Management of Patients with Primary Immunodeficiency Disorders

Human Immunodeficiency Virus (HIV)

• Primary target is the CD4 T lymphocytes, which are crucial for immune response.
• If left untreated, HIV progresses to Acquired Immunodeficiency Syndrome (AIDS), severely compromising the immune system.

Hematopoietic Stem Cell Transplantation (HSCT)

• A common limitation is the risk of graft-versus-host disease (GVHD), where transplanted cells attack the recipient’s body.

Gene Therapy

• Curative modality involving the use of cells as vehicles for delivering genes or gene products is known as gene therapy.

HIV Symptoms

• Important to note that some individuals may remain asymptomatic for several years, which can lead to delayed diagnosis and treatment.

Human Immune System

• Main function is to protect the body against infections and diseases by identifying and eliminating pathogens.

Primary Immunodeficiency Diseases (PIDDs)

• Approximately 330 different forms of PIDDs are estimated to affect people in the United States.

Clinical Manifestations of PIDDs

• A major clinical manifestation includes increased susceptibility to infections, which can lead to severe health complications.

Laboratory Tests for PIDDs

• Laboratory tests, such as immunoglobulin quantification, are used to identify antibody deficiencies in PIDDs.

Inflammation and Healing

• Inflammation plays a critical role in the immune system by initiating healing processes, fighting infections, and repairing tissues.

Learn about the complexity of the human immune system and the pathophysiology of Primary Immunodeficiency Disorders (PIDDs), which affect a large number of people in the United States. Gain insights into the management of patients with PIDDs.