HYPEREOSINOPHILIC SYNDROME: EXPLORING DIAGNOSTIC DILEMMA

Introduction Hypereosinophilic Syndrome (HES) is a group of disorders with peripheral eosinophilia (1500/mm3) and end organ manifestations from eosinophils or inexplicable otherwise. HES's heterogeneity makes diagnosis and treatment challenging. Case Description A 53 yo female presented with significant extremity edema with 15-pound weight gain in two months despite oral furosemide. Initial labs revealed an AEC of 2100, albumin of 2.8 g/dL, CRP of 24.7 mg/L., re-check showed AEC of 1500, CRP of 33.1 mg/L, albumin of 3 g/dL two weeks later. Ultrasound of her lower extremities, ESR, CK, coags, UA, BNP, TSH, lipid profile, CK, ESR, HIV, Hepatitis panel, CCP, ANA, SSA, UA, and Urine Protein/Cr were all normal. At our visit Vitamin B12 was 1211 pg/mL. B cell rearrangement, IgE, Tryptase, Strongyloides IgG, TCR, lymphocyte profile, CHIC2, troponins, ECHO, and CT chest/abd/pelvis were normal. Bone marrow biopsy revealed trilineage hematopoietic activity (M/E = 4:1), with increased Eos. The remainder of the molecular oncologic HES workup was negative. Prednisone provided some relief but symptoms continued despite no peripheral eosinophilia. Aldolase of 9.6 U/L points toward involvement of skeletal muscle and a diagnosis of eosinophilic fasciitis. Discussion HES is a heterogeneous group of illnesses. Care must be taken to clarify the etiology and start treatment. Anti IL-5 therapy or cyclophosphamide may be used as steroid-sparing therapies for HES, cyclophosphamide has shown complete remission. Prednisone is the treatment of choice for eosinophilic fasciitis; other medications are being investigated as possible therapies.