Patient with a known history of myelofibrosis presents with sudden sharp left upper quadrant abdominal pain.
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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 calibre. Some pelvic fluid is shown.
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The above case demonstrates hepatosplenomegaly and splenic infarction.
Myelofibrosis is a clonal stem cell disorder characterised by splenomegaly, chronic myeloproliferation, bone marrow infiltration, and extramedullary haematopoiesis (EMH) 1. It commonly presents with anaemia, palpable splenomegaly, and constitutional symptoms. Hepatosplenomegaly is a complication of significant EMH 2. Hepatomegaly and splenomegaly is 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, anaemia and increased oxygen requirements of an enlarged spleen.
- 1. Randhawa J, Ostojic A, Vrhovac R, Atallah E, Verstovsek S. Splenomegaly in myelofibrosis--new options for therapy and the therapeutic potential of Janus kinase 2 inhibitors. J Hematol Oncol. 2012 Aug 1;5:43. doi: 10.1186/1756-8722-5-43.
- 2. Abdel-Wahab OI, Levine RL. Primary myelofibrosis: update on definition, pathogenesis, and treatment. Annu Rev Med. 2009;60:233-45. doi: 10.1146/annurev.med.60.041707.160528
- 3. Murakami J, Shimizu Y. Hepatic Manifestations in Hematological Disorders. Int J Hepatol. 2013;2013:484903. doi: 10.1155/2013/484903. Epub 2013 Mar 31