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.
Causality ; Coloring Agents ; Communicable Diseases ; HIV Infections ; Lymphocytes ; Mycobacterium kansasii* ; Mycobacterium* ; Pulmonary Alveolar Proteinosis* ; T-Lymphocytopenia, Idiopathic CD4-Positive*

Child ; Cryptococcosis ; complications ; immunology ; Cryptococcus neoformans ; immunology ; pathogenicity ; Diagnosis, Differential ; Humans ; T-Lymphocytopenia, Idiopathic CD4-Positive ; complications ; diagnosis

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.
Axons ; Diagnosis, Differential ; Electromyography ; Extremities ; Humans ; Immune System Diseases ; Lymphopenia ; Needles ; Neural Conduction ; Opportunistic Infections ; T-Lymphocytes ; T-Lymphocytopenia, Idiopathic CD4-Positive

BACKGROUNDRegulatory 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).

METHODSIn 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.

RESULTSThe 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.

CONCLUSIONT(reg) cells expansion might account for the abnormal T cell immunity in PV patients and thus contribute to the pathogenesis of PV.

Adolescent ; Adult ; Aged ; Female ; Humans ; Interleukin-2 Receptor alpha Subunit ; analysis ; Male ; Middle Aged ; Polycythemia Vera ; etiology ; T-Lymphocytes, Regulatory ; physiology ; T-Lymphocytopenia, Idiopathic CD4-Positive ; physiopathology

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.
Candidiasis ; Central Nervous System ; Colitis ; Cytomegalovirus Infections ; Fluconazole ; Ganciclovir ; Humans ; Lymphoma ; Lymphopenia ; Middle Aged ; Mycobacterium avium ; Opportunistic Infections ; Retinitis ; Sepsis ; T-Lymphocytopenia, Idiopathic CD4-Positive ; Trichosporon

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.
Adult ; Centers for Disease Control and Prevention (U.S.) ; Diagnosis ; Extremities ; Female ; HIV Infections ; HIV-1 ; Humans ; Lung ; Lymphopenia* ; Pneumonia, Pneumocystis ; Sarcoma, Kaposi* ; Scalp ; Serologic Tests ; T-Lymphocyte Subsets ; T-Lymphocytes ; T-Lymphocytes, Helper-Inducer ; T-Lymphocytopenia, Idiopathic CD4-Positive ; Thorax ; Toes

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.
Anemia, Hemolytic, Autoimmune* ; Centers for Disease Control and Prevention (U.S.) ; Child, Preschool ; Down Syndrome ; HIV ; HIV Infections ; Humans ; Lymphoma, Non-Hodgkin ; Male ; Opportunistic Infections ; Otitis Media ; Risk Factors ; Sarcoidosis, Pulmonary ; Sjogren's Syndrome ; T-Lymphocytes ; T-Lymphocytopenia, Idiopathic CD4-Positive*