Contributions
Abstract: PB2509
Type: Publication Only
Background
Idiopathic hypereosinophilic syndrome (HES) is a disease characterized by an absolute eosinophil count (AEC) greater than 1500/mm3 lasting for more than 6 months in the absence of any other known cause of eosinophilia and with the evidence of eosinophil mediated tissue damage. HES presenting as multi-organ thrombosis is rare in children.
Aims
To report a pediatric case with idiopathic HES who presented with pulmonary embolism and deep venous thrombosis of lower limbs.
Methods
A retrospective review of patient’s electronic chart was conducted to retrieve clinical and laboratory data.
Results
A 14-year-old boy presented with shortness of breath, chest pain and swelling of the left leg to pediatric emergency. A Doppler ultrasound of lower limbs revealed thrombosis of the left femoral vein extending in to the left popliteal vein. A chest CT scan demonstrated segmental pulmonary embolism along with pleural effusion. He was started on Dalteparin and antibiotics. A complete blood count showed an AEC of 7770/mm3 and a platelet count of 79,000/mm3. The patient subsequently developed persistent fever along with worsening respiratory distress. His right lower limb became swollen and tender. A repeat Doppler ultrasound of lower limbs showed a new extensive occlusive clot of right femoral vein and popliteal vein. A worsening pleural effusion was noted on chest imaging. Marked eosinophilia was detected in pleural fluid (eosinophils-21%). His AEC was persistently elevated (Range: 6020/mm3-8250/mm3). A full diagnostic work up for infectious, immunological and rheumatolgical causes of eosinophilia came back negative. Bone marrow aspirate and biopsy were normocellular with marked non-dysplastic eosinophilia (23%). Bone marrow cytogenetics and flow cytometry were normal. BCR/ABL and JAK2 V617F mutations were negative by polymerase chain reaction (PCR). FIP1L1, CHIC2, PDGFRA and PDGFRB gene regions were normal by PCR. No evidence of B- or T-cell clonality was seen. He was started on methylprednisolone at a dose of 30mg/kg for 3 days with the working diagnosis of idiopathic HES. His clinical status improved significantly within 24 hours of starting steroids. Peripheral blood AEC normalized (AEC-60/mm3) within 3 days. Due to persistently sub-therapeutic anti-Xa levels, Dalteparin was changed to Apixaban. Oral prednisolone was started at dose of 2mg/kg/day following the pulse dose of methylprednisolone and was tapered based on his clinical status and AEC. Assessments done three months in to treatment showed resolving pulmonary emboli with normal pulmonary function and persistence of old thrombus in right and left leg with minimal collaterals. Clinically, he continues to do well on Apixaban and minimum doses of prednisolone.
Conclusion
Hypereosinophilic syndrome can present as multiple life-threatening thromboses in children. One should be aware of the thromboembolic complications of HES.
Session topic: 35. Thrombosis and vascular biology & translational Research
Keyword(s): Children, Hypereosinophilic syndrome, Thrombosis
Abstract: PB2509
Type: Publication Only
Background
Idiopathic hypereosinophilic syndrome (HES) is a disease characterized by an absolute eosinophil count (AEC) greater than 1500/mm3 lasting for more than 6 months in the absence of any other known cause of eosinophilia and with the evidence of eosinophil mediated tissue damage. HES presenting as multi-organ thrombosis is rare in children.
Aims
To report a pediatric case with idiopathic HES who presented with pulmonary embolism and deep venous thrombosis of lower limbs.
Methods
A retrospective review of patient’s electronic chart was conducted to retrieve clinical and laboratory data.
Results
A 14-year-old boy presented with shortness of breath, chest pain and swelling of the left leg to pediatric emergency. A Doppler ultrasound of lower limbs revealed thrombosis of the left femoral vein extending in to the left popliteal vein. A chest CT scan demonstrated segmental pulmonary embolism along with pleural effusion. He was started on Dalteparin and antibiotics. A complete blood count showed an AEC of 7770/mm3 and a platelet count of 79,000/mm3. The patient subsequently developed persistent fever along with worsening respiratory distress. His right lower limb became swollen and tender. A repeat Doppler ultrasound of lower limbs showed a new extensive occlusive clot of right femoral vein and popliteal vein. A worsening pleural effusion was noted on chest imaging. Marked eosinophilia was detected in pleural fluid (eosinophils-21%). His AEC was persistently elevated (Range: 6020/mm3-8250/mm3). A full diagnostic work up for infectious, immunological and rheumatolgical causes of eosinophilia came back negative. Bone marrow aspirate and biopsy were normocellular with marked non-dysplastic eosinophilia (23%). Bone marrow cytogenetics and flow cytometry were normal. BCR/ABL and JAK2 V617F mutations were negative by polymerase chain reaction (PCR). FIP1L1, CHIC2, PDGFRA and PDGFRB gene regions were normal by PCR. No evidence of B- or T-cell clonality was seen. He was started on methylprednisolone at a dose of 30mg/kg for 3 days with the working diagnosis of idiopathic HES. His clinical status improved significantly within 24 hours of starting steroids. Peripheral blood AEC normalized (AEC-60/mm3) within 3 days. Due to persistently sub-therapeutic anti-Xa levels, Dalteparin was changed to Apixaban. Oral prednisolone was started at dose of 2mg/kg/day following the pulse dose of methylprednisolone and was tapered based on his clinical status and AEC. Assessments done three months in to treatment showed resolving pulmonary emboli with normal pulmonary function and persistence of old thrombus in right and left leg with minimal collaterals. Clinically, he continues to do well on Apixaban and minimum doses of prednisolone.
Conclusion
Hypereosinophilic syndrome can present as multiple life-threatening thromboses in children. One should be aware of the thromboembolic complications of HES.
Session topic: 35. Thrombosis and vascular biology & translational Research
Keyword(s): Children, Hypereosinophilic syndrome, Thrombosis