Hypereosinophilic Syndrome, Multiorgan Involvement and Response to Imatinib

Abstract

Hypereosinophilic syndrome (HES) is an uncommon syndrome characterized by peripheral blood eosinophils count of more than 1,500/mm³ with associated tissue damage. It can be either primary or secondary, for example, due to parasitic infections or inflammation. We present a case of a 49-year-old Asian female with recurrent hospital admissions for cholecystitis, gastritis, urinary cystitis, and pancreatitis. Her peripheral blood count showed excessive eosinophils 15,600-19,000/mm³ on different occasions. Pathology of her gallbladder and her gastric biopsies showed eosinophilic infiltration. Her bone marrow biopsy showed a normocellular marrow with active trilineage hematopoiesis, eosinophilia, mild megakaryocytic hyperplasia with a few atypical forms, and mild T-cell lymphocytosis. Flow cytometry showed no evidence of acute leukemia, or T-cell or B-cell lymphoproliferative disorder. On fluorescent in-situ hybridization (FISH), myeloproliferative neoplasms (MPN) testing was negative for platelet-derived growth factor receptor-alpha (PDGFRA), platelet-derived growth factor receptor-beta (PDGFRB), and fibroblast growth factor receptor-1 (FGFR1) rearrangement. Despite not having the FIP1L1-PDGFRA (factor interacting with PAPOLA and CPSF1-platelet-derived growth factor receptor, alpha polypeptide) gene fusion, our patient responded to the treatment with a significant decrease in her absolute eosinophils count and resolution of her symptoms.

Keywords: eosinophilic cholecystitis; eosinophilic cystitis; eosinophilic pancreatitis; hypereosinophilic syndrome; imatinib; myeloproliferative neoplasm.

Figure 1. Ultrasound picture showing the bladder wall thickening

Figure 2. CT scan of the abdomen and pelvis showing the posterior 2.5 x 6.6 cm bladder mass (a) and anterior 1.3 x 2.1 cm bladder mass (b)