Ignite Creation Date:
2024-05-05 @ 9:20 PM
Last Modification Date:
2024-10-26 @ 10:03 AM
Study NCT ID:
NCT00867269
Status:
RECRUITING
Last Update Posted:
2024-07-12
First Post:
2009-03-20
Brief Title:
Etiology Pathogenesis and Natural History of Idiopathic CD4 Lymphocytopenia
Sponsor:
National Institute of Allergy and Infectious Diseases NIAID
Organization:
National Institutes of Health Clinical Center CC
Study Overview
Official Title:
Etiology Pathogenesis and Natural History of Idiopathic CD4 Lymphocytopenia
Status:
RECRUITING
Status Verified Date:
2024-10-01
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
Background
Idiopathic CD4 lymphocytopenia ICL is a condition in which there is a decreased level of CD4 lymphocytes a type of white blood cell which can lead to opportunistic infections or autoimmune disorders and diseases
Objectives
To characterize the natural history with regard to CD4 T cell count and onset of infection malignancy and autoimmunity
To describe the immunological status of patients affected by ICL while providing the best possible standard therapy to eradicate opportunistic infections
To establish the timeline of CD4 lymphocytopenia with particular focus on defining subgroups of patients according to the decline stabilization or rise of CD4 T cell counts over time
To characterize the opportunistic infections that occur in ICL patients at microbiologic and molecular levels
To characterize the immunophenotype and possible genetic immunodeficiency causes of ICL
To determine whether measurable immunologic parameters correlate with the development of opportunistic infections or other comorbidities such as lymphoma in patients with ICL
To determine whether there is any association between ICL and autoimmunity
To determine CD4 T cell turnover survival functionality and cytokine responsiveness in ICL patients
Eligibility
Patients 2 years of age and older with an absolute CD4 count less than 300 in children 6 years or older and adults or less than 20 of T cells in children younger than 6 on two occasions at least 6 weeks apart
Patients with negative results of HIV testing by ELISA Western Blot and viral load
Patients must not have underlying immunodeficiency conditions be receiving cytotoxic chemotherapy anti-cancer drugs that kill cells or have cancer
Design
At the initial visit to the National Institutes of Health the following evaluations will be conducted
Personal and family medical histories
Physical examination including rheumatology evaluation and other consultations as medically indicated eg dermatology pulmonology ophthalmology imaging studies
Blood samples for analysis of red and white blood cell counts liver function immune hormones and antibody and autoantibody levels white blood cell growth and function and DNA
Urinalysis and urine pregnancy testing for female patients of childbearing age
Evaluation and treatment of active infections as medically indicated including biopsies buccal swabs pulmonary function tests and imaging studies
Follow-up visits will take place approximately every 12 months or more frequently if indicated and will continue for a minimum of 4 years and a maximum of 10 years
Evaluations at follow-up will include blood samples ie CBC with differential biochemical profile HIV testing etc and urinalysis and rheumatology consults
Idiopathic CD4 lymphocytopenia ICL is a condition in which there is a decreased level of CD4 lymphocytes a type of white blood cell which can lead to opportunistic infections or autoimmune disorders and diseases
Objectives
To characterize the natural history with regard to CD4 T cell count and onset of infection malignancy and autoimmunity
To describe the immunological status of patients affected by ICL while providing the best possible standard therapy to eradicate opportunistic infections
To establish the timeline of CD4 lymphocytopenia with particular focus on defining subgroups of patients according to the decline stabilization or rise of CD4 T cell counts over time
To characterize the opportunistic infections that occur in ICL patients at microbiologic and molecular levels
To characterize the immunophenotype and possible genetic immunodeficiency causes of ICL
To determine whether measurable immunologic parameters correlate with the development of opportunistic infections or other comorbidities such as lymphoma in patients with ICL
To determine whether there is any association between ICL and autoimmunity
To determine CD4 T cell turnover survival functionality and cytokine responsiveness in ICL patients
Eligibility
Patients 2 years of age and older with an absolute CD4 count less than 300 in children 6 years or older and adults or less than 20 of T cells in children younger than 6 on two occasions at least 6 weeks apart
Patients with negative results of HIV testing by ELISA Western Blot and viral load
Patients must not have underlying immunodeficiency conditions be receiving cytotoxic chemotherapy anti-cancer drugs that kill cells or have cancer
Design
At the initial visit to the National Institutes of Health the following evaluations will be conducted
Personal and family medical histories
Physical examination including rheumatology evaluation and other consultations as medically indicated eg dermatology pulmonology ophthalmology imaging studies
Blood samples for analysis of red and white blood cell counts liver function immune hormones and antibody and autoantibody levels white blood cell growth and function and DNA
Urinalysis and urine pregnancy testing for female patients of childbearing age
Evaluation and treatment of active infections as medically indicated including biopsies buccal swabs pulmonary function tests and imaging studies
Follow-up visits will take place approximately every 12 months or more frequently if indicated and will continue for a minimum of 4 years and a maximum of 10 years
Evaluations at follow-up will include blood samples ie CBC with differential biochemical profile HIV testing etc and urinalysis and rheumatology consults
Detailed Description:
Idiopathic CD4 lymphocytopenia ICL is a disorder characterized by decreased numbers of circulating CD4 T lymphocytes in the absence of known causes of CD4 lymphocytopenia ICL is defined as an absolute CD4 T cell count of less than 300 cellsmicroL in a patient with no human immunodeficiency virus infection or known immunodeficiency syndrome The causes and frequency of the disorder remain unknown The condition is typically diagnosed when patients present with a serious infection In this natural history protocol we will evaluate patients with CD4 T cell counts below 300 cellsmicroL We propose to follow 300 ICL patients for a minimum of 4 and maximum of 20 years with a particular focus on the association between ICL and autoimmune disease In addition to the ICL patients we will enroll blood relatives and household contacts to better understand pathogenesis and etiologies of the syndrome We will collect blood and other tissues for immunologic rheumatologic and genetic testing in an effort to identify and understand the underlying defects that cause ICL and follow its course in a cohort of patients who will receive best standard therapy for opportunistic infections
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None
Secondary IDs
| Secondary ID | Type | Domain | Link |
|---|---|---|---|
| 09-I-0102 | None | None | None |