Biomaterial Database

A review of essential thrombocythemia and its complications. 2023

Rodrick Babakhanlou, and Lucia Masarova, and Srdan Verstovsek

Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.

Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm characterized by an increased platelet count in the peripheral blood and excessive megakaryopoiesis in the bone marrow. In one-third of cases, the disease remains benign and does not lead to complications. In the remaining cases, however, ET may present with thromboembolic and hemorrhagic complications and transform into more aggressive myeloid neoplasms, with a negative effect on morbidity and mortality. Despite extensive research and a better understanding of the pathogenesis and etiology of the complications associated with ET, limited data are available on the management of complications and emergencies. This article highlights the complications and emergencies associated with ET and discusses treatment options and the controversies associated with management.

UI	MeSH Term	Description	Entries
D009196	Myeloproliferative Disorders	Conditions which cause proliferation of hemopoietically active tissue or of tissue which has embryonic hemopoietic potential. They all involve dysregulation of multipotent MYELOID PROGENITOR CELLS, most often caused by a mutation in the JAK2 PROTEIN TYROSINE KINASE.	Disorder, Myeloproliferative,Disorders, Myeloproliferative,Myeloproliferative Disorder
D011087	Polycythemia Vera	A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs.	Erythremia,Osler-Vaquez Disease,Polycythemia Rubra Vera,Polycythemia Ruba Vera,Primary Polycythemia,Disease, Osler-Vaquez,Erythremias,Osler Vaquez Disease,Polycythemia Ruba Veras,Polycythemia Rubra Veras,Polycythemia, Primary,Polycythemias, Primary,Primary Polycythemias,Ruba Vera, Polycythemia,Ruba Veras, Polycythemia,Vera, Polycythemia Ruba,Vera, Polycythemia Rubra,Veras, Polycythemia Ruba,Veras, Polycythemia Rubra
D001853	Bone Marrow	The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells.	Marrow,Red Marrow,Yellow Marrow,Marrow, Bone,Marrow, Red,Marrow, Yellow
D004630	Emergencies	Situations or conditions requiring immediate intervention to avoid serious adverse results.	Emergency
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013920	Thrombocythemia, Essential	A clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.	Hemorrhagic Thrombocythemia,Thrombocythemia, Hemorrhagic,Thrombocythemia, Idiopathic,Thrombocythemia, Primary,Primary Thrombocythemia,Thrombocytosis, Autosomal Dominant,Thrombocytosis, Primary,Autosomal Dominant Thrombocytoses,Autosomal Dominant Thrombocytosis,Dominant Thrombocytoses, Autosomal,Dominant Thrombocytosis, Autosomal,Essential Thrombocythemia,Essential Thrombocythemias,Hemorrhagic Thrombocythemias,Idiopathic Thrombocythemia,Idiopathic Thrombocythemias,Primary Thrombocythemias,Primary Thrombocytoses,Primary Thrombocytosis,Thrombocythemias, Essential,Thrombocythemias, Hemorrhagic,Thrombocythemias, Idiopathic,Thrombocythemias, Primary,Thrombocytoses, Autosomal Dominant,Thrombocytoses, Primary