Idiopathic hypereosinophilic syndrome complicated by central sinovenous thrombosis.
Idiopathic hypereosinophilic syndrome (IHES) is a rare disorder affecting cardiac, pulmonary and nervous systems with peripheral neuropathy, encephalopathy and cerebral thromboembolism. We report a 7-year-old boy with IHES who developed central sinovenous thrombosis and cerebral hemorrhage. Although he had hypereosinophilia for more than 6 months, he was asymptomatic until the sudden onset of vomiting and headache due to superior sagittal sinus thrombosis. Molecular analysis in peripheral blood did not reveal any mutation in the Fip1-like-platelet-derived growth factor receptor alpha chain (FIP1L1-PDGFRA) gene which was recently reported to be mutated in IHES. Since there must be symptoms or signs of organ involvement to fulfill the IHES criteria, we could not make a diagnosis of IHES until the onset of central nervous system involvement. We suggest that anti-coagulant therapy should be considered for asymptomatic patients with hypereosinophilia because hypereosinophilia may induce thrombosis in various organs.
Sakuta
Ryoichi
R
Department of Pediatrics, Dokkyo Medical University, Koshigaya Hospital, Koshigaya, Saitama 343-8555, Japan.
Tomita
Yuzo
Y
Ohashi
Makiko
M
Nagai
Toshiro
T
Murakami
Nobuyuki
N
D000925:Anticoagulants / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D002543:Cerebral Hemorrhage / Q000209:etiology; D002648:Child; D004252:DNA Mutational Analysis; D004827:Epilepsy / Q000150:complications / Q000188:drug therapy; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications*; D007958:Leukocyte Count; D008297:Male; D008583:Meningitis, Haemophilus / Q000150:complications; D012851:Sinus Thrombosis, Intracranial / Q000150:complications*; D014635:Valproic Acid / Q000627:therapeutic use; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics
D000925:Anticoagulants; D000927:Anticonvulsants; C479818:FIP1L1 protein, human; D039221:mRNA Cleavage and Polyadenylation Factors; D014635:Valproic Acid