CD4+ T lymphocytopenia associated with sideropenia: case report

Importance of the issue: Idiopathic CD4 T lymphocytopenia is an unusual immune defect in which there is an unexplained deficit of CD4 T cells. This case presents a 39-year-old female patient, with CD4+ T lymphocytopenia, who was not infected with immunosuppressive viruses neither was she subjected to immunosuppressive therapies. Comments: While monitoring the patient, she was found to have very low serum ferritin and, after parenteral iron therapy, there were changes in CD4+ cell levels, indicating that, in this case, lymphocytopenia was secondary to sideropenia. The patient is being kept under strict control of serum iron and periodic immunological evaluation, and she has not showed any clinical and/or laboratory adverse events so far. It is known that iron deficiency is an important factor in the genesis of immunological changes that occur in patients with iron deficiency anemia. It is important to understand the effects of iron deficiency on the immune system due to its high prevalence worldwide. Moreover, it could also help to clarify several cases of idiopathic CD4 lymphocytopenia (AU)

Importância da questão: Linfocitopenia T CD4+ idiopática é um defeito imune incomum em que há um déficit inexplicável de células T CD4. Este caso clínico apresenta uma paciente do sexo feminino de 39 anos de idade, com linfocitopenia T CD4+, que não estava infectada por vírus imunossupressores nem foi submetida a terapias imunossupressoras. Comentários: Durante o acompanhamento da paciente, ela apresentou níveis muito baixos de ferritina sérica e, após a terapia parenteral de ferro, houve aumento da quantidade de células CD4+, indicando que, neste caso, linfocitopenia era secundária à sideropenia. A paciente estava sendo mantida sob rigoroso controle de ferro sérico e avaliação imunológica periódica, e não mostrou quaisquer eventos adversos clínicos e/ou laboratoriais até o momento. Com base em mudanças na reatividade imunológica dos pacientes observadas por outros pesquisadores após a suplementação com ferro, é evidente que a deficiência de ferro seria um fator importante na gênese das alterações imunológicas que ocorrem em pacientes com anemia ferropriva. É importante compreender os efeitos da deficiência de ferro no sistema imune, devido à sua elevada prevalência mundial. Essas informações também auxiliariam a esclarecer vários casos de linfocitopenia T CD4+ idiopática. (AU)

Interleucina-2 no tratamento adjuvante de paciente com linfocitopenia CD4 idiopática e tuberculose disseminada / Interleukin 2 as adjuvant treatment in a patient with idiopathic CD4 lymphocytopenia and disseminated tuberculosis

Linfocitopenia CD4 idiopática (LCI) é uma imunodeficiência rara e grave caracterizada por uma diminuição inexplicável da contagem absoluta de linfócitos T CD4, a qual está associada a infecções oportunistas. Existem poucos relatos de casos na literatura que descrevem a IL2 como uma opção terapêutica em infecções oportunistas associadas à LCI. Relatamos os benefícios da adição de IL2 ao tratamento padrão em um paciente com ICL e infecções oportunistas. Um homem de 38 anos de idade foi internado por acidente vascular cerebral isquêmico devido à vasculite infecciosa. A análise do líquido cefalorraquidiano mostrou meningite neutrofílica. Cultura e PCR foram positivos para Mycobacterium tuberculosis. A tomografia de tórax foi compatível com tuberculose pulmonar. O paciente também apresentava candidíase oral, onicomicose e dermatite seborreica. A contagem de células sanguíneas mostrou linfocitopenia. O tratamento padronizado para tuberculose disseminada (RIPE) e fluconazol foram iniciados e mantidos em casa após a alta do paciente. Após cinco meses de seguimento, o paciente foi encaminhado ao imunologista clínico, pois não apresentava melhora clínica significativa, tendo sido hospitalizado diversas vezes. A avaliação imunológica mostrou uma contagem sanguínea de CD4 T consistentemente inferior a 100 células/mm3 e o diagnóstico de LCI foi confirmado (linfocitopenia inexplicável com menos de 300 células/mm3 ou menos de 20% de células T CD4+ em mais de uma ocasião com pelo menos 2 meses de intervalo). O paciente também apresentava episódios raros de linfocitopenia de células B e hipogamaglobulinemia, tendo recebido gamaglobulina. Como tratamento adjuvante, a IL2 subcutânea foi associada ao tratamento padronizado. Até agora, o paciente recebeu cinco ciclos consecutivos de IL2, mostrando melhora clínica e aumento da contagem de células T CD4 no sangue, atingindo um valor máximo de 401 células/mm3. As células CD8, B e natural killer também aumentaram. Novas análises do líquido cefalorraquidiano foram normais, e a cultura de Mycobacterium tuberculosis e a PCR foram negativas. Nosso paciente com infecções oportunistas associadas à LCI apresentou evolução laboratorial e clínica favoráveis após a adição de IL2 ao tratamento padrão. Este relato de caso apoia o uso de IL2 como um coadjuvante seguro e potencialmente eficaz para infecções oportunistas associadas à LCI. O caso destaca a importância da avaliação e acompanhamento de pacientes com suspeita de imunodeficiência por imunoalergologistas.

Idiopathic CD4 T lymphocytopenia (ICL) is a rare and severe immunodeficiency disorder characterized by an unexplained decrease of absolute CD4 T-lymphocyte cell counts, which is associated with opportunistic infections. There are few case reports in the literature describing the use of interleukin 2 (IL2) as a therapeutic option in ICL-associated opportunistic infections. We report the benefits of adding IL2 to the standard treatment in a patient with ICL and opportunistic infections. A 38-year-old male patient was admitted with ischemic stroke due to infectious vasculitis. Cerebrospinal fluid analysis revealed neutrophilic meningitis. Culture and PCR were positive for Mycobacterium tuberculosis. Chest CT was compatible with pulmonary tuberculosis. The patient also presented oral candidiasis, onychomycosis, and seborrheic dermatitis. Blood cell count revealed lymphocytopenia. Standardized treatment for disseminated tuberculosis (RIPE therapy) and fluconazole were initiated and maintained at home after the patient's discharge. After five months of follow-up, the patient was referred to a clinical immunologist, due to the absence of significant clinical improvement, with multiple hospitalizations over the follow-up period. Immunological assessment showed CD4 T cell counts consistently below 100 cells/mm3, and the diagnosis of ICL was confirmed (unexplained lymphocytopenia with less than 300 cells/mm3 or less than 20% of CD4+ T cells on more than one occasion at least 2 months apart). The patient also presented rare episodes of B cell lymphocytopenia and hypogammaglobulinemia, treated with gammaglobulin. As an adjuvant treatment, subcutaneous IL2 was added to the standard treatment. So far the patient underwent five consecutive cycles of IL2, showing clinical improvement and increased CD4 T cell counts, reaching a maximum value of 401 cells/mm3. CD8, B and natural killer cells also increased. New cerebrospinal fluid analyses were normal, and new Mycobacterium tuberculosis culture and PCR were negative. Our patient had opportunistic infections associated with ICL and presented favorable laboratory and clinical outcomes after the association of IL2 to the standard treatment. This case report supports the use of IL2 as a safe and potentially effective adjuvant treatment for ICL-associated opportunistic infections. The case highlights the importance of immunological assessment and follow-up of patients with suspected immunodeficiency.

Motor Axonal Neuropathy Associated With Idiopathic CD4+ T-Lymphocytopenia

Idiopathic CD4+ T-lymphocytopenia is a rare immune disorder characterized by an unexplained deficit of CD4+ T cells and results in various opportunistic infections. Herein, we report a case of new onset weakness in a 10-year-old boy secondary to motor axonal neuropathy associated with idiopathic CD4+ T-lymphocytopenia. The patient was referred to rehabilitation for an evaluation of progressive weakness involving all four limbs. A subsequent nerve conduction study and needle electromyography identified motor axonal neuropathy. At that time, laboratory studies specific to the differential diagnosis of motor axonal neuropathy were performed; however, the abnormality noted was a decreased CD4+ T-lymphocyte count. Motor axonal neuropathy represents an uncommon manifestation of idiopathic CD4+ T-lymphocytopenia and is probably associated with an underlying immune process.

Multiple Opportunistic Infections and Primary Central Nervous System Lymphoma in a Patient with Idiopathic CD4+ T-lymphocytopenia

We describe a case of idiopathic CD4+ T-lymphocytopenia (ICL) in a 59-year-old patient who presented with various opportunistic infections. The patient was diagnosed with disseminated Mycobacterium avium infection, cytomegalovirus colitis and retinitis, and esophageal candidiasis. He was successfully treated with anti-mycobacterial drugs, ganciclovir, and fluconazole, respectively. However, the patient was diagnosed with primary central nervous system lymphoma, and then died of a Trichosporon beigelii sepsis during the 2nd cycle of systemic chemotherapy.

Proportion of urinary schistosomiasis among HIV-infected subjects in Benin city, Nigeria

This study aimed to determine the prevalence of urinary schistosomiasis, and the effect of CD4+T cell counts and demographics on its prevalence among HIV-positive patients in Benin City, Nigeria. Urine and blood samples were collected from 2000 HIV-positive subjects. A wet preparation of the urine deposit was examined microscopically to identify ova of Schistosoma haematobium. The blood specimens were analyzed using the flow cytometry for CD4+T-lymphocyte count. An overall prevalence rate of 0.3% was reported. Gender and CD4 count<200 cells/micro L did not affect the prevalence of urinary schistosomiasis, while HIV patients that were single had significantly higher prevalence of urinary schistosomiasis [p=0.002]. The prevalence of urinary schistosomiasis among HIV patients in Benin City is low. CD4+count did not affect the prevalence of urinary schistosomiasis

Idiopathic CD4+ T-cell lymphocytopenia.

Idiopathic CD4+ T lymphocytopenia (ICL) is an unusual immune defect in which there is an unexplained deficit of CD4+ T cells, leading to serious opportunistic infections. In view of the rarity of this clinical entity, we report two cases of ICL who presented with low CD4+ count or percentage and various opportunistic infections like candida, cytomegalovirus, Mycobacterium tuberculosis.

Involvement of CD4+CD25+ regulatory T cells in the pathogenesis of polycythaemia vera / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Regulatory T cells (T(reg)) have been shown to play an important role in the regulation of hematopoietic activity. However, there is no information about the effect of T(reg) cells in the pathogenesis of polycythaemia vera (PV).</p><p><b>METHODS</b>In this study, we investigated the percentage and function of T(reg) cells in the peripheral blood of 21 PV patients and 25 healthy donors. T(reg) cells were identified and characterized as CD4+CD25+ FOXP3+ by flow cytometry. The suppressive activity of CD4+CD25+ T(reg) cells was assessed by the proliferation and cytokine secretion of the co-cultured CD4+CD25- fractions.</p><p><b>RESULTS</b>The results showed that the percentage of T(reg) cells in the peripheral blood of PV patients significantly increased compared to healthy controls ((10.93 +/- 4.02)% vs (5.86 +/- 1.99)%, P < 0.05). Moreover, the mRNA and protein expression of FOXP3 was higher in CD4+CD25+ T(reg) cells. Coordinately, when co-cultured with the activated CD4+CD25- cells, the CD4+CD25+ T(reg) cells showed enhanced suppressive function in PV. Yet, the underlying mechanism for the increased frequency and function of CD4+CD25+ T(reg) cells is still to be clarified.</p><p><b>CONCLUSION</b>T(reg) cells expansion might account for the abnormal T cell immunity in PV patients and thus contribute to the pathogenesis of PV.</p>

A Case of Idiopathic CD4+ T-lymphocytopenia Associated with Autoimmune Hemolytic Anemia / 대한혈액학회지

Idiopathic CD4+ T-lymphocytopenia (ICL) is defined by the CDC as depressed numbers of circulating CD4+ T-lymphocytes (<300 cells/microliter or <20% of the total T cells) on more than one determination, with the absence of HIV infection and other known causes of immunodeficiency. The clinical spectrum of ICL ranges from asymptomatic laboratory abnormalities to severe opportunistic infections that mimic the clinical course of human immunodeficiency virus (HIV) infected patients. There are a few reports of ICL associated with different diseases such as Sjogren's syndrome, pulmonary sarcoidosis, Down syndrome or non-Hodgkin's lymphoma. We describe here a 5-year-old male patient with a three-year history of recurrent otitis media and pulmonary infection, and he was without any risk factors for HIV infection; this patient presented with autoimmune hemolytic anemia and was ultimately found to have idiopathic CD4+ T-lymphocytopenia.

A case of Idiopathic CD4+ T-Lymphocytopenia with disseminated Mycobacterium kansasii infection and Pulmonary alveolar proteinosis / 결핵

Idiopathic CD4+ T-lymphocytopenia is defined as a depletion of CD4+ lymphocytes below 300/mm 3 in the absence of HIV infection or other known causes of immunodeficiency. Many infectious diseases have been reported to be associated with idiopathic CD4+ T-lymphocytopenia, and there have also been a few cases of mycobacterial infection in idiopathic CD4+ T-lymphocytopenia. Until now, it has been unclear as to whether CD4+ T-lymphocytopenia is a predisposing factor for or a consequence of the mycobacterial infection. Pulmonary alveolar proteinosis is an uncommon disease characterized by the intraalveolar deposition of amorphous granular material that stains positive with PAS, and its association with mycobacterial infection has rarely been reported. Recently, we experienced a previously healthy young man who had been diagnosed as idiopathic CD4+ T-lymphocytopenia with disseminated mycobacterium kansasii infection and pulmonary alveolar proteinosis, and report this case.

A Case of Idiopathic CD4 + T Lymphocytopenia Associated with Kaposi's Sarcoma / 대한피부과학회지

Idiopathic CD4+ T lymphocytopenia (ICL) is a new disease entity characterized by depletion of helper T cells apparently without any evidence of HIV infection. We report a case of ICL associated with Kaposis sarcoma (KS) and pneumocystis carinii pneumonia (PCP) in a 34-year old woman. She developed violaceous, protruding masses on scalp, back, both extremities, palms, soles, left. first toe and peritonsillar region for 2 months. These lesions were confirmed as KS by histopathologic findings. Chest X-ray and HRCT findings represented PCP and KS. Absolute deficiency of CD4+ T cell was detected in the count of T cell subsets. Serologic tests for HIV-1, 2 and HTLV I, -II were negative. And she was absent any defined immunodeficiency or therapy associated with decreased levels of CD4+ T cells. By CDC criteria, a diagnosis of ICL was made. Because of aggravation of PCP and lung involuement of KS, she died at 22nd day after admission.