Reappraisal of IdiopathicCD4Lymphocytopenia at 30 Years. IdiopathicCD4lymphocytopenia (ICL) is a clinical syndrome that is defined by CD4 lymphopenia of less than 300 cells per cubic millimeter in the absence of any primary or acquired cause of immunodeficiency. Some 30 years after its original identification, ICL has remained a disease of obscure cause, with limited evidence with respect
Disseminated nontuberculous mycobacterial infection in a patient with idiopathicCD4lymphocytopenia and IFN-γ neutralizing antibodies: a case report. Disseminated nontuberculous mycobacterial (NTM) infection usually occurs in immunodeficient patients, such as those with human immunodeficiency virus infection and idiopathic CD4 lymphopenia. However, disseminated NTM diseases have also been . Histopathological evaluation of the subcutaneous mass in the right inguinal region showed numerous granulomas consisting of epithelioid cells with Langhans-type giant cells. He was diagnosed with idiopathicCD4lymphocytopenia. Interestingly, he also had anti-IFN-γ autoantibodies with suppression of IFN-γ-dependent signal transducer and activator of transcription 1 (STAT1) phosphorylation. Two-drug combination
Disseminated muco-cutaneous leishmaniasis in a traveller with idiopathicCD4lymphocytopenia. We reported here a case of disseminated cutaneo-mucosal leishmaniasis caused by L. braziliensis in a traveller returning from Bolivia, probably favoured by an underlying idiopathicCD4-lymphocytopenia. Third-line therapy with 51 mg/kg total dose of liposomal amphotericin B led to a sustained complete
Pulmonary Cryptococcus infections as a manifestation of idiopathicCD4lymphocytopenia: case report and literature review. IdiopathicCD4lymphocytopenia (ICL) is a rare clinical disease with relative CD4 deficiency in the absence of HIV infection. The pathogenicity of ICL is poorly understood with an unclear incidence rate in the general population. Sequelae of ICL includes AIDS-defining
Humanized mouse models reveal an immunologic classification of idiopathicCD4lymphocytopenia subtypes. IdiopathicCD4lymphocytopenia (ICL) is a clinically heterogeneous immunodeficiency disorder defined by low numbers of circulating CD4+ T cells and increased susceptibility to opportunistic infections. CD8+ T cells, NK, and/or B cells may also be deficient in some patients. To delineate
IdiopathicCD4lymphocytopenia: Pathogenesis, etiologies, clinical presentations and treatment strategies. IdiopathicCD4lymphocytopenia (ICL) is a rare condition characterized by an unexplained deficit of circulating CD4 T cells leading to increased risk of serious opportunistic infections. The pathogenesis, etiology, clinical presentation, and best treatment options remain unclear
A rare case of disseminated Sporothrix schenckii with bone marrow involvement in a patient with idiopathicCD4lymphocytopenia is a pathogen with a predilection for dissemination in immunocompromised individuals, often with HIV. We report a case of disseminated sporotrichosis in an unfortunate 25 year old male (without HIV) who was originally treated for presumed pneumonia. The patient
Osteomyelitis due to multiple rare infections in a patient with idiopathicCD4lymphocytopenia A 26-year-old male patient presented with features suggestive of osteomyelitis involving the entire left femur, hip joint and knee joint. Culture from the debrided tissue grew and he was treated with sensitivity based antibiotics but the symptoms did not resolve. The synovial biopsy showed
IdiopathicCD4lymphocytopenia. IdiopathicCD4lymphocytopenia (ICL) is a rare disorder of unknown etiology. Diagnostic criteria include a persistent CD4 T-cell lymphopenia with no underlying primary or secondary immune deficiencies and a CD4 T-cell count of 300 cells/mL or 20% total lymphocyte on multiple occasions. To increase awareness of ICL and to provide a review of the clinical
Clinically HIV but negative serology: Think of idiopathicCD4+ lymphocytopeniaidiopathicCD4(+) lymphocytopenia (ICL) is a rare disorder characterized by the presence of depleted CD4 cell line without the presence of HIV infection. Slight male preponderance is noticed and is usually seen in the middle age group. Opportunistic infections are the reason for their discovery and here we describe
Administration of interleukin-7 increases CD4 T cells in idiopathicCD4lymphocytopenia. Idiopathic CD4 lymphopenia (ICL) is a rare syndrome defined by low CD4 T-cell counts (<300/µL) without evidence of HIV infection or other known cause of immunodeficiency. ICL confers an increased risk of opportunistic infections and has no established treatment. Interleukin-7 (IL-7) is fundamental
Isolated cutaneous cryptococcosis in clinically unsuspected idiopathicCD4lymphocytopeniaIdiopathicCD4lymphocytopenia first defined in 1992 by the U.S. Centers for Disease Control and Prevention, as the repeated presence of a CD4(+) T-lymphocyte count of fewer than 300 cells/cumm or of <20% of total T-cells with no evidence of human immunodeficiency virus (HIV) infection and therapy
IdiopathicCD4lymphocytopenia: clinical and immunologic characteristics and follow-up of 40 patients. Idiopathic CD4 T lymphocytopenia (ICL) is a rare and severe condition with limited available data. We conducted a French multicenter study to analyze the clinical and immunologic characteristics of a cohort of patients with ICL according to the Centers for Disease Control criteria.We recruited
IdiopathicCD4lymphocytopenia manifesting as refractory genital dysplasia. IdiopathicCD4lymphocytopenia is an immunodeficiency disorder with low absolute CD4 T-lymphocyte count with no evidence of human immunodeficiency virus or other known cause. A 22-year-old woman presented with a high-grade Pap test result. Work-up demonstrated cervical intraepithelial neoplasia 3 and vaginal intraepithelial neoplasia 3 with extensive condyloma. She presented 6 months after her initial treatment with recurrent disease and was referred to the immunology department, where she was found to have profound lymphopenia. After further evaluation, idiopathicCD4lymphocytopenia was diagnosed. IdiopathicCD4lymphocytopenia is a rare acquired immunodeficiency. Although genital dysplasia is common in young
Vulval and perianal intraepithelial neoplasia: response to chemoradiotherapy in a woman with idiopathicCD4lymphocytopenia. Treatment of multicentric or multifocal anogenital intraepithelial neoplasia is challenging, especially if the patient has immunodeficiency. We report the case of a woman with idiopathicCD4lymphocytopenia and vulval and perianal intraepithelial neoplasia with early
Recurrent pulmonary nocardiosis due to Nocardia Otitidiscaviarum in a patient with isolated CD4 lymphocytopenia: a case report. IdiopathicCD4lymphocytopenia (ICL) is an underdiagnosed immunodeficiency syndrome characterised by persistent low CD4 counts in the absence of HIV and other causes of lymphocytopenia. ICL patients are susceptible to opportunistic infections, with human papillomavirus
. Repeated lymph node biopsies with analytic cytometric immunophenotypic analysis were normal, as was the bone marrow biopsy with detailed immunophenotypic and cytogenetic studies. The patient was hence being treated as a case of idiopathicCD4lymphocytopenia.
Idiopathic lymphocytopenia. IdiopathicCD4⁺ lymphocytopenia (ICL) is defined by the reduction of the main lymphocyte subtype in peripheral blood and CD4⁺ T cells below 300/μl in the absence of any secondary known causes of lymphopenia, including viral causes. The present review aims to state the latest available data on clinical, pathological and therapeutic aspects related to ICL, published
Psoriasis associated with idiopathic CD4+ T-cell lymphopenia: a regulatory T cells defect? IdiopathicCD4(+) lymphocytopenia (ICL) is a rare immunodeficiency syndrome of unknown origin for which the increased risks of opportunistic infections and of malignancies have been well established; however, skin dysimmune diseases, including psoriasis, have been scarcely reported up to now. We