Dyspnea secondary to pulmonary hematopoiesis as presenting symptom of myelofibrosis with myeloid metaplasia

Elisa Rumi, Francesco Passamonti, Emanuela Boveri, Mara De Amici, Cesare Astori, Marta Braschi, Carlo Castagnola, Umberto Magrini, Mario Cazzola, Mario Lazzarino

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)124-127
Number of pages4
JournalAmerican Journal of Hematology
Volume81
Issue number2
DOIs
Publication statusPublished - Feb 2006

Keywords

  • Extramedullary hematopoiesis
  • Lung
  • Myelofibrosis
  • Polycythemia
  • Thrombocythemia

ASJC Scopus subject areas

  • Hematology

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