title: Dyspnea secondary to pulmonary hematopoiesis as presenting symptom of myelofibrosis with myeloid metaplasia.
abstract: We report a case of a patient with myelofibrosis with myeloid metaplasia (MMM) who presented with progressive dyspnea of unexplained origin. Splenomegaly, blood smear, and bone marrow findings allowed diagnosis of MMM. High-resolution CT chest scan revealed diffuse septal thickening, while echocardiography and electrocardiogram showed no indirect evidence of pulmonary hypertension. Finally, lung biopsy revealed irregularly distributed interstitial fibrosis with islands of erythroblasts, immature granulocytic elements, and dysplastic megakaryocytes, allowing diagnosis of pulmonary extramedullary hematopoiesis (EMH). The patient received hydroxyurea as cytoreductive agent, obtaining a good hematologic response and an improvement of dyspnea. Note that, in this patient, dyspnea was the first clinical symptom of MMM; the dyspnea was not associated with pulmonary hypertension and improved following cytoreductive treatment. This case points to the importance of suspecting pulmonary EMH when unexplained progressive dyspnea occurs in a patient with MMM. Early recognition of pulmonary EMH may prevent PH and favor a better response to therapy.
authors.affiliation: Division of Hematology, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Rumi
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
authors.forename: Emanuela
authors.initials: E
authors.lastname: Boveri
authors.forename: Mara
authors.initials: M
authors.lastname: De Amici
authors.forename: Cesare
authors.initials: C
authors.lastname: Astori
authors.forename: Marta
authors.initials: M
authors.lastname: Braschi
authors.forename: Carlo
authors.initials: C
authors.lastname: Castagnola
authors.forename: Umberto
authors.initials: U
authors.lastname: Magrini
authors.forename: Mario
authors.initials: M
authors.lastname: Cazzola
authors.forename: Mario
authors.initials: M
authors.lastname: Lazzarino
mesh_terms: D001706:Biopsy; D004417:Dyspnea / Q000209:etiology*; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D008168:Lung / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed