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Case Reports
. 2020 Jun 17:8:2050313X20931996.
doi: 10.1177/2050313X20931996. eCollection 2020.

A rare case of peliosis hepatis in primary immune deficiency

Evelyn Angulo  1 , Sydney Joyner  2 , Nasma K Majeed  3 , Sharmilee Nyenhuis  4
Affiliations
Case Reports

A rare case of peliosis hepatis in primary immune deficiency

Evelyn Angulo et al. SAGE Open Med Case Rep. .

Abstract

Peliosis hepatis is a rare condition characterized by blackish-blue blood-filled cavities in hepatic parenchyma caused by dilatation of hepatic sinusoids. Peliosis hepatis has been described in secondary immunodeficiencies and certain medications. We present the first case of peliosis hepatis in a patient with a primary immunodeficiency, common variable immunodeficiency. A 44-year-old African-American male presented with gastrointestinal bleeding and elevated liver function tests. His medical history included common variable immunodeficiency and chronic kidney disease. The patient had jaundice, regenerative nodules on liver pathology, and low immunoglobulin levels. A magnetic resonance imaging of the abdomen with contrast revealed a cirrhotic liver, a 5 × 3 cm lesion, and poorly defined nodules which had decreased enhancement. A computed tomography-guided liver biopsy revealed peliosis hepatis, focal nodular hyperplasia, and fibrosis. No other etiology of his liver disease was found. The etiology of peliosis hepatis in patients with primary immunodeficiencies remains unclear. Additional studies are needed to understand the underlying mechanisms.

Keywords: Peliosis hepatis; common variable immunodeficiency; focal nodular hyperplasia.

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Conflict of interest statement

Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
MRI abdomen: 5 × 3 cm liver lesion and numerous poorly defined nodules.
Figure 2.
Figure 2.
Sinusoidal dilatation.
Figure 3.
Figure 3.
Perisinusoidal fibrosis.
See this image and copyright information in PMC

References

    1. Samyn M, Hadzic N, Davenport M, et al. Peliosis hepatis in childhood: case report and review of the literature. J Pediatr Gastroenterol Nutr 2004; 39(4): 431–434. - PubMed
    1. Tsokos M, Erbersdobler A. Pathology of peliosis. Forensic Sci Int 2005; 149: 25–33. - PubMed
    1. Scoazec J, Marche C, Girard P, et al. Peliosis hepatis and sinusoidal dilation during infection by the human immunodeficiency virus (HIV): an ultrastructural study. Am J Pathol 1988; 131(1): 38–47. - PMC - PubMed
    1. Tzirogiannis K, Papadimas G, Kondyli V, et al. Peliosis hepatis: microscopic and macroscopic type, time pattern, and correlation with liver cell apoptosis in a model of toxic liver injury. Dig Dis Sci 2006; 51: 1998–2006. - PubMed
    1. Crocetti D, Palmieri A, Pedulla G, et al. Peliosis hepatis: personal experience and literature review. World J Gastroenterol 2015; 21(46): 13188–13194. - PMC - PubMed

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