Primary/Congenital WBC Disorders Flashcards
(12 cards)
What is Chediak-Higashi Syndrome?
PrimaryPrimary immunodeficiency with recurrent neutropenia and neutrophil function defects along with platelet function defects
Neutrophils, eosinophils and other cells contain abnormal fused granules
Which breed is most likely to have Chediak-Higashi Syndrome?
Persian cats
One disorder is partial oculocutaneous albinism –> manifests in Persian cats as a blue smoke coat color
What is Pelger-Huet Anomaly?
Causes defective terminal granulocyte maturation (“laminopathy”)
Granulocytes have hyposegmented nuclei with mature chromatin
What breed is overrepresented for Pelger-Huet anomaly?
Australian Shepherds
What is Leukocyte Adhesion Deficiency?
Mutations in leukocyte adhesion proteins and prevent the normal adherence and migration of WBC through luminal endothelium
What breed is associated with Leukocyte Adhesion Deficiency?
Irish Setters
Type 1: defect in the beta-2 subunit (CD18) of the heterodimeric integrins
What is trapped neutrophil syndrome?
Characterized by peripheral neutropenia with a degenerative left shift and marked monocytosis and myeloid hyperplasia in the bone marrow with increased mature neutrophils
What breed is known to have trapped neutrophil syndrome?
Border Collie
What is X-linked Severe Combined Immunodeficiency?
Caused by mutations in gene encoding the common IL-2 receptor gamma chain
Affects developing CD4-CD8-thymocytes
What breeds are associated with X-linked severe combined immunodeficiency?
Cardigan Welsh Corgi and Basset Hounds
What is Autosomal Recessive severe combined immunodeficiency?
Characterized by defective recombination events during T and B lymphocyte maturation
Affected dogs have:
- Severe lymphopenia
- Decreased globulin concentration (due to agammaglobulinemia)
- Marked lymphoid hypoplasia in the spleen, thymus and other lymphoid tissues
What breed mainly gets autosomal recessive severe combined immunodeficiency?
Jack Russel Terrier
