Globular hepatic amyloid is highly sensitive and specific for LECT2 amyloidosis

Vishal S. Chandan, Sejal S. Shah, Dora M. Lam-Himlin, Giovanni De Petris, Oana M. Mereuta, Ahmet M Dogan, Michael S. Torbenson, Tsung Teh Wu

Research output: Contribution to journalArticle

15 Scopus citations

Abstract

Globular hepatic amyloid (GHA) is rare, and its clinical significance remains unclear. Recently, leukocyte chemotactic factor-associated amyloidosis (ALECT2) has been reported to involve the liver, showing a globular pattern. We reviewed 70 consecutive cases of hepatic amyloidosis to determine the prevalence and morphology of hepatic amyloid subtypes, especially ALECT2 and its association with GHA. Each case was reviewed for amyloid subtype (immunohistochemistry and/or mass spectrometry), its pattern (linear or globular), and distribution (vascular, perisinusoidal, or stromal). In addition, 24 cases of confirmed hepatic ALECT2 on mass spectrometry from our consultation files were also reviewed. LECT2 immunostaining was performed in 49 cases. Of the 70 cases, immunoglobulin light chain (AL) type was most common with 41 cases (59%), followed by transthyretin (ATTR) 15 cases (22%), 3 cases each of fibrinogen A (AFib) (4%), serum amyloid A (AA) (4%), and ALECT2 (4%), 2 cases of apolipoproteins (AApoA1) (3%), and 3 cases (4%) were unclassified. Three of our 70 cases (4%), with ALECT2, and all 24 cases (100%) of mass spectrometry- confirmed hepatic ALECT2 showed only GHA deposits in the hepatic sinusoids and portal tracts. Three (4%) other cases of AL type showed a focal globular pattern admixed with prominent linear amyloid. None of the other amyloid subtypes showed GHA. LECT2 immunostain was positive in all 27 cases (100%) of ALECT2 and negative in the other 22 non-ALECT2 cases (100%) (14 AL, 5 ATTR, 1 AA, 1 AFib, 1 AApoA1). Pure GHA is uncommon (4%) but is highly specific for ALECT2, and LECT2 immunostain is helpful in confirming this amyloid type.

Original languageEnglish (US)
Pages (from-to)558-564
Number of pages7
JournalAmerican Journal of Surgical Pathology
Volume39
Issue number4
DOIs
StatePublished - Mar 30 2015

Keywords

  • ALECT2
  • amyloid
  • globular
  • hepatic

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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    Chandan, V. S., Shah, S. S., Lam-Himlin, D. M., De Petris, G., Mereuta, O. M., Dogan, A. M., Torbenson, M. S., & Wu, T. T. (2015). Globular hepatic amyloid is highly sensitive and specific for LECT2 amyloidosis. American Journal of Surgical Pathology, 39(4), 558-564. https://doi.org/10.1097/PAS.0000000000000373