Primary Myelofibrosis with Mesenteric Extramedullary Haematopoiesis

Abstract

Primary myelofibrosis or chronic idiopathic myelofibrosis is a myeloproliferative neoplasm characterized by proliferation of abnormal haematopoietic stem cells in bone marrow resulting in replacement of marrow with fibrotic scar tissue. Extramedullary Haematopoiesis (EMH) as a compensatory phenomenon in myelofibrosis can lead to hepatosplenomegaly with diffuse organ infiltration or focal deposits. Peritoneal involvement of EMH is rare and can present with peritoneal or serosal implants which can mimic malignancy. We present a case of diffuse peritoneal /mesenteric extramedullary haematopoiesis with hepatosplenomegaly as an initial presentation in patient of idiopathic myelofibrosis.

Keywords

• megakaryocytes
• myeloproliferative neoplasm
• osteosclerosis