TY - JOUR
T1 - Sarcoidosis-lymphoma syndrome with portal hypertension
T2 - diagnostic clues and approach
AU - Chikamori, Fumio
AU - Yorita, Kenji
AU - Yoshino, Tadashi
AU - Ito, Satoshi
AU - Mizobuchi, Miki
AU - Ueta, Koji
AU - Mizobuchi, Kai
AU - Shimizu, Shigeto
AU - Nanjo, Kazumasa
AU - Yukishige, Sawaka
AU - Iwabu, Jun
AU - Matsuoka, Hisashi
AU - Hokimoto, Norihiro
AU - Yamai, Hiromichi
AU - Onishi, Kazuhisa
AU - Tanida, Nobuyuki
AU - Sharma, Niranjan
N1 - Publisher Copyright:
© 2021
PY - 2021/8
Y1 - 2021/8
N2 - 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 the 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.
AB - 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 the 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.
KW - Hepatic sarcoidosis
KW - Laparoscopic liver biopsy
KW - PET/CT
KW - Portal hypertension
KW - Primary hepatic Hodgkin lymphoma
KW - Sarcoidosis-lymphoma syndrome
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U2 - 10.1016/j.radcr.2021.05.045
DO - 10.1016/j.radcr.2021.05.045
M3 - Article
AN - SCOPUS:85107720630
VL - 16
SP - 2192
EP - 2201
JO - Radiology Case Reports
JF - Radiology Case Reports
SN - 1930-0433
IS - 8
ER -