sero 2.1

• deficiencies in which component of the immune system result in recurrent bacterial infections and autoimmune type manifestations

  
  

  Complement system
• What is the prevalence of selective IgA deficiency in American or European descent populations?

  1 in 500
• Which of the following autoimmune diseases is NOT commonly associate with selective IgA deficiency?

  Psoriasis
• How is selective IgA deficiency typically diagnosed if the serum IgA level is two standard deviations below the age-adjusted mean but greater than 50 mg/dL?

  Partial deficiency
• Which of the following statements about replacement IgA therapy is TRUE?

  It can induce the development of anti-IgA antibodies
• Which of the following is NOT a common manifestation of common variable imnaunodeficiency (CVI)?

  Diabetes mellitus
• How is CVI usually diagnosed?

  By demonstrating a low serum IgG level.
• Which subclass deficiency of IgG is the most common?

  IgG4
• What is the characteristic phenotype of severe combined immunodeficiency to (SCID)?

  T+B+NK-
• Which category of primary immunodeficiencies is characterized by defects in cell-mediated immunity along with nonimmunologic features?

  Category 2
• What is the main reason why defects in cellular immunity are more info challenging to manage compared to defects in humoral immunity?Absence of soluble products for administration
• Graft-versus-host disease (GVHD) can occur in patients with severe defects in cell-mediated immunity due to:

  Enhanced function of the donor lymphocytes
• Which syndrome is characterized by a quantitative defect in thymocytes resulting in decreased mature T cells, but the function of the existing T cells remains normal?

  DiGeorge syndrome
• Which disorder is caused by a mutation in the LYST gene and is characterized by hypopigmentation, reduced number of natural killer (NK) cells, and increased production of inflammatory proteins?

  Chediak-Higashi syndrome
• How is chronic granulomatous disease (CGD) primarily diagnosed?

  Flow cytometric assay using dihydrorhodamine
• Which disorder involves a deficiency in a protein called CD18 leading to abnormal adhesion, motility, and chemotaxis of leukocytes?

  Leukocyte adhesion deficiency (LAD)
• Defects in which complement components are usually associated with a lupuslike syndrome?

  C1q, C4, and C2
• Which category of primary immunodeficiencies includes disorders with an inherited genetic component as well as an acquired component such as somatic mutations or autoantibody production?

  Category 9
• Which syndrome involves a deficiency of mevalonate kinase, resulting in  periodic fever syndrome?

  Hyper IgD syndrome.
• Which of the following statements accurately describes the initial step in evaluating suspected immunodeficiency?

  Evaluation begins with patient history followed by CBC and WBC differential
• What is the purpose of screening tests in the evaluation of immunodeficiency?

  To detect abnormalities that warrant further specialized testing
• How can delayed hypersensitivity-type skin reactions aid in screening for immunodeficiency?

  By evaluating cell-mediated immune response
• What laboratory technique is used to confirm a diagnosis of leukocyte adhesion deficiency type 1?

  Flow cytometry
• What is the primary purpose of performing bone marrow biopsy in the evaluation of immunodeficiency?

  To establish the diagnosis and exclude other diseases
• Which statement accurately describes newborn screening for immunodeficiencies?

  Newborn screening typically involves detecting the presence of TRECS
• In what scenario is genetic testing particularly useful in diagnosing immunodeficiency?

  When identifying the mode of inheritance of the immunodeficiency
• A100 Which statement accurately reflects the role of laboratory results in the diagnosis of immunodeficiencies?

  Laboratory results must be integrated with the entire clinical picture
•  

  What is the gold standard test for contact dermatitis?

  Patch test
• Hypersensitivity pneumonitis is characterized by inflammation of

  The lung parenchyma
• Skin testing for tuberculosis is based on the principle of:

  T-cell-mediated memory response
• What is the main purpose of skin testing for delayed hypersensitivity?

  Evaluating cell-mediated immune responses
• A 50-year-old woman presents with joint pain, swelling, and morning stiffness in her hands and feet. She reports a family history of autoimmune diseases and has recently been diagnosed with rheumatoid arthritis. Which type of hypersensitivity reaction is most likely associated with her condition?

  Type III hypersensitivity reaction reaction
• A-28-year-old woman presents to the emergency room with sudden onset difficulty breathing, hives all over her body, and swelling of her face and lips shortly after eating seafood at a restaurant. She reports a history of similar episodes after consuming nuts and shellfish. Which type of hypersensitivity reaction is most likely responsible for her symptoms?

  Type I hypersensitivity reaction reaction
• A 35-year-old man with a history of rheumatoid arthritis presents with joint pain, swelling, and morning stiffness in his hands and feet. He also complains of dry eyes and mouth. Laboratory tests reveal the presence of rheumatoid factor and antinuclear antibodies. Which type of hypersensitivity reaction is most likely associated with his condition?

  Type III hypersensitivity reaction reaction
• A 20-year-old male presents with a diffuse rash and joint pain 2 weeks after starting a course of penicillin for a throat infection. He reports feeling generally unwell and experiencing fever and malaise. Laboratory tests reveal elevated levels of antinuclear antibodies. Which type of hypersensitivity reaction is most likely associated with his symptoms?

  Type II hypersensitivity reaction
• A 45-year-old woman with a history of systemic lupus erythematosus (SLE) presents with fever, joint pain, and a rash. Laboratory tests reveal low complement levels and the presence of immune complexes in the skin biopsy. Which type of hypersensitivity reaction is most likely associated with her presentation?Type III hypersensitivity reaction
•  A 30-year-old man presents with a localized skin rash, erythema, and edema at the site of a recent bee sting. He reports similar reactions to precious bee stings. Which type of hypersensitivity reaction is most likely associated with his condition?

  Type IV hypersensitivity reaction
•  A 40-year-old man presents with a fever, cough, and shortness of breath. He works in a dusty environment and has a history of exposure to bird droppings. Chest X-ray reveals interstitial infiltrates. Which type of hypersensitivity reaction is most likely associated with his condition?

  Type IV hypersensitivity reaction
• Which of the following best describes immunodeficiencies?

  Disorders where the immune system is missing or dysfunctional
• Which of the following is NOT a consequence of immunodeficiencies?

  Decreased risk of autoimmune diseases