AL amyloidosis

A plasma cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains usually produced by a plasma cell tumor. It usually presents as primary systemic amyloidosis (PSA) with multiple organ involvement and less frequently as primary localized amyloidosis (PLA) restricted to a single organ.

Hepatosplenomegaly

Simultaneous enlargement of the liver and spleen.


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PMID (PMCID)
6830397
 MALE Middle Aged
Resolution of factor X deficiency in primary amyloidosis following splenectomy.
Rosenstein ED, Itzkowitz SH, Penziner AS, Cohen JI, Mornaghi RA.
Arch Intern Med. 1983;143(3):597-9.
A 57-year-old man with primary amyloidosis was initially seen with hematuria, cutaneous bleeding, and hepatosplenomegaly.