1. Sarcoidosis-lymphoma syndrome with portal hypertension: diagnostic clues and approach
- Author
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Kai Mizobuchi, Tadashi Yoshino, Kazumasa Nanjo, Nobuyuki Tanida, Miki Mizobuchi, Hiromichi Yamai, Kenji Yorita, Satoshi Ito, Niranjan Sharma, Shigeto Shimizu, Koji Ueta, Kazuhisa Onishi, Hisashi Matsuoka, Fumio Chikamori, Jun Iwabu, Sawaka Yukishige, and Norihiro Hokimoto
- Subjects
Pathology ,medicine.medical_specialty ,Liver tumor ,PET/CT ,medicine.medical_treatment ,Splenectomy ,R895-920 ,Hepatosplenomegaly ,Case Report ,030218 nuclear medicine & medical imaging ,Medical physics. Medical radiology. Nuclear medicine ,03 medical and health sciences ,0302 clinical medicine ,hemic and lymphatic diseases ,Biopsy ,medicine ,Laparoscopic liver biopsy ,Radiology, Nuclear Medicine and imaging ,Sarcoidosis-lymphoma syndrome ,Portal hypertension ,PET-CT ,medicine.diagnostic_test ,business.industry ,medicine.disease ,Lymphoma ,Primary hepatic Hodgkin lymphoma ,Hepatic sarcoidosis ,Sarcoidosis ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
Sarcoidosis-lymphoma syndrome associated with portal hypertension is very rare. A 68-year-old female presented with a 5 kg weight loss in 6 months. Soluble interleukin-2 receptor activity was increased and total platelet count was decreased. Contrast-enhanced computed tomography showed the presence of hepatosplenomegaly and a 3 cm-sized tumor in segment 3 of the liver. The hepatic venous catheterization showed mild portal hypertension. On fluorodeoxyglucose-positron emission tomography/computed tomography, progressive malignant lymphoma was suspected. However, bone marrow biopsy showed multiple noncaseating granulomas. A laparoscopic liver biopsy revealed that the liver tumor had features of Hodgkin lymphoma. There were multiple noncaseating epithelioid granulomas in the portal tracts of the liver. Splenectomy for splenomegaly and partial hepatectomy for the liver tumor were performed. Pathological examination of the resected specimens revealed multiple noncaseating epithelioid granulomas in the liver and spleen. Histopathology of th e liver tumor confirmed classic Hodgkin lymphoma with mixed cellularity. We conclude that hepatic venous catheterization, positron emission tomography/computed tomography, and pathological examinations of bone marrow, liver, and spleen are crucial for the diagnosis of sarcoidosis-lymphoma syndrome associated with portal hypertension.
- Published
- 2021