nature of the lymphomatous cells. In situ hybridization tests for Epstein-Barr and herpes virus subtypes were negative. Our case underlines i) how difficult diagnosis can be despite extensive investigations, ii) the usefulness of immunosuppressive treatment in the early stage of the disease, iii) the importance of immunostaining and genome analysis for distinguishing between different types of human neurolymphomatosis, and iv) the fact that the initial inflammatory process in the muscle biopsy may be interpreted either as a paraneoplastic effect of the lymphoma or as a viral inflammatory neuromyopathy that triggers the development of the malignant lymphoma.
Mots-clé
Aged Female Humans Marek Disease/*pathology/*physiopathology Neural Conduction/physiology Spinal Cord/*pathology
Pubmed
Web of science
Création de la notice
25/01/2008 11:46
Dernière modification de la notice
20/08/2019 15:58
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