Primary pulmonary non-Hodgkin's lymphoma (PP-NHL) poses diagnostic challenges owing to its rarity, nonspecific clinical symptoms, and elusive imaging findings. We present a case of a 67-year-old woman who was admitted to the hospital with exacerbation of cough and dyspnea. On admission, her SpO2 was 88% (10 L/min reservoir mask), with bilateral diffuse ground-glass opacities, centrilobular nodules, and thickened interlobular septa in both lung fields. There was no significant lymphadenopathy, apart from small periaortic lymph nodes. Although she had severe respiratory failure, transbronchial lung biopsy (TBLB) was performed to confirm the diagnosis. Septic shock and acute renal failure necessitated vasopressor support along with the initiation of methylprednisolone 1 g pulse therapy. Cytology and histopathology confirmed anaplastic lymphoma kinase (ALK) + anaplastic large cell lymphoma (ALCL). Respiratory improvement allowed ventilator withdrawal by day 8, and cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone (CHOP) therapy commenced on day 18. Although ALCL constitutes 13% of reported PP-NHL cases, ALK+ ALCL with diffuse pulmonary involvement is rare. For patients with severe respiratory failure, the life-threatening complications of TBLB should be fully considered. For our patient, the diagnostic value of TBLB was high due to her complex medical condition, which included respiratory failure, acute renal failure, and sepsis.
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