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Published on: July 31, 2016
Chronic idiopathic myelofibrosis with myeloid metaplasia presenting as refractory ascites
Bijoy Patra1, Anu Maheshwari, Jagdish Chandra
1Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India.
Abstract:
Chronic idiopathic myelofibrosis (CIM) with myeloid metaplasia is a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extra-medullary hematopoesis (EMH), and varying degree of myelofibrosis. CIM, presenting as refractory ascites secondary to peritoneal hematopoesis, is extremely rare with only six adult cases reported in literature. This is a report of a child with CIM presenting as refractory ascites as a consequence of EMH in the peritoneum. The patient was treated with intermittent hydroxyurea with favorable response over 3 weeks. The patient was thereafter lost to follow up.
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