Unusual Presentation of Primary Myelofibrosis with Spontaneous Bleeding after Laparoscopic Adrenalectomy: A Case Report

INTRODUCTION:We described unusual presentation of primary myelofibrosis with spontaneous bleeding after laparoscopic adrenalectomy. This case is written following the SCARE criteria.PRESENTATION OF CASE:A 73 years old Caucasian man underwent laparoscopic right adrenalectomy for a rapidly increasing expansive mass (3.5 × 2.5 cm) of the right adrenal gland. The night of intervention, patient underwent urgent explorative laparotomy, that highlighted massive haemoperitoneum, clots in the abdomen, on the splenic side, on Morrison's space, and at the confluence between right renal vein and inferior vena cava. Surgical specimen examination showed extra-medullary myeloid proliferation of the right periadrenal tissue.DISCUSSION:Bone marrow biopsy was performed. The list of differential diagnoses included: chronic myelomonocitic leukaemia, atypical CML and primary myelofibrosis (PMF). After discharge, several results became available: conventional cytogenetics was normal, PDGFR-α, PDGFR-β and FGFR1 mutations were negative but V617 F mutation of the JAK2 gene was positive. Therefore, the final diagnosis was pre-fibrotic primary myelofibrosis according to the 2016 WHO classification, Dynamic International Prognostic Scoring System (DIPSS) plus 2 (intermediate-2).CONCLUSION:To our knowledge, we report uncommon case of primary myelofibrosis associated with extra-medullary myeloid proliferation of the right periadrenal tissue.