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)
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 _________ __________.
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.
•Parenteral Drug Use
•Infected Blood Products
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
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?
•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.