Abstract Background Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder characterized by monocytopenia and profound defects in cellular immunity, predisposing patients to severe mycobacterial infections. Although numerous case reports exist, the clinical features and outcomes of these infections have not been systematically synthesized. Methods We conducted a systematic review following PRISMA guidelines, searching PubMed, Embase, and Web of Science from inception to January 31, 2025, without language restrictions. Studies were eligible if they contained individual patient-level data on microbiologically confirmed mycobacterial infection in HCL. Data on demographics, clinical presentation, organ involvement, pathogen distribution, treatment timing, and outcomes were extracted and analyzed descriptively. Results Thirty-six articles describing 48 patients met the inclusion criteria. The mean age was 53.7 ± 12.7 years, and 39/48 (81%) were male. Disseminated infection was common, occurring in 34/48 (71%) cases, with frequent involvement of lymph nodes (60%), lungs (56%), liver (27%), spleen (25%), and bone marrow (19%). Infections were caused by Mycobacterium tuberculosis complex in 12/48 (25%) and by non-tuberculous mycobacteria in 33/48 (69%), most commonly M. kansasii (55%) and M. avium complex (30%). Overall, 19/48 (40%) patients died, with significantly higher mortality among those with pulmonary involvement (79% vs 41%; p = 0.01). Conclusions Mycobacterial infections in HCL are frequently disseminated and associated with substantial mortality. Clinicians should maintain a high index of suspicion, ensure timely species-level diagnosis, and evaluate for multisystem involvement when managing suspected mycobacterial disease in HCL.
Tirlangi et al. (Thu,) studied this question.