Fulminant plasmapheresis-refractory thrombotic microangiopathy associated with advanced gastric cancer.
We report a case of a 27-year-old female with thrombotic microangiopathy as an initial presentation of an unexpected disseminated gastric carcinoma. Based on clinical features and laboratory findings, thrombotic thrombocytopenic purpura (TTP) was diagnosed and plasma exchange started. However, she had responded poorly to plasmapheresis, developed multiorgan failure and died 72 h after admission. Autopsy revealed a disseminated gastric adenocarcinoma with metastatic infiltration of dura mater and disseminated tumor cell emboli in the microcirculation of the liver and lungs. Genetic analysis revealed amplification of KRAS oncogene and aberrations in DCC tumor suppressor gene, which can explain the young age and advanced disease at presentation. The role of plasmapheresis in cancer-associated TTP is uncertain. Plasmapheresis delivers fresh coagulation factors and may theoretically promote microthrombi formation and lead to worsening of the disease. Thrombotic thrombocytopenic purpura seems to be a late and prognostically poor manifestation of an underlying malignancy, with majority of patients dying soon after diagnosis. It is important to be aware of this possibility in thrombotic microangiopathy, especially with atypical features and poor response to standard treatment.
Vasko
Radovan
R
Departments of Nephrology and Rheumatology, Georg-August-University, Goettingen, Germany. rvasko@inbox.com
Koziolek
Michael
M
Füzesi
Laszlo
L
König
Fatima
F
Strutz
Frank
F
Müller
Gerhard Anton
GA
D000230:Adenocarcinoma / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D009102:Multiple Organ Failure / Q000209:etiology; D010956:Plasmapheresis / Q000379:methods*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology* / Q000628:therapy; D013274:Stomach Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D017211:Treatment Failure