title: Agnogenic myeloid metaplasia with pulmonary hematopoiesis.
abstract: Agnogenic myeloid metaplasia (AMM) is characterized by bone marrow fibrosis, splenomegaly and leukoerythroblastic anemia and is frequently accompanied by extramedullary hematopoiesis (EMH). Pulmonary interstitial EMH associated with myelofibrosis has rarely been described in the medical literature and is usually fatal. We report the case of a 77-year-old man with agnogenic myeloid metaplasia (AMM) treated with hydroxyurea, who seven years after diagnosis presented with dyspnea and severe hypoxemia. Radionuclide bone marrow scanning demonstrated increased tracer activity on the bases of both lungs, consistent with non-hepatosplenic EMH. Pulmonary EMH is rare in patients with AMM, but should be considered in patients with hypoxemia and respiratory distress.
authors.affiliation: University Hospital of Heraklion, Department of Hematology, Heraklion, Crete, Greece.
authors.forename: Anna
authors.initials: A
authors.lastname: Boula
authors.forename: Elpis
authors.initials: E
authors.lastname: Mantadakis
authors.forename: Irini
authors.initials: I
authors.lastname: Xilouri
authors.forename: Andreas
authors.initials: A
authors.lastname: Foudoulakis
authors.forename: George
authors.initials: G
authors.lastname: Samonis
mesh_terms: D000368:Aged; D017809:Fatal Outcome; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D008297:Male; D055728:Primary Myelofibrosis / Q000188:drug therapy / Q000473:pathology*; D012131:Respiratory Insufficiency / Q000209:etiology
chemical_list: D006918:Hydroxyurea