|IJCEP Copyright © 2007-All rights reserved.
Int J Clin Exp Pathol 2013;6(11):2636-2639
Dubin-Johnson syndrome with multiple liver cavernous hemangiomas: report of a
Peifeng Li, Yingmei Wang, Jinmei Zhang, Ming Geng, Zengshan Li
Department of Pathology, General Hospital of Jinan Military Command, Jinan, Shandong Province 250031, China; Department of Pathology,
State Key Laboratory of Cancer Biology, Fourth Military Medical University, Xi’an 710032, China; Department of Hepatobiliary Surgery, Weifang
People’s Hospital, Weifang, China. Equal contributors.
Received August 2, 2013; Accepted August 27, 2013; Epub October 15, 2013; Published November 1, 2013
Abstract: Dubin-Johnson syndrome (DJS) is a rare autosomal recessive inheritance disorder of bilirubin metabolism. Herein we reported a
complicated but interesting case which is readily resulted in misdiagnosis or an indefinite diagnosis, and this is the first reported familial case
of DJS with multiple liver cavernous hemangiomas. A 49-year-old man was referred to our hospital for jaundice and multiple low-density liver
masses. Extensive laboratory investigations showed conjugated hyperbilirubinaemia and positive urine bilirubin. Microscopically, lesions were
composed of blood-filled vascular channels of various sizes lined by a single layer of flat endothelial cells supported by fibrous tissue. Coarse
brown granules presented in the hepatocytes of the liver lobules locating beside the tumor, particularly in the centrilobular hepatocytes, and the
granules showed blue-green with Schmorl’s reaction lipofuscin staining. Interestingly, one of the patient’s six siblings (female) shared the
same condition with him. The relationship between DJS and hemangiomas remains unclear, and it might be contributed to some hereditary
factors, or probably occurred simultaneously by chance. It was certified that the true reason for the long-term unclear jaundice was DJS, which
was presumed clinically to be caused by bile excretion obstacles associated with the hemangiomas. Liver biopsy and histochemical stain may
be helpful to identify the reason of jaundice and avoid misdiagnosis or an indefinite diagnosis. (IJCEP1308004).
Keywords: Dubin-Johnson syndrome, cavernous hemangiomas, hyperbilirubinemia, jaundice
Address correspondence to: Ming Geng, Department of Pathology, General Hospital of Jinan Military Command, Jinan, Shandong Province
250031, China. E-mail: email@example.com; Zengshan Li, Department of Pathology, State Key Laboratory of Cancer Biology, Fourth Military
Medical University, Xi’an 710032, China. E-mail: firstname.lastname@example.org