Paroxysmal Nocturnal Hemoglobinuria and Complement System

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14 Questions

What can uncontrolled complement activation lead to?

Severe pathology

In paroxysmal nocturnal hemoglobinuria (PNH), what results in a deficiency of glycosylphosphatidylinositol-linked surface proteins?

CD59 on hematopoietic stem cells

What is the consequence of CD59 deficiency on PNH red blood cells?

Intravascular hemolysis

What is the main mechanism by which eculizumab acts?

Blocking the proinflammatory effects of terminal complement activation

What has the recent approval of eculizumab validated?

The concept of complement inhibition as an effective therapy

How does eculizumab prevent C5 entry into the C5 convertase?

By binding to C5 with high affinity

What is the primary challenge Rickettsia pose to the immune system?

They infect nonphagocytic cells

What is the main function of CD59 on hematopoietic stem cells?

Inhibiting terminal complement activation

What must work in conjunction with activated macrophages to kill infected endothelial cells in the early stages of infection?

Helper T cells

In the chronological order of the phases of T cell responses, what comes after the Recognition phase?

Clonal Expansion

What specifically results in the clonal expansion of T cells?

Interleukin 2 (IL-2)

What is the first signal when an extracellular antigen encounters a T cell?

Presentation by class II MHC molecules

To where must effector cytotoxic T lymphocytes (CTLs) migrate?

Peripheral tissues

Where do the complementary determining regions (CDRs) occur on the B cell receptor (BCR)?

VH and VL regions

Learn about the rare hemolytic disease Paroxysmal Nocturnal Hemoglobinuria (PNH) and its relationship with the complement system. Understand how somatic mutations in PNH lead to a deficiency of surface proteins, including CD59, and the resulting pathology.

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