Disorders of spleen and thymus

Question 1

Appearance of lymphoid follicles and germinal centers composed of B cells within the thymus

Answer 1

Thymic follicular hyperplasia

Question 2

Inheritance of primary hemophagocytic lymphohistiocytosis

Answer 2

Autosomal recessive

Question 3

Mutation in primary hemophagocytic lymphohistiocytosis

Answer 3

Loss of function mutations in FHL genes that code for proteins used by CTLs and NKs

Question 4

Cause of secondary hemophagocytic lymphohistiocytosis

Answer 4

Inappropriate host response to infection, malignancy, or autoimmune disease

Question 5

Possible outcome of untreated hemophagocytic lymphohistiocytosis

Answer 5

Progress rapidly to multiorgan failure, shock, and death

Question 6

Cytopenia affecting at least 2 of 3 lineages, hemophagocytosis, high serum ferritin, soluble IL-2 receptor

Answer 6

Hemophagocytic lymphohistiocytosis