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Generalized crystal-storing histiocytosis with noncirrhotic portal hypertension: an autopsy case report
Yasunao Numata1, Shigeru Sasaki2, Kazufumi Magara3
1Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan.
Insights
Crystal-storing histiocytosis (CSH) is a rare disorder causing fibrosis and portal hypertension (PH) without cirrhosis. This case highlights CSH as a potential underlying cause of noncirrhotic PH.
Area of Science:
- Pathology
- Gastroenterology
- Rare Diseases
Background:
- Crystal-storing histiocytosis (CSH) is a rare disorder characterized by histiocyte accumulation of crystalline material.
- Portal hypertension (PH) typically presents in patients with cirrhosis, but noncirrhotic causes are less understood.
Observation:
- A patient with Tolosa-Hunt syndrome and idiopathic retroperitoneal fibrosis developed progressive, unexplained portal hypertension.
- Autopsy revealed retroperitoneal fibrosis with extensive hepatic vein and porta hepatis involvement.
- Histology confirmed crystal-storing histiocytosis in retroperitoneal tissues and nodular regenerative hyperplasia in the liver.
Findings:
- The retroperitoneal fibrosis due to CSH was identified as the cause of portal hypertension.
- Nodular regenerative hyperplasia, potentially exacerbated by gastric varices treatment, may have contributed to worsening PH.
- Cirrhosis was absent, underscoring a noncirrhotic etiology for the observed PH.
Implications:
- Crystal-storing histiocytosis should be considered in the differential diagnosis of noncirrhotic portal hypertension.
- This case expands the understanding of CSH's systemic manifestations and its potential to cause significant fibrotic complications.
- Further research is warranted to elucidate the mechanisms linking CSH to fibrotic diseases and portal hypertension.
Abstract:
Crystal-storing histiocytosis (CSH) is a rare disease associated with the accumulation of histiocytes containing crystalline matter within their cytoplasm. Herein, we present the case of a female patient who was diagnosed with Tolosa-Hunt syndrome at 45 years of age and idiopathic retroperitoneal fibrosis when she was 48 years. She developed portal hypertension (PH), but did not present with cirrhosis; as such, the cause of PH was not identified. Her PH gradually worsened when she was 54 years, and at the age of 60 years, she died from an acute subdural hematoma. Autopsy revealed retroperitoneal fibrosis with severe fibrosis extending around the hepatic veins and into the porta hepatis. Histologically, the retroperitoneal tissue showed a dense infiltrate of eosinophilic histiocytes with crystal structures in the cytoplasm, which was pathologically diagnosed as CSH. Nodular regenerative hyperplasia was observed in the liver parenchyma, whereas cirrhosis was not. In the present case, CSH caused fibrosis, which was believed to be the cause of PH. In addition, we considered that nodular regenerative hyperplasia caused by the altered hepatic blood flow due to treatment of gastric varices contributed to worsening PH. Hence, CSH should be considered as an underlying disease in noncirrhotic portal hypertension.
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