A Case of Nodular Extramedullary Hematopoiesis of the Spleen with Latent Essential Thrombocythemia That Was Difficult to Diagnose

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  • 本態性血小板血症が潜在し診断に難渋した脾臓の結節状髄外造血の1例

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Abstract

<p>A 72-year-old man was found to have splenomegaly and an intrasplenic mass in a medical examination. Hamartoma, inflammatory pseudotumor, and sclerosing angiomatoid nodular transformation (SANT) were included as differential diagnoses, but malignant disease could not be ruled out; therefore, laparoscopic splenectomy was performed. A postoperative peripheral blood test showed extreme thrombocytosis and pseudo-hyperkalemia. Thereafter, the patient was diagnosed with essential thrombocythemia and myelofibrosis, with a mutant JAK2-V617F gene. The pathological diagnosis was focal nodular extramedullary hematopoiesis (EMH). EMH sequentially occurs in many hematological disorders, and sometimes presents as hematopoietic masses at several sites; however, presentation as a nodular mass localized in the spleen is rare. It is extremely difficult to diagnose EMH in patients without a past medical history of hematological disorders. However, for a focal nodular mass of the spleen, it is important to determine the treatment strategy with consideration of potential hematological diseases and extramedullary hematopoiesis.</p>

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