Lysosomal acid lipase deficiency

Lysosomal acid lipase deficiency, abbreviated as LAL-D, is a lysosomal storage disorder that is inherited autosomal recessive.

It is also known as Wolman disease.

General

• Diagnosis clinical: dried blood spot testing.

Gross

• Hepatomegaly.

Microscopic

Features:

• Microvesicular or mixed steatosis (microvesicular and macrovesicular).

Note:

• Usually microvesicular predominant.

DDx:

• Microvesicular steatosis.

See also

• Medical liver disease.
• Lysosomal storage disease.