Hypereosinophilic Syndrome Presenting As Acute Pancreatitis

Abstract

RationaleHypereosinophilic syndrome (HES) is characterized by heterogeneous clinical presentations depending on the specific organ involvement. Few cases with pancreatic involvement have been reported in the adult literature. We present a case of pediatric HES presenting with acute pancreatitis.MethodsA 17-year-old male presented with acute abdominal pain and elevated lipase (3000 u/L), amylase (237 u/L), and ALP (1371 u/L). His absolute eosinophilic count (AEC) was 1400/mm3. He was diagnosed with autoimmune pancreatitis and was started on 30 mg of prednisone daily with excellent clinical response and normalization of laboratory values. After tapering to 5 mg every other day, the patient experienced a relapse of epigastric pain. Laboratory tests revealed AEC of 30,000/mm3, normal lipase and amylase, elevated sedimentation rate (47 mm/hr), ALP (707 u/L), AST (93 u/L) and ALT (66 u/L).ResultsBone marrow biopsy showed marked eosinophilia without evidence of B and T cell lymphoproliferative disease. Testing for parasitic infections was negative; mutation analysis for JAK2V617F mutation was negative and is pending for FIP1LI PDGFRA mutation. ECG showed sinus bradycardia (42/min). 2D cardiac Echo and troponin levels were normal as was a serum tryptase level. He was treated with high dose prednisolone at 60mg daily with rapid resolution of clinical symptoms as well as peripheral eosinophilia.ConclusionsA pubmed search revealed only 3 cases of HES with pancreatic involvement reported in adult patients. To the best of our knowledge, this is the first reported case of HES presenting with pancreatitis in the pediatric age group. RationaleHypereosinophilic syndrome (HES) is characterized by heterogeneous clinical presentations depending on the specific organ involvement. Few cases with pancreatic involvement have been reported in the adult literature. We present a case of pediatric HES presenting with acute pancreatitis. Hypereosinophilic syndrome (HES) is characterized by heterogeneous clinical presentations depending on the specific organ involvement. Few cases with pancreatic involvement have been reported in the adult literature. We present a case of pediatric HES presenting with acute pancreatitis. MethodsA 17-year-old male presented with acute abdominal pain and elevated lipase (3000 u/L), amylase (237 u/L), and ALP (1371 u/L). His absolute eosinophilic count (AEC) was 1400/mm3. He was diagnosed with autoimmune pancreatitis and was started on 30 mg of prednisone daily with excellent clinical response and normalization of laboratory values. After tapering to 5 mg every other day, the patient experienced a relapse of epigastric pain. Laboratory tests revealed AEC of 30,000/mm3, normal lipase and amylase, elevated sedimentation rate (47 mm/hr), ALP (707 u/L), AST (93 u/L) and ALT (66 u/L). A 17-year-old male presented with acute abdominal pain and elevated lipase (3000 u/L), amylase (237 u/L), and ALP (1371 u/L). His absolute eosinophilic count (AEC) was 1400/mm3. He was diagnosed with autoimmune pancreatitis and was started on 30 mg of prednisone daily with excellent clinical response and normalization of laboratory values. After tapering to 5 mg every other day, the patient experienced a relapse of epigastric pain. Laboratory tests revealed AEC of 30,000/mm3, normal lipase and amylase, elevated sedimentation rate (47 mm/hr), ALP (707 u/L), AST (93 u/L) and ALT (66 u/L). ResultsBone marrow biopsy showed marked eosinophilia without evidence of B and T cell lymphoproliferative disease. Testing for parasitic infections was negative; mutation analysis for JAK2V617F mutation was negative and is pending for FIP1LI PDGFRA mutation. ECG showed sinus bradycardia (42/min). 2D cardiac Echo and troponin levels were normal as was a serum tryptase level. He was treated with high dose prednisolone at 60mg daily with rapid resolution of clinical symptoms as well as peripheral eosinophilia. Bone marrow biopsy showed marked eosinophilia without evidence of B and T cell lymphoproliferative disease. Testing for parasitic infections was negative; mutation analysis for JAK2V617F mutation was negative and is pending for FIP1LI PDGFRA mutation. ECG showed sinus bradycardia (42/min). 2D cardiac Echo and troponin levels were normal as was a serum tryptase level. He was treated with high dose prednisolone at 60mg daily with rapid resolution of clinical symptoms as well as peripheral eosinophilia. ConclusionsA pubmed search revealed only 3 cases of HES with pancreatic involvement reported in adult patients. To the best of our knowledge, this is the first reported case of HES presenting with pancreatitis in the pediatric age group. A pubmed search revealed only 3 cases of HES with pancreatic involvement reported in adult patients. To the best of our knowledge, this is the first reported case of HES presenting with pancreatitis in the pediatric age group.