Document Detail

Dyspnea secondary to pulmonary hematopoiesis as presenting symptom of myelofibrosis with myeloid metaplasia.
MedLine Citation:
PMID:  16432861     Owner:  NLM     Status:  MEDLINE    
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.
Elisa Rumi; Francesco Passamonti; Emanuela Boveri; Mara De Amici; Cesare Astori; Marta Braschi; Carlo Castagnola; Umberto Magrini; Mario Cazzola; Mario Lazzarino
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of hematology     Volume:  81     ISSN:  0361-8609     ISO Abbreviation:  Am. J. Hematol.     Publication Date:  2006 Feb 
Date Detail:
Created Date:  2006-01-30     Completed Date:  2006-04-14     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  7610369     Medline TA:  Am J Hematol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  124-7     Citation Subset:  IM    
Copyright Information:
2006 Wiley-Liss, Inc.
Division of Hematology, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
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MeSH Terms
Dyspnea / etiology*
Hematopoiesis, Extramedullary*
Lung / pathology*
Middle Aged
Primary Myelofibrosis / diagnosis*
Tomography, X-Ray Computed

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