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Dyspnea secondary to pulmonary hematopoiesis as presenting symptom of myelofibrosis with myeloid metaplasia
Author(s) -
Rumi Elisa,
Passamonti Francesco,
Boveri Emanuela,
De Amici Mara,
Astori Cesare,
Braschi Marta,
Castagnola Carlo,
Magrini Umberto,
Cazzola Mario,
Lazzarino Mario
Publication year - 2006
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20509
Subject(s) - medicine , myelofibrosis , extramedullary hematopoiesis , pulmonary hypertension , pulmonary fibrosis , myeloid , biopsy , metaplasia , lung biopsy , lung , myeloproliferative neoplasm , bone marrow , pathology , haematopoiesis , stem cell , biology , genetics
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. Am. J. Hematol. 81:124–127, 2006. © 2006 Wiley‐Liss, Inc.