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Deficiencies in what type of lymphocytes lead to immunogloculin deficiencies and what does this mean for the patient?
•Deficiencies in B lymphocytes lead to immunoglobulin deficiencies, which render patients susceptible to infections
Defects of T lymphocytes leads to defeciencies in what type of immunity and what does this mean for the patient?
•Defects in T lymphocytes lead to deficiencies in cell-mediated immunity, which render patients susceptible to opportunistic infections
List 4 important characteristics of x-linked agammaglobulinemia.
- •Inherited disorder where patients are unable to produce gamma globulin IgG
- •Absence of B lymphocytes and plasma cells from lymph nodes, spleen, intestine, blood and marrow.
- •Usually detected in the second year of life
- •Infants contract persistent, reoccurring pyogenic infections
What are two diagnostic lab features associatied with x-linked agammaglobulinemia and how is it usually treated?
- •Decrease in serum IgG of less than 100mg/dL
- •Undetectable amounts of IgA, IgD, IgM and IgE
- •Usually treated with infusion of gamma globulin for life
What are three disorders associated with Wiskott-Aldrich Syndrome (aka Cellular Immune Deficiency Syndrome)
- recurrent infections
Patients with Wiskott-Aldrich Syndrome are unable to mount a response to ________. It is characterized by a progressive depletion of lymphocytes from ________ and _______ ________. Disorder affects _________ and _________ __________.
- polysaccharide antigens
- thymus, lymph nodes
- lymphocytes, platelet function
Are males of females more likely to have Wiskott-Aldrich Syndrome?
The disease is x-linked; therefore, boys are more likely to have the disease, and they rarely survive beyond their first decade of life.
How is Wiskott-Aldrich Syndrome (Aquired Immunodeficiency Syndrome) diagnosed?
- •Initially based upon clinical symptoms and signs
- •Revised to include a CD4 count of less than 200/mL or 0.2 x 109/L•Definition now given in stages based upon antigen/antibody detection
What is HIV-1?
- •RNA virus that induces a chronic cellular infection by converting their RNA genome into a DNA provirus that is integrated into the genome of the infected cell (T-Helper Cell)
- Infection is characterized by long periods of clinical latency followed by gradual onset of disease related symptoms
List 4 ways that HIV can be transmitted.
- •Sexual Transmission
- •Parenteral Drug Use
- •Infected Blood Products
- •Mother-to-Child Transmission
How is HIV detected in the lab?
- •PCR during acute phase of infection
- •Western blot analysis of antibodies against HIV viral proteins from patient serum
- •Unique pattern of proteins detected will include bands for gp160, gp120, p66, p54, p51, gp41, p31, p24, p17
- •Lag time of 2-6 months from presence of antigen to serum antibody formation (sero-conversion)
HIV has a high-affinity for binding with what type of surface membrane protein? What types of cells express the receptor molecule for this protein?
- •High-affinity binding for CD4+ surface membrane protein
- •T-helper cells, monocytes, langerhans’ cells, follicular dendritic cells, megakaryocytes, and thymic cells express the CD4 receptor molecule
Describe 3 imortant points of the pathogenesis of HIV.
- •Depletion of CD+ T-Cells
- •Defects in B-Cell Immunity–Increase risk of autoimmune phenomenon, esp. against hematopoietic cells
- •Defects in Natural Killer Cells
What are some lab features of associated with the disease progression of HIV?
- •Increased viral load
- •Quantitation of plasma CD 4+ cells
- •Non-specific markers
- • Beta-microglobulin
- •CD4/CD8 Ratio
- CD4 count progressively decreases over time
A CD4+ count of ___________ is diagnositc of HIV.
Name 5 hematologic abnormalities associated with HIV.
What percentage of patients with HIV will present with anemia? The anemia is due to what 3 factors?
- 1. decreased rbc production
- 2. ineffective rbc production
- 3. increased rbc destruction
Describe the decreased RBC production of a patient with HIV.
- •Neoplasm infiltration of Bone Marrow
- •Abnormal Growth of BFU-Erythroid
- •Anemia of Chronic Disease
- •Blunted EPO response
- •IDA secondary to blood loss
Describe the ineffective rbc production of a HIV patient.
- •Folic Acid Deficiency due to malabsorption
- •B12 Deficiency due to malabsorption of jejunum
- •Gastric pathology with decreased production of intrinsic factor
What are 4 causes of increased RBC destruction that occurs in a patient with HIV?
- •Hemophagocytic Syndrome
- •HIV Drugs enhance G6PD Deficiency
Neutropenia is seen in ______ of HIV patients. It includes a decrease in ______ and decreased levels of _______. Defective qualitative function of ________ in neutrophils and macrophages.
Thrombocytopenia is seen in _____ of HIV patients. Cross-reactive antibody against _______and _______ . Causes _________ destruction of platelets leading to a significant decrease in platelet production for untreated HIV patients. There is a direct infection of HIV into the ________.
Describe the HIV malignancies associated with HIV patients.
- •Seen in 40% of HIV Patients
- •Karposis Sarcoma
- •High-grade B-Cell lymphoma
- •Chronic use of immunosuppressive drugs
- •Disregulated expression of cytokines