Idiopathic portal hypertension in a patient with mixed connective tissue disease and protein C deficiency.
We report a 29-year-old woman with a 2.5 year history of mixed connective tissue disease (MCTD) who developed idiopathic portal hypertension (IPH) and thrombocytopenia as a result of hypersplenism. She had recurrent esophagogastric variceal rupture. Hematological examination also revealed low levels of protein C activity. The liver biopsy specimen showed non-specific mild inflammation and no thrombi. However, portal vein thrombosis developed after splenectomy. This was a rare case of severe complications of IPH accompanying MCTD and protein C deficiency.
Horai
Yoshiro
Y
Department of Internal Medicine and Rheumatology, Clinical Research Center, National Hospital Organization Kyushu Medical Center, Fukuoka. yoshirohorai0518@qmed.hosp.go.jp
Miyamura
Tomoya
T
Hirata
Akie
A
Nakamura
Masataka
M
Takahama
Soichiro
S
Ando
Hitoshi
H
Minami
Rumi
R
Yamamoto
Masahiro
M
Suematsu
Eiichi
E
D000328:Adult; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000175:diagnosis; D008103:Liver Cirrhosis / Q000150:complications / Q000175:diagnosis; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000175:diagnosis*; D010198:Pancytopenia / Q000150:complications / Q000175:diagnosis; D020151:Protein C Deficiency / Q000150:complications* / Q000175:diagnosis*; D013163:Splenomegaly / Q000150:complications / Q000175:diagnosis; D000094724:Idiopathic Noncirrhotic Portal Hypertension