C46 A RARE CASE OF LANGERHANS CELLS HISTIOCYTOSIS CARDIO–VASCULAR INVOLVMENT

A 54–year–old woman presented at our department for clinical follow–up. She was affected by hypertension, hypercholesterolemia, diabetes insipidus and autoimmune hypotiroidism in substitutive therapy. She had chronic coronary syndrome treated by medical therapy after unsuccessfull left anterior descending artery percutaneous revascularization and underwent to left common iliac artery stenting. The echo–doppler study showed a thickening of both common and internal carotid. Moreover, the patients underwent a skin biopsy of nodular, purple lesions which showed a xanthomatous infiltration suggestive of Langherans cell histiocytosis (CD1A+ e S100+). A subsequent PET–TC total body demonstrated a FDG uptake tissue around the great vessels and a focal increased uptake in the proximal thirds of the left tibia and in the distal thirds of fibula. The patient underwent to a new skin biopsy which confirmed histiocytosis (S100+, langherin+, CD1a+, BRAFV600E negative) and to a negative bone biopsy. Since the diagnosis of histiocytosis was confirmed the association of mercaptopurine and steroid therapy was started. In our department we performed a follow–up echocardiography which showed normal left and right ventricle dimensions and a good bi–ventricular function (LVEF according to Simpson 55% and FAC 50%) and surprisingly a focal right atrial wall thinckening of 3 mm. Therefore, the cardiac MRI showed no oedema in triple T2, no late gadolinium enhancement inIR–GRE T1 and at the posteriori wall of the right ventricle a focal thickening of 2.3 x 2.4 cm of increased intensity and a disomogenous enhancement after contrast agent administration (Figure 1); the same non stenotic tissue was showed along the aortic arch and the discending aorta (Figure 2). The MRI characteristic of these cardio–vascular lesions are consistent with the diagnosis of Langeran’s cells histiocytosis.