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Myeloproliferative Syndromes and Thrombocythemia

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Abstract

Several types of myeloproliferative syndrome (MPS) can involve the hepatobiliary tract, with a wide array of pathologic conditions. In MPSs with infiltration of liver parenchyma, hepatomegaly is a general feature and may be massive, often in conjunction with marked splenomegaly. Primary myelofibrosis (agnogenic myeloid metaplasia) is a clonal myeloproliferative neoplasm characterized by a growth of predominantly granulocyte precursors and megakaryocytes. Involvement of the liver shows accumulation of these cells mainly within sinusoids. This intrahepatic proliferation is frequently associated with liver fibrosis, mainly perisinusoidal fibrosis. The neoplastic cells can also form myeloid tumors or granulocytic sarcomas in the liver. Chronic myelogenous leukemia can also cause proliferation of immature myeloid cells in the liver, with massive hepatomegaly, together with signs of extramedullary hepatic hematopoiesis. In chronic neutrophilic leukemia, maturing and mature neutrophils can accumulate in the liver. In essential thrombocythemia, a myeloproliferative neoplasm involves primarily the megakaryocyte lineage; abnormal megakaryocytes accumulate in hepatic sinusoids. In this MPS, thrombotic complications of liver veins are a well-known phenomenon.

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    Arthur Zimmermann

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Zimmermann, A. (2017). Myeloproliferative Syndromes and Thrombocythemia. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_81

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