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Ch. 17 - Adaptive Immunity: Specific Defenses of the Host
Chapter 13, Problem 17.10a

Patients with Chédiak-Higashi syndrome suffer from various types of cancer. These patients are most likely lacking which of the following?
a. Treg cells
b. TH1 cells
c. B cells
d. NK cells
e. TH2 cells

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1
Understand the context of Chédiak-Higashi syndrome, which is a rare genetic disorder that affects the immune system.
Recognize that patients with this syndrome have a defect in lysosomal trafficking, leading to impaired function of certain immune cells.
Consider the role of each cell type listed in the options: Treg cells, TH1 cells, B cells, NK cells, and TH2 cells.
Focus on NK cells (Natural Killer cells), which are crucial for targeting and destroying cancerous and virus-infected cells.
Conclude that a deficiency in NK cells would lead to an increased susceptibility to cancer, which aligns with the symptoms of Chédiak-Higashi syndrome.

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Key Concepts

Here are the essential concepts you must grasp in order to answer the question correctly.

Chédiak-Higashi Syndrome

Chédiak-Higashi syndrome is a rare genetic disorder characterized by immunodeficiency, partial oculocutaneous albinism, and neurological problems. It results from mutations in the LYST gene, which affects lysosomal trafficking in cells. This syndrome leads to an increased susceptibility to infections and various hematological malignancies, making understanding its immunological implications crucial for addressing the question.
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Natural Killer (NK) Cells

Natural Killer (NK) cells are a type of lymphocyte in the immune system that play a critical role in the body's defense against tumors and virally infected cells. They are part of the innate immune response and can recognize and destroy compromised cells without prior sensitization. In Chédiak-Higashi syndrome, the dysfunction of NK cells contributes to the increased risk of cancer and infections, making them a key focus in understanding the syndrome's immunological deficiencies.
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T Helper Cells (TH1 and TH2)

T Helper cells, including TH1 and TH2 subsets, are essential for orchestrating the adaptive immune response. TH1 cells are primarily involved in cell-mediated immunity, promoting responses against intracellular pathogens, while TH2 cells help in humoral immunity, facilitating antibody production. The balance and function of these cells are crucial in immune responses, and their dysregulation can lead to increased susceptibility to infections and malignancies, relevant to the context of Chédiak-Higashi syndrome.
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