Idiopathic CD4 lymphocytopenia (ICL) is a rare and underrecognized immunodeficiency characterized by persistent CD4⁺ T-cell depletion in the absence of HIV infection or other identifiable causes. Patients are often diagnosed only after presenting with severe opportunistic infections. Massive hemoptysis is a life-threatening complication typically attributed to a single pulmonary pathology, and the coexistence of multiple opportunistic infections in HIV-negative patients is exceedingly rare. We report a 29-year-old previously healthy female who presented with progressive cough, fever, and hemoptysis, culminating in life-threatening massive hemoptysis requiring intensive care. Imaging demonstrated diffuse tree-in-bud nodularity with necrotizing left upper lobe consolidation. Microbiologic evaluation revealed concurrent pulmonary tuberculosis, necrotizing methicillin-resistant Staphylococcus aureus pneumonia, and Pneumocystis jirovecii pneumonia. HIV testing was repeatedly negative. Subsequent immunologic evaluation demonstrated persistent isolated CD4 lymphopenia on serial testing, establishing a diagnosis of ICL. The patient was successfully treated with targeted antimicrobial therapy and supportive management, with stabilization of hemoptysis and clinical improvement. This case highlights a rare presentation of massive hemoptysis caused by synergistic pulmonary infections in the setting of previously undiagnosed ICL. The presence of Pneumocystis jirovecii pneumonia in an HIV-negative patient should prompt immediate evaluation for underlying cellular immune dysfunction. Early recognition of overlapping infectious etiologies and structured immune assessment are critical to timely diagnosis and optimal management in similar high-risk presentations.
Palacio et al. (Sun,) studied this question.
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