Progressive disseminated histoplasmosis in idiopathic CD4 lymphocytopenia an underdiagnosed combination - a case report

Progressive disseminated histoplasmosis (PDH) usually presents as fever, anemia, leukopenia, hepatosplenomegaly, lymphadenopathy and pulmonary symptoms. There are few reports showed the association of Idiopathic CD4 lymphocytopenia (ICL) with histoplasmosis. We describe a 65-year-old female presented with history of fever, papulo-nodular rash and significant weight loss and diagnosed as progressive disseminated histoplasmosis. All immunocompromised conditions were ruled out. In addition, her 2 consecutive CD4 counts were below 300. The patient was diagnosed with PDH associated with ICL. Patient showed significant improvement with liposomal amphotericin B and itraconazole. Absolute CD4 counts should be done in all cases of progressive disseminated histoplasmosis even in HIV negative individuals to rule out associated ICL.

Idiopathic Cd4+ Lymphocytopenia In Overlap Syndrome (Systemic Sclerosis With Dermatopolimyositis)

Background : Idiopathic CD4 T cell lymphocytopenia (ICL) is a rare syndrome with varied clinical manifestation, characterized with lymphopenia and decreased in CD4 level without HIV infection or other possible cause of immunodeficiency state. Autoimmune diseases might be a clinical manifestation of ICL. However, it is not known whether ICL triggered an autoimmune diseases, or it is a complication of said diseases. Objective : Awareness of ICL in patient with known autoimmune diseases whom admitted to the hospital for severe infection. Methods : This case report showed a 24-years old woman with prolonged fever since 4 moths ago. It was accompanied with oral ulcers, skin rash in face and trunks, and weakness of lower extremities. She was diagnosed with systemic sclerosis since 2016 and routinely came to rheumatology outpatient clinic in Hasan Sadikin Hospital but stopped coming for past 4 months since pandemic. Her current medication was only 4 mg of methylprednisolone. Results : She had high temperature (38.5 degree Celsius) and tachycardia. Physical examination revealed a single lymphadenopathy at neck. Raynaud phenomenon, calcinosis, and sclerodactyly was found in lower extremities. Dermatomyositis was diagnosed based by heliotropic skin rash. Laboratory tests showed leukopenia, absolute lymphocyte count 135.2 cell/mm3, absolute CD4 39/uL, CK level of 3296 and nonreactive anti-HIV. The patient underwent empirical antibiotic treatment, but unfortunately passed away. Conclusion : ICL is a rare case, following an infection, autoimmune diseases, or unspecified malignancy. Clinician’s awareness toward ICL could prevent fatal opportunistic infection which often happens to patients with immunodeficiency state.

Preserved MAIT cell numbers and function in idiopathic CD4 lymphocytopenia

Mucosal-associated invariant T (MAIT) cells constitute a subset of unconventional, MR1-restricted T-cells involved in antimicrobial responses as well as inflammatory, allergic and autoimmune diseases. Chronic infection and inflammatory disorders as well as immunodeficiencies are often associated with decline and/or dysfunction of MAIT cells. Herein, we investigate the MAIT cells in patients with idiopathic CD4 + lymphocytopenia (ICL), a syndrome characterized by consistently low CD4 T-cell counts (<300 cell/µL) in the absence of HIV infection or other known immunodeficiency, and by susceptibility to certain opportunistic infections. The numbers, phenotype and function of MAIT cells in peripheral blood were preserved in ICL patients compared to healthy controls. Furthermore, administration of IL-7 to ICL patients expanded the CD8 + MAIT cell subset, with maintained responsiveness and effector functions after IL-7 treatment. In conclusion, ICL patients maintain normal levels and function of MAIT cells preserving some antibacterial responses despite the deficiency in CD4 + T cells.