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From questions on page 71.
ANSWER TO QUESTION 1
The correct answer is C; langerhans cell histiocytosis (LCH). The second lateral cervical spine radiograph shows complete collapse of C3 vertebral body (figure 4, arrowed), also called a vertebra plana, with abnormal vertebral alignment indicating cervical spine instability.
LCH is a non-neoplastic proliferative disorder of histiocytes/dendritic cells (Langerhans cells being the histiocytes limited to skin). Clinical presentation can vary widely and LCH has historically been described as a number of different entities such as eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease often just representing the extent of organs/tissues involved. Treatment of LCH is based on risk stratification dependent on the extent of disease at presentation, namely; single bony-site, multiple bony-site or multisystem (multiorgan) involvement. Significant mortality is associated with multisystem disease in patients aged less than 2 years. Patients with only bone involvement have the best prognosis. LCH is seen from birth …
Competing interests None.
Provenance and peer review Commissioned; internally peer reviewed.