Abstract
A 64-year-old man was admitted to our institution due to anasarca and lymphadenopathy. His blood pressure was 100/60 mmHg under vasopressor support and oxygen saturation was 85% despite oxygen supplementation. Blood tests showed anemia and hypoalbuminemia, and soluble interleukin-2 receptor was 47,920 U/mL. Computed tomography demonstrated marked subcutaneous edema and a large volume of pleural fluid in the thoracic cavity associated with lung atelectasis. A biopsy of the axillary lymph node revealed anaplastic lymphoma receptor tyrosine kinase-positive (ALK+) anaplastic large cell lymphoma (ALCL); ALK was expressed solely in the cytoplasm. G-banding revealed two marker chromosomes derived from chromosome 2, determined as inv(2)(p23q35) and der(2)inv(2)(p23q35)del(2)(p21) by combination with fluorescence in situ hybridization using the Vysis ALK probe. ATIC-ALK fusion mRNA was confirmed by reverse transcriptase-mediated polymerase chain reaction and sequencing. The patient was emergently admitted to the intensive care unit to be treated by mechanical ventilation and continuous hemodiafiltration. However, he responded poorly to chemotherapy and died of disease progression. Postmortem examination revealed that the lymphoma had disseminated predominantly in the lung; multiple nodular lesions composed of lymphoma cells were distributed in the organizing lung parenchyma and associated with bronchovascular bundles. The alveoli and small airways around the nodules were filled with collagenous fibrous tissue. This report indicates that ALK+ ALCL can occur in older individuals and the clinical outcome may be poor in elderly patients. It is possible that the presenting symptoms (i.e., anasarca and cardiopulmonary failure) resulted from systemic capillary leak syndrome, and that the disseminated lung involvement of ALCL affected the organizing process of the lung parenchyma.
