Agnogenic myeloid metaplasia with extramedullary hematopoiesis and fibrosis in the lung. Report of two cases.
نویسندگان
چکیده
Agnogenic myeloid metaplasia (AMM) is a chronic hematologic disorder with a long clinical course, characteristically accompanied by extramedullary hematopoiesis (EMH) in various organs, most commonly the spleen and liver. We describe two cases of AMM with clinically significant and ultimately fatal EMH and associated fibrosis in the lung and pleura. The literature on AMM and EMH involving the lung and pleura is reviewed. Three similar cases were found.
منابع مشابه
Myelofibrosis with myeloid metaplasia (MMM), Idiopathic myelofibrosis, Agnogenic myeloid metaplasia
MMM usually presents with fatigue, weight loss, splenomegaly with or without symptoms. Anemia and various alterations of the white blood cell and/or platelet count are frequently seen at diagnosis. Thrombocytopenia-related bleeding may occur. MMM must be distinguished from myelodysplasia with fibrosis, from acute megakayoblastic leukemia and acute myelofibrosis. As the disease progresses, incre...
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Idiopathic myelofibrosis is a rare chronic myeloproliferative disease leading to extramedullary hematopoiesis (myeloid metaplasia) with splenomegaly. The liver and less frequently other organs including the lung can be involved, therefore portal hypertension is relatively common. Pulmonary hypertension (PH) is only occasionally reported, although recent studies have suggested an association bet...
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Introduction Idiopathic myelofibrosis, or agnogenic myeloid metaplasia, is a chronic myeloproliferative disorder that is characterised by marrow fibrosis and extramedullary haematopoiesis. The origin of the disease remains an enigma and a source of confusion in management, not least because until recently no meaningful progress had been made regarding its pathophysiology. Research during the pa...
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The splenomegaly associated with myelofibrosis and agnogenic myeloid metaplasia should not be considered a manifestation of the fundamental proliferative process, nor should it be considered as necessarily compensatory for reduced marrow haematopoiesis. In deserving cases splenectomy may cause an improvement in the patient's general and haematopoietic status. Removal of the source of functional...
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Results The 384 cases included 206 benign and 178 carcinomas in mixed tumors. Osseous metaplasia was present in 16.1% and calcified areas exclusively in 3.1% of lesions. Among all osseous metaplasia, 33.9% presented some type of extramedullary hematopoiesis, of which 71.4% were classified as myeloid metaplasia and 28.6% as extramedullary hematopoiesis. Myeloid metaplasia cases consisted of 67% ...
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ورودعنوان ژورنال:
- Chest
دوره 105 6 شماره
صفحات -
تاریخ انتشار 1994