Immunodeficiency Diseases in Hematology

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  1. 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
  2. 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
  3. 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
  4. 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
  5. What are three disorders associated with Wiskott-Aldrich Syndrome (aka Cellular Immune Deficiency Syndrome)
    • Eczema
    • thrombocytopenia
    • recurrent infections
  6. 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
  7. 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.
  8. 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
  9. 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
  10. List 4 ways that HIV can be transmitted.
    • •Sexual Transmission
    • •Parenteral Drug Use
    • •Infected Blood Products
    • •Mother-to-Child Transmission
  11. 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)
  12. 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
  13. 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
  14. 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
  15. A CD4+ count of ___________ is diagnositc of HIV.
  16. Name 5 hematologic abnormalities associated with HIV.
    • •Anemia
    • •Neutropenia
    • •Thrombocytopenia
    •         –ITP
    • •Lymphoma
    • •Malignancies
  17. What percentage of patients with HIV will present with anemia?  The anemia is due to what 3 factors?
    • 70-80%
    • 1. decreased rbc production
    • 2. ineffective rbc production
    • 3. increased rbc destruction
  18. Describe the decreased RBC production of a patient with HIV.
    • •Neoplasm infiltration of Bone Marrow
    • •Infection
    • •Abnormal Growth of BFU-Erythroid
    • •Anemia of Chronic Disease
    • •Blunted EPO response
    • •IDA secondary to blood loss
  19. 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
  20. What are 4 causes of increased RBC destruction that occurs in a patient with HIV?
    • •Hemophagocytic Syndrome
    • •TTP
    • •DIC
    • •HIV Drugs enhance G6PD Deficiency
  21. Neutropenia is seen in ______ of HIV patients.  It includes a decrease in ______ and decreased levels of _______.  Defective qualitative function of ________ in neutrophils and macrophages.
    • 50%
    • CFU-GM
    • G-CSF
    • opsonization
  22. 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 ________.
    • 40%
    • GP160/120
    • GPIIb/IIIa
    • immune
    • megakaryocyte
  23. 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
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Immunodeficiency Diseases in Hematology
2013-02-11 16:52:47
Hematology Exam two

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