MEDizzy - A 45-year-old man has had increasing abdominal discomfort

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Combined Hematology II

A 45-year-old man has had increasing abdominal discomfort with abdominal enlargement for the past two years. On physical examination, the spleen can be felt below the left costal margin. There is no fluid wave. An abdominal CT scan reveals massive (estimated 3000 gm size) splenomegaly. Laboratory data include Hgb 9 g/dL, WBC count 5,000/microliter, and platelet count 50,000/microliter. Which of the following underlying conditions is he most likely to have?

ExplanationA. Clinically this pt is having massive splenomegaly without ascites (absent fluid wave/thrill), so the most likely Answer would be myelofibrosis Splenomegaly is absolutely not seen in sickle cell disease and hemochromatosis. In infectious mononucleosis the splenomegaly is not massive. As he is not having ascites so portal hypertension is also unlikely The causes of massive splenomegaly are: • CML • HCL • Myelofibrosis • Portal hypertension • Chronic malaria • Storage disorders Idiopathic myelofibrosis (IMF) is a bone marrow disease characterized by excessive production of reticulin and collagen fibers. Although fibrosis of bone marrow can be the outcome of numerous hematologic and nonhematologic conditions, the term IMF is commonly used in primary MF without secondary cause. The clinical signs of IMF include splenomegaly due to extramedullary hematopoiesis; leukocytosis and thrombocytosis, with predisposition to thrombotic events, due to clonal cellular proliferation affecting mainly megakaryocytes and granulocytes; cytopenias, a later finding that worsens with the progression of fibrosis; and constitutional symptoms (e.g. fatigue, weight loss, low-grade fever, night sweats), most likely induced by abnormal levels of circulating cytokines