Polycythemia vera (PV) is a clonal myeloproliferative neoplasm (MPN) of hematopoietic stem cells. Although the management of MPN patients generally focuses on the prevention of thromboembolic events caused by hypercoagulability, it is true that the patients with hematological malignancy often suffer from pulmonary diseases with atypical radiological patterns. We present here a 56-year-old woman with PV harboring a JAK2(V617F) mutation that had a diffuse reticulonodular pattern on chest radiography and was initially suspected of having military tuberculosis. Pathological assessment of a video-assisted thoracoscopic surgery lung biopsy revealed that the lesions were in fact organizing pneumonia (OP). Interestingly, pulmonary extramedullary hematopoiesis with a diffuse plugging of the alveolar blood capillaries by numerous atypical megakaryocytes was also observed around the granulation components. The histological findings of our case of unusual OP suggest that local activated neoplastic megakaryocytes and platelets played an important role in the development of spreading fibrotic lesions. JAK2 mutation or the preleukemic phase of MPN may accelerate the activation of megakaryocytes and result in the proliferative process of fibrosis.
[Show abstract][Hide abstract] 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.
American Journal of Hematology 02/2006; 81(2):124-7. DOI:10.1002/ajh.20509 · 3.80 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Bronchiolitis obliterans with organizing pneumonia (BOOP) is an infrequently encountered clinical condition that can mimic a number of other pathologic lung processes. The presentation of this treatable condition in cancer patients has not been described in any large series. We conducted a retrospective study of patients with BOOP at Memorial Sloan-Kettering Cancer Center, NewYork, NY, U.S.A. from January 1992 to December 1999. The type and treatment of primary cancer, clinical and radiographic features of initial BOOP presentation, and outcome following therapy were recorded. Forty-three patients with an underlying diagnosis of cancer were found on lung biopsy to have BOOP as an isolated entity. BOOP was encountered in patients with a variety of clinical presentations, and many types of malignancies. The symptom patterns were non-specific, as were the physiological abnormalities. The only clear relationship between the underlying malignancyand the diagnosis of BOOP at presentation was in the chest radiographic findings. Patients with solid organ tumors were more likely to have nodular or mass like radiographic abnormalities (81%) than to have diffuse infiltrates (19%). We observed the opposite pattern in patients with hematologic malignancies (22% vs.67%). The vast majority of patients recovered from this condition. In conclusion, For cancer patients, BOOP represents a treatable cause of lung disease with protean manifestations. BOOP can mimic pulmonary malignancy and pulmonary infection. In cancer patients, the evaluation of new pulmonary symptoms accompanied by radiographic changes should include a consideration of this diagnosis.
Respiratory Medicine 05/2002; 96(4):280-6. DOI:10.1053/rmed.2001.1269 · 3.09 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Patients with hematologic malignancies are at increased risk of developing tuberculosis because of the T-cell immunodeficiency associated with the disease and/or its treatment. The objective of this study was to evaluate risk factors, clinical findings and the attributable mortality associated with tuberculosis in patients with hhematologic malignancies.
We performed a retrospective review of the clinical records of 917 patients observed between 1990 and 2000. A risk classification for tuberculosis (low vs. high risk) was developed based on the underlying disease and previous exposure to agents that deplete T-cell mediated immunity. Patients with and without tuberculosis were compared by univariate and multivariate analyses with regard to demographic and clinical characteristics, underlying diseases and their treatment. The attributable mortality was assessed by matching cases and controls using the independent variables identified as risk factors as the matching parameters, and was estimated by subtracting the crude mortality of the controls from the crude mortality of the cases.
We found 24 cases of tuberculosis (2.6%). Risk factors by multivariate analysis were malnutrition (OR 55.66, 95% CI 2.47--1254.82), use of fludarabine (OR 6.08, 95% CI 1.22--30.25), use of corticosteroids (OR 5.32, 95% CI 1.15--24.39) and belonging to the high-risk group (OR 3.73, 95% CI 1.09--12.76). The crude mortality of patients with tuberculosis was 75%, and the attributable mortality was 62.5% (risk ratio 6.0, 95% CI 2.03--17.70).
The mortality attributable to tuberculosis is high in patients with hematologic malignancies. The identification of risk factors may be useful for evaluating strategies to be applied in high-risk patients.
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