Agnogenic myeloid metaplasia with pulmonary hematopoiesis.
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.
Boula
Anna
A
University Hospital of Heraklion, Department of Hematology, Heraklion, Crete, Greece.
Mantadakis
Elpis
E
Xilouri
Irini
I
Foudoulakis
Andreas
A
Samonis
George
G
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
D006918:Hydroxyurea