Presentation
Sudden sharp left upper quadrant abdominal pain. Known history of myelofibrosis.
Patient Data
This spleen is markedly enlarged and has a craniocaudal dimension of 28 cm. The liver has a craniocaudal dimension of approximately 26 cm. There are several areas of low attenuation present within the spleen, the largest of which is inferolateral and anteriorly located. It is approximately 4 cm in size on axial imaging. The spleen otherwise shows no definite abnormality. The pancreas and kidneys have a normal appearance. There aorta is of normal caliber. Some pelvic fluid is shown.
Case Discussion
This case demonstrates hepatosplenomegaly with splenic infarction.
Myelofibrosis is a clonal stem cell disorder characterized by splenomegaly, chronic myeloproliferation, bone marrow infiltration, and extramedullary hematopoiesis (EMH) 1. It commonly presents with anemia, palpable splenomegaly, and constitutional symptoms. Hepatosplenomegaly is a complication of significant extramedullary hematopoiesis 2. Hepatomegaly and splenomegaly are seen in up to 40-70% and 90% of patients respectively 3.
Splenic infarction in myelofibrosis results from a combination of infiltration of the splenic parenchyma in EMH, anemia and increased oxygen requirements of an enlarged spleen.