Un polmone ematologico Rassegna di Patologia dell'Apparato Respiratorio. Fascicolo 6/2014

This article describes the case of a 58 years old male with abrupt onset of dyspnoea, fever, bilateral pulmonary consolidations and acute respiratory failure. Clinical reasoning and laboratory findings (pancytopenia, increased values of ferritin, transaminases and LDH), together with the presence of splenomegaly, led to the hypothesis of hemophagocytic syndrome. This rare and serious disease can be primitive or, more frequently, secondary, in particular to infections, inflammatory diseases or malignancy, mostly lymphoma. Cytologic analysis of BAL (broncho-alveolar lavage) showed atypical pneumocytes (diffuse alveolar damage), alveolar hemosiderin-laden macrophages and blast lymphoid cells, thus confirming the suspicion of hemophagocytic syndrome secondary to a neoplastic disease. The bone marrow biopsy led to the definitive diagnosis of extranodal peripheral T-cell lymphoma, with a cytotoxic phenotype. The hemophagocytic syndrome is characterized by non-malignant proliferation of histiocytes and uncontrolled hemophagocytosis in bone marrow, spleen and lymph nodes; when associated with lymphoma, it has acute and explosive onset, often masking the underlying lymphoproliferative disease. In this particular case report, the respiratory manifestation was due to the presence in the lung of both the lymphoproliferative process itself and the secondary hemophagocytic syndrome; this combination of elements was found to be quite clear on BAL cytology. In conclusion, pulmonologists are not usually directly involved in the diagnosis and management of these diseases, however, the assessment of respiratory problems can not be confined to the lung