Annals of hematology
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Mastocytosis consists of a group of disorders characterized by a pathologic increase in mast cells in tissues including skin, bone marrow, liver, spleen, and lymph nodes. Mastocytosis is a rare disease. Because of this, general practitioners have limited exposure to its clinical manifestations, diagnosis, classification, and management. ⋯ Despite recent advances in knowledge about the pathophysiology, diagnosis, and classification of mastocytosis, a curative treatment for mastocytosis does not now exist. Management of patients within all categories of mastocytosis includes: (1) a careful counseling of patients (parents in pediatric cases) and care providers, (2) avoidance of factors triggering acute mediator release, (3) treatment of acute mast cell mediator release, (4) treatment of chronic mast cell mediator release, and if indicated (5) an attempt to treat organ infiltration by mast cells. The goal of this manuscript is to provide an overview of the mediators produced and released by mast cells, the diagnostic criteria for the different variants of mastocytosis, and the treatment options currently available.
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Annals of hematology · Dec 2002
Bilateral adrenal swelling as a cause of chest, back, and upper abdominal pain in essential thrombocythemia and polycythemia vera is due to microvascular ischemic thrombosis rather than to hemorrhage.
Bilateral massive adrenal swelling (BAS) on computed tomography (CT) scan with no enhancement after injection of intravenous contrast media has been observed in two completely different clinical settings. On the one hand, BAS is the result of ischemic necrosis and subsequent hemorrhagic infarction in patients with sepsis and hypotension in critically ill situations. On the other hand, BAS is the result of microvascular thrombosis, ischemia, and secondary inflammatory swelling in the setting of thrombotic conditions such as antiphospholipid syndrome (APS), heparin-induced thrombocytopenia and thrombosis (HITT), and thrombocythemia. In this study we present evidence that the etiology of unilateral or BAS in reported cases of essential thrombocythemia (ET) and polycythemia vera (PV) is similar to the etiology of microvascular circulation disturbances in thrombocythemia caused by platelet-mediated inflammation and thrombosis in the peripheral, cerebral, and/or coronary endarterial microvascular circulation.