Abstract

Introduction: No-cirrhotic portal hypertension (NCPH) is a portal hypertension in the absence of cirrhosis, typically divided into prehepatic, intrahepatic, and post hepatic causes. Of these, nodular regenerative hyperplasia (NRH) is estimated to be responsible for 20% of NCPH in the western world, making it the most common cause of NCPH. NRH is characterized by benign transformation of the hepatic parenchyma into small regenerative nodules. Vascular injury is thought to be the inciting event leading to development of NRH. Many thrombophilic disorders have been associated with the development of NRH, including autoimmune, myeloproliferative, and lymphoproliferative diseases. No cases of NRH due solely to intravenous drug use without infectious complications have been reported. In this case report, we detail the presentation of a young female with NCPH due to NRH, likely secondary to IV heroine use. Case: A 38-year-old female with a pmhx of pancytopenia, splenomegaly, and IV heroin use presented for GI evaluation after an ER visit notable for a Hgb of 6.6 g/dL requiring transfusion prompted further evaluation. She underwent extensive hematologic work-up, including a negative bone marrow biopsy and exclusion of infectious, malignant, and cytogenetic diseases. However, CT abdomen and pelvis noted a spleen measuring 22.4 cm without evidence of cirrhosis. In the office, the patient was without complaints. She disclosed a history of rash following use of bactrim for a UTI, but denied use of prescription or herbal medications. Labs revealed a platelet count of 56,000/ÎĽL and iron deficiency. Upper and lower endoscopy revealed several columns of large esophageal varices and medium rectal varices. A transjugular liver biopsy with hepatic venogram showed patent vasculature, with histologic findings on liver biopsy consistent with nodular regenerative hyperplasia. Additionally, macrophages with granular material were noted, raising the possibility of infection vs. reactive macrophages to foreign material deposition. As the histological findings could not be attributed to an infectious process, it was felt that foreign material deposition from IVDU was responsible. Discussion: NRH is a form of NCPH, typically affecting patients over the age of 60. The diagnosis of NRH requires histological confirmation by liver biopsy. In this case, a young female with prior IVDU presented with pancytopenia and splenomegaly. Portal hypertension was confirmed endoscopically by the presence of varices, and liver histology was consistent with NRH. Given the findings on liver biopsy and negative infectious work-up, we propose that intravenous use of tainted heroin caused hepatic microvascular injury and subsequent development of NRH.

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