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+A 66-year-old man was admitted into our Hospital in November 2008 with splenomegaly, a high red cell count, and subnormal serum erythropoietin levels.
+Cytogenetic analysis showed a normal male karyotype.
+Molecular studies were negative for the BCR-ABL1 rearrangement, but revealed the JAK2V617F mutation with an allele burden of 44.9%.
+A diagnosis of PV was made on the basis of a morphological bone marrow analysis according to the WHO 2008 criteria.
+The patient was started on aspirin, hydroxyurea, and phlebotomies, all of which were well tolerated.
+Six years later, an abdominal ultrasound scan revealed the presence of dense bilateral perinephric infiltration, which was confirmed by a whole-body computed tomography (CT) scan, and a fluotine-18 fluordeoxyglucose positron emission tomography (F-FDG-PET) scan demonstrated increased glucose uptake in the medullary channels of both humeri and femurs.
+Morphological bone marrow analysis confirmed the previous diagnosis of PV and showed no evident change in the grading of bone marrow fibrosis (MF-0 according to the EUMNET consensus),13 whereas cytogenetic analysis revealed the following complex male karyotype: 47,XY,+9[2/20],47,XY,add(6)(p25),+9[2/20],46,XY[16/20].
+The histological nature of the perinephric infiltration was identified by means of a CT-guided core biopsy of the lesion.
+Morphological analysis demonstrated the accumulation of foamy histiocytes that were positive for CD68 and negative for the dendritic cell markers CD1a and S100, surrounded by fibrosis and a scanty lympho-monocytic infiltrate (Figure ​(Figure1).1).
+The detection of the BRAFV600E mutation led to a diagnosis of ECD.
+Cardiac magnetic resonance imaging (MRI) revealed pseudo-tumoral infiltration of the atrioventricular wall,14 and a bone scan showed symmetrically irregular radiotracer uptake in the long bones of the limbs consistent with ECD (Figure ​(Figure22)2; the findings of cerebral and pulmonary imaging were negative.
+In accordance with the most recent guidelines for the treatment of ECD,1 the patient started therapy with pegylated interferon (IFN)-alpha and, after 3 months of treatment, a new abdominal ultrasound scan revealed the complete resolution of the dense bilateral perinephric infiltration, and the normalization of the blood cell counts was achieved.