At 5 months of age, Christina Kitchenman was admitted to the hospital with a fever and a severe non-productive cough that was subsequently determined by immunofluorescence staining to be pneumocystosis, a form of pneumonia caused by an opportunistic yeast-like fungus. Her CD4 T-cell count (220 μl –1) was much lower than expected, at only one-third of her CD8 T-cell count (650 μl –1). Her B-cell count was slightly higher than normal. An immunodeficiency was suspected, so T-lymphocyte functionality tests were carried out, proving that Christina’s T cells did not respond to a specific antigen stimulus involving tetanus toxoid as a recall antigen, even though she had received routine vaccination for tetanus (DPT vaccine) several months previously. Normal T-cell proliferation responses, however, were detected upon exposure of peripheral blood mononuclear cells to either the plant lectin mitogen phytohemagglutinin or to allogeneic B lymphocytes. Further tests revealed hypogammaglobulinemia, a deficiency of all MHC class II isotypes on her white blood cells, but no deficiency of MHC class I isotypes. Christina was successfully treated with pentamidine and received a bone marrow transplant without complication after being diagnosed with bare lymphocyte syndrome, type II (BLSII). A genetic defect in which of the following would support this diagnosis? a. HLA-DQ b. CIITA (MHC class II transactivator) c. RAG-1 d. TAP-1 e. CD3ε.

Principles Of Pharmacology Med Assist

6th Edition

ISBN:9781337512442

Author:RICE

Publisher:RICE

Chapter14: Allergy: An Overview

Section: Chapter Questions

Problem 23RQ

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At 5 months of age, Christina Kitchenman was admitted to the hospital with a fever and a severe non-productive cough that was subsequently determined by immunofluorescence staining to be pneumocystosis, a form of pneumonia caused by an opportunistic yeast-like fungus. Her CD4 T-cell count (220 μl –1) was much lower than expected, at only one-third of her CD8 T-cell count (650 μl –1). Her B-cell count was slightly higher than normal. An immunodeficiency was suspected, so T-lymphocyte functionality tests were carried out, proving that Christina’s T cells did not respond to a specific antigen stimulus involving tetanus toxoid as a recall antigen, even though she had received routine vaccination for tetanus (DPT vaccine) several months previously. Normal T-cell proliferation responses, however, were detected upon exposure of peripheral blood mononuclear cells to either the plant lectin mitogen phytohemagglutinin or to allogeneic B lymphocytes. Further tests revealed hypogammaglobulinemia, a deficiency of all MHC class II isotypes on her white blood cells, but no deficiency of MHC class I isotypes. Christina was successfully treated with pentamidine and received a bone marrow transplant without complication after being diagnosed with bare lymphocyte syndrome, type II (BLSII). A genetic defect in which of the following would support this diagnosis?

a. HLA-DQ

b. CIITA (MHC class II transactivator)

c. RAG-1

d. TAP-1

e. CD3ε.

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