Chronic idiopathic myelofibrosis with myeloid metaplasia presenting as refractory ascites.
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.
Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India.
Bijoy
B
Patra
Anu
A
Maheshwari
Jagdish
J
Chandra
Satinder
S
Aneja
Shilpi
S
Agarwal
Anita
A
Nangia
Anju
A
Seth
D001201:Ascites / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D002648:Child; D002908:Chronic Disease; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D055728:Primary Myelofibrosis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D012008:Recurrence