287 Frequency of factor ii, factor v leiden and mthfr mutations in children with cancer

Jelena Roganovic Roganovic, Ana Djordjevic, Blazenka Grahovac, Lidija Bilic Zulle, Jelena Roganovic
2021 Abstracts   unpublished
Aim To present seven cases of Posterior Reversible Encephalopathy Syndrome (PRES), diagnosed between 2014-2019, as a complication of acute leukemia treatment in children. PRES was cited for the first time in 1996 by Hinkey and collaborators as a posterior leukoencephalopathy syndrome. Methods These pacients were admitted and treated for Acute lymphoblastic leukaemia during a 5 years period. From the total number of 162 patients with leukemia only 7 cases developed PRES. They were 3 to 12 yo, 5
more » ... ales and 2 females. None of them had a preexisting neurologic disorder. PRES occured during the induction chemotherapy. All patients underwent clinical evaluation, ECG, echocardiography and laboratory tests. At the onset of the neurological symptoms emergency head CT-scan was performed, followed by head MRI which confirmed the diagnosis. Results Neurologic manifestation of PRES consisted of upper or lower limb muscle spasm, marked agitation, aggressive behavior, generalized seizures followed by coma, right upper limb hemiparesis and facial paresis. The first patient developed PRES as early as the 9-th day of treatment after receiving high dose Prednison that induced hypertension. In the rest of the children PRES occurred simultaneously with sepsis (due to severe bone marrow aplasia), SIADH (Syndrome of inappropriate antidiuretic hormone secretion) or uncontrolled high blood pressure due to corticotherapy. Blood pressure was controlled only with combined antihypertensive agents at maximal dose. The PRES diagnose was consistently based on cerebral MRI neuroimages that showed typical lesions of vasogenic edema with hyperintense T2 and FLAIR signals with a characteristic location for PRES. Prompt anticonvulsant and antihypertensive therapy was started, and the patients made a full neurological recovery. The MRI was repeated with minimal ischemic lesions after 1 mo and almost normal after 3 mo. The treatment protocol for leukemia was interrupted from 12 to 43 days. Conclusions PRES is a rare, severe and almost new described complication in children with leukemia. We have to recognize and to treat it promptly in order to save the patient. A multidisciplinary team (pediatric oncologist, cardiologist, neurologist, radiologist and intensive care specialists) is necessary to manage such cases. A positive diagnosis was established after performing cerebral MRI. Severe acute hypertension was controlled only with a combination of for anti-hypertensive drugs administered at a maximum dose. The question regarding the etiology of PRES is still present, because only patients with sepsis, SIADH and hypertension due to corticotherapy developed the syndrome from all leukemia patients.
doi:10.1136/archdischild-2021-europaediatrics.287 fatcat:3dzggvkdmvcmljpdrtq6afbhgq