Fatal thrombotic thrombocytopenic purpura (TTP) presenting concurrently with metastatic multiple endocrine neoplasia (MEN) type I.
A 44-year-old women was treated for hyperparathyroidism resulting from parathyroid hyperplasia. Several months later, following a flu-like episode, she developed fever, confusion, abdominal pain, and diffuse petechiae, with severe thrombocytopenia and hemolytic anemia. She died on the 11th day of hospitalization. At autopsy she had multiple endocrine neoplasia type I, with two islet cell tumors, adrenal adenoma, pituitary adenoma, and bronchial carcinoid with liver metastasis. Florid visceral microthrombi involved arterioles and capillaries of the heart, including the conduction system. Brain, kidney, pancreas, adrenal, and portal areas of the liver were also heavily involved, but thrombi were rare in the liver sinusoids and the lungs. PAS-positive subendothelial deposits were demonstrated. In spite of the disseminated malignancy, the morphologic and laboratory findings were inconsistent with disseminated intravascular coagulation (DIC), and supported the clinical diagnosis of TTP. To the best of our knowledge this is the first report association of TTP with MEN and raises the question of a genetic linkage and/or hormonal interaction.
Kouides
P A
PA
Department of Medicine, Rochester General Hospital, University of Rochester School of Medicine, NY 14621, USA.
Phatak
P D
PD
Cramer
S F
SF
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications* / Q000473:pathology; D009362:Neoplasm Metastasis; D011696:Purpura, Thrombocytopenic / Q000150:complications* / Q000473:pathology