Adult Langerhans Cell Histiocytosis: A Rare Etiology of Spinal Cord Compression

Fadila Kouhen,Naoual Benhmiddou,Hanan Elkacemi,Mohammed Afif,Ibrahim Ghissassi,Tayeb Kebdani,Fadoua Rais,Youssef Mahdi,Basma Khannoussi,Noureddine Benjaafar,Khouloud Boussouni,Khadija Bellahamou,Sanaa Elmajjaoui

doi:10.4236/ojim.2015.53005

Fadila Kouhen, Naoual Benhmiddou + Show 11 more

Open Access

https://doi.org/10.4236/ojim.2015.53005

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Abstract

Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. Clinical presentation is variable, ranging from a single location in the bone to severe multivisceral involvement. Moreover, spinal involvement causing myelopathy is even rare and unusual. We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution.

Highlights

Langerhans cell histiocytosis (LCH) is a rare group of idiopathic disorders encompassing large variants of pathologic entities due to diversity of clinical course, evolutionary aspects and prognosis [1]
We report a rare case of adult LCH in the dorsal spine causing a spinal cord compression treated by surgery, radiotherapy and systemic therapy with good evolution
It’s may be induced by a viral infection (human herpesvirus 6 (HHV-6), a defect in intercellular communication (T cell-macrophage interaction), and/or a cytokine-driven process mediated by tumor necrosis factor, IL-11, and leukemia inhibitory factor [7]-[9]

Summary

Introduction

Langerhans cell histiocytosis (LCH) is a rare group of idiopathic disorders encompassing large variants of pathologic entities due to diversity of clinical course, evolutionary aspects and prognosis [1]. They all share histologically a significant infiltration of affected tissues by langerhans cells. How to cite this paper: Kouhen, F., et al (2015) Adult Langerhans Cell Histiocytosis: A Rare Etiology of Spinal Cord Compression. Different therapeutic approaches can be considered depending on the affected organ, including surgery, radiotherapy and chemotherapy. We report a rare case of adult LCH in the dorsal spine causing a spinal cord compression treated by surgery, radiotherapy and systemic therapy with good evolution

Case Report

Discussion

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