PMC6172546_01
Histology on a liver biopsy at the time of admission. (A) Hematoxylin and Eosin staining shows porphyrin deposition in the liver. Porphyrin deposition was preferentially seen in the hepatocytes rather than in the bile duct. There were no findings suggestive of autoimmune hepatitis, including plasma cell infiltration and rosette formation. (B) Polarizing microscopy confirmed the marked deposition of Maltese-cross-positive porphyrin.
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There were no findings suggestive of autoimmune hepatitis, such as plasma cell infiltration or rosette formation (Fig. 1).
Clinical course before and after admission. After admission, plasma exchange was performed for a total of 5 times, no decrease in the blood protoporphyrin value was observed, and improvement of liver disorder was not observed either. Therefore, when switching to phlebotomy treatment (200 mL ~ 400 mL) once/week, the blood protoporphyrin value was markedly decreased to 2,710 mug/dL, and AST 21 U/L, ALT 16 U/L, T-bil 0.6 mg/dL and liver function also improved.
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We then used phlebotomy to remove red blood cells and plasma protoporphyrin (Fig. 2).