Idiopathic myelofibrosis with generalized periostitis in a 4-year-old girl.
Idiopathic myelofibrosis, a chronic myeloproliferative disorder of unknown origin, is characterized by splenomegaly, extramedullary hematopoiesis, leukoerythroblastosis, teardrop erythrocytes, and myelofibrosis. It is a rare disorder in childhood. The authors describe a 4-year-old girl with features consistent with idiopathic myelofibrosis, who also had generalized solid laminated periosteal reaction involving all long bones. The presence of thrombocytopenia at the onset and lack of leukocytosis were in contrast to the reported features seen in children. Recent case reports describe a relatively indolent course in children. Spontaneous remissions have also been described in pediatric cases. The fulminant course of this patient without any features of malignant transformation was noteworthy in this regard.
Walia
Mandeep
M
Department of Pediatrics, V. M. Medical College & Safdarjang Hospital, New Delhi, India. mandeepkaurwalia@hotmail.com
Mehta
Rajesh
R
Paul
Premila
P
Saluja
Sumita
S
Kapoor
Sujala
S
Sharma
Monika
M
D001853:Bone Marrow / Q000473:pathology; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007194:India; D010522:Periostitis / Q000175:diagnosis* / Q000473:pathology; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000473:pathology