Idiopathic CD4+ T lymphocytopenia with epidermodysplasia verruciformis-like skin eruption, Nocardia farcinica brain abscesses and pulmonary tuberculosis: A case report with fatal outcome

Göktay, Fatıh; Mansur, Ayşe Tülin; Erşahin, Mehmet; Adaleti, Rıza; Güneş, Pembegül

doi:10.1111/j.1346-8138.2010.01117.x

Cited by 5 publications

(4 citation statements)

References 10 publications

(11 reference statements)

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“…In our series, patients had opportunistic infections that have already been described in the setting of ICL, including Cryptococcus neoformans meningitis, 34 Pneumocystis jirovecii pneumonia, 20 and mycobacterial 26 and JC virus 15 infection. However, to our knowledge, Nocardia infection was previously reported in only 2 patients with ICL, 11 , 28 and cutaneous Alternaria species infection has never been described. Although ICL patients have been reported with HPV infection, 2 the follow-up of these patients revealed that mucocutaneous lesions due to HPV were common in ICL and can be responsible for neoplasms and/or require surgical treatment.…”

Section: Discussionmentioning

confidence: 72%

Idiopathic CD4 Lymphocytopenia

et al. 2014

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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 40 patients (24 female) of mean age 44.2 ± 12.2 (19–70) years. Patients underwent T-lymphocyte phenotyping and lymphoproliferation assay at diagnosis, and experiments related to thymic function and interferon (IFN)-γ release by natural killer (NK) cell were performed. Mean follow-up was 6.9 ± 6.7 (0.14–24.3) years. Infectious, autoimmune, and neoplastic events were recorded, as were outcomes of interleukin 2 therapy.In all, 25 patients had opportunistic infections (12 with human papillomavirus infection), 14 had autoimmune symptoms, 5 had malignancies, and 8 had mild or no symptoms. At the time of diagnosis, the mean cell counts were as follows: mean CD4 cell count: 127/mm3 (range, 4–294); mean CD8: 236/mm3 (range, 1–1293); mean CD19: 113/mm3 (range, 3–547); and mean NK cell count: 122/mm3 (range, 5–416). Most patients had deficiency in CD8, CD19, and/or NK cells. Cytotoxic function of NK cells was normal, and patients with infections had a significantly lower NK cell count than those without (p = 0.01). Patients with autoimmune manifestations had increased CD8 T-cell count. Proliferation of thymic precursors, as assessed by T-cell rearrangement excision circles, was increased. Six patients died (15%). CD4 T-cell count <150/mm3 and NK cell count <100/mm3 were predictors of death.In conclusion, ICL is a heterogeneous disorder often associated with deficiencies in CD8, CD19, and/or NK cells. Long-term prognosis may be related to initial CD4 and NK cell deficiency.

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Section: Discussionmentioning

confidence: 72%

Idiopathic CD4 Lymphocytopenia

et al. 2014

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“…One, similar to our patient, was associated with N. farcinica brain abscesses, and the second nocardiosis case was disseminated N. asteroides without brain abscesses [ 5 ]. The patient with the Nocardia brain abscesses was a 19-year-old heterosexual male who had accompanying epidermodysplasia verruciformis-like skin eruptions with dysplasia by HPV-16, along with pulmonary tuberculosis [ 5 ]. The patient died of respiratory failure during treatment [ 5 ].…”

Section: Discussionmentioning

confidence: 79%

“…The patient with the Nocardia brain abscesses was a 19-year-old heterosexual male who had accompanying epidermodysplasia verruciformis-like skin eruptions with dysplasia by HPV-16, along with pulmonary tuberculosis [ 5 ]. The patient died of respiratory failure during treatment [ 5 ]. After a thorough review of the literature, the case of N. farcinica brain abscess of the 19-year-old male was the only case of Nocardia brain abscesses reported in a patient with Idiopathic CD4 + Lymphocytopenia [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

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NocardiaBrain Abscess and CD4⁺Lymphocytopenia in a Previously Healthy Individual

Adjamian

Kikam

Wessell

et al. 2015

Case Reports in Immunology

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Nocardia brain abscesses are a known occurrence in patients with immunocompromised conditions. Nocardial infection is commonly an unfortunate sequela to other complications which these patients are being followed up and treated for. The incidence of nocardial brain abscess in an otherwise healthy patient is extremely rare. We present a case of Nocardia brain abscess in a previously healthy individual, who, upon workup for vision and gait abnormalities, was shown to have multiple brain abscesses and a decreased absolute CD4+ lymphocyte count. Adding to the rarity of our case, the finding of lymphocytopenia in our patient was unrelated to any known predisposing condition or infectious state.

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Idiopathic CD4 lymphocytopenia

Yarmohammadi

Cunningham‐Rundles

2017

Annals of Allergy, Asthma & Immunology

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Background Idiopathic CD4 lymphocytopenia (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. Objective To describe the clinical presentation, treatment strategies, and outcome of patients with ICL seen in a single referral center. Methods In a retrospective study, from January 1993 to January 2014, the demographic characteristics, clinical presentation, and treatments of patients diagnosed with ICL were reviewed. Results Twenty-four patients (14 female [58%] and 10 male [42%]) were evaluated. The mean age was 45 ± 17.6 years (range 7–76 years). Mean CD4 and CD8 T-cell counts at the time of diagnosis were 119 ± 84/mm3 (range 4–294/mm3) and 219 ± 258/mm3 (range 7–630/mm3), respectively. Seventeen patients (71%) had opportunistic infections, 4 (17%) had malignancies, and 3 (13%) had unexplained demyelinating disease and neurologic problems. Most patients had normal levels of immunoglobulins. Thirteen patients had abnormally low to absent response to phytohemagglutinin, concanavalin A, and antigens (candida and tetanus). Three patients had resolution of warts and 1 had mycobacterial lung infection on interleukin-2 with increases in CD4 count. The 11 patients on trimethoprim and sulfamethoxazole had no further hospital admissions for infections. Conclusion The pathogenesis of ICL remains unclear. Although only some patients are healthy, most patients present with opportunistic infections. There is no known standard treatment aside from prophylactic antibiotics.

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Idiopathic CD4+ T lymphocytopenia with epidermodysplasia verruciformis-like skin eruption, Nocardia farcinica brain abscesses and pulmonary tuberculosis: A case report with fatal outcome

Cited by 5 publications

References 10 publications

Idiopathic CD4 Lymphocytopenia

Idiopathic CD4 Lymphocytopenia

NocardiaBrain Abscess and CD4⁺Lymphocytopenia in a Previously Healthy Individual

Idiopathic CD4 lymphocytopenia

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Idiopathic CD4+ T lymphocytopenia with epidermodysplasia verruciformis-like skin eruption, Nocardia farcinica brain abscesses and pulmonary tuberculosis: A case report with fatal outcome

Cited by 5 publications

References 10 publications

Idiopathic CD4 Lymphocytopenia

Idiopathic CD4 Lymphocytopenia

NocardiaBrain Abscess and CD4+Lymphocytopenia in a Previously Healthy Individual

Idiopathic CD4 lymphocytopenia

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NocardiaBrain Abscess and CD4⁺Lymphocytopenia in a Previously Healthy Individual