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Disseminated cryptococcosis with granuloma formation in idiopathic CD4 lymphocytopenia
Hayato Shimizu1, Shigeo Hara2, Hiroaki Nishioka1
1Department of General Internal Medicine, Kobe City Medical Center General Hospital, Hyogo, Japan.
Insights
Idiopathic CD4 lymphocytopenia (ICL) is a rare condition. This case demonstrates that granulomas with CD4 T-cells can form in ICL patients with cryptococcal infections.
Area of Science:
- Immunology
- Infectious Diseases
- Rare Diseases
Background:
- Idiopathic CD4 lymphocytopenia (ICL) is a rare condition characterized by a significant decrease in CD4 T-cells without HIV infection.
- CD4 T-cells are crucial for forming granulomas during cryptococcal infections.
- The ability of ICL patients to form granulomas has been uncertain.
Observation:
- A 39-year-old woman with ICL presented with disseminated cryptococcal infection.
- She had a lung mass, osteolytic lesion, and subcutaneous mass, along with elevated C-reactive protein.
- Biopsy revealed granulomas containing CD4 T-cells within the lung mass.
Findings:
- The patient had severe CD4 lymphocytopenia (33 cells/μL) but no HIV infection.
- Cryptococcus neoformans was identified as the causative agent.
- Granuloma formation, including CD4 T-cells, was observed despite profound lymphocytopenia.
Implications:
- This case confirms that granulomas containing CD4 T-cells can form in ICL patients with cryptococcal infections.
- It highlights the complex interplay between CD4 T-cell counts and immune responses in rare diseases.
- Understanding granuloma formation in ICL is vital for managing opportunistic infections.
Abstract:
Idiopathic CD4 lymphocytopenia (ICL) is a rare disease characterized by marked loss of CD4 T-cells without human immunodeficiency virus infection. CD4 T-cells play an important role in granuloma formation in cryptococcal infection. Thus far, among ICL patients, it has not been concluded definitely whether granuloma is formed or not. We report the case of a 39-year-old woman with ICL and disseminated cryptococcal infection with granuloma formation. She was referred to our department because of a lung mass, osteolytic lesion, and a subcutaneous mass identified on a computed tomography scan, and an elevated C-reactive protein level. Cryptococcus neoformans was isolated from the tissues. She also had marked CD4 lymphocytopenia (33 cells/μL), without human immunodeficiency virus infection. In a biopsy specimen of the lung mass, granulomas containing CD4 T-cells were observed. The cryptococcosis was treated with liposomal amphotericin B followed by fluconazole and she was found to be cured. The CD4 T-cell count was persistently low. This case showed that granulomas containing CD4 T-cells can be formed in ICL patients with cryptococcal infection despite very low CD4 T-cell counts.
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