Unit 11 - Storage Disease

The spleen is the organ most involved with __________.

Amyloidosis

What is typically the size of the spleen with Amyloidosis?

• Normal or Enlarged

  • Depending on the amount and distribution of amyloid

With Amyloidosis, what are the 2 types of involvement?

• Nodular

• Diffuse

In the nodular type, where is amyloid found?

• Walls of arteries

• Follicles

  • NOT IN RED PULP

In the diffuse type, where is amyloid found?

• Red pulp is involved

  • NOT IN FOLLICLES

In the diffuse type, the spleen is usually ______ & ______.

Enlarged & firm

Gaucher’s Disease is a…

Rare familial disorder

• Reticulum cell hyperplasia

What age groups are affected by Gaucher’s Disease?

• All age groups

  • 50% are under 8 years old

The clinical features of Gaucher’s Disease follow a ______ course.

Chronic

Clinical findings of Gaucher’s Disease?

• Bone pain

• Skin pigmentation changes

What is the sonographic appearance of Gaucher’s Disease?

• Splenomegaly

• Diffuse inhomogeneity

• Multiple hypoechoic splenic nodules

What is this image showing?

Gaucher’s Disease

What is this image showing?

Gaucher’s Disease

Niemann-Pick Disease is a…

Rapidly progressing fatal disease (A, B, C)

What group of people are most affected by Niemann-Pick Disease?

Female infants (Jewish)

What are the clinical features of Niemann-Pick Disease?

• Splenomegaly

• Hepatomegaly

• Digestive disturbances

• Lymphadenopathy

What is this image showing?

Sagittal CT of Niemann-Pick Disease

What is this image showing?

Cross-sectional CT of Niemann-Pick Disease