Heterogeneity among characteristics of hypereosinophilic syndromes

The 10 patients with F/P- CEL were treated with a median number of 3 therapeutic approaches, including allogeneic hematopoietic stem cell transplantation.\n2 (1.5-88) 11.5 (2.5-40) 9.6 (1.5-136) 6.6 (1.5-24) 6.1 (1.6-13.8) Eosinophils in bone marrow (%), median (range) 30.5 (11-80) 35 (7-61) 37 (14-64) 31 (24-40) 29 (15-40) Serum IgE >N (IU/mL)[low *] 27/40 (68%) 2/16 (13%) 5/8 (63%) 2/7 (29%) 2/3 (67%) Serum vitamin B12 >N (pg/mL)[low *] 3/38 (8%) 2/16 (13%) 2/8 (25%) 1/4 (25%) 0/3 (0%) Serum IL-5 >N (pg/mL)[low *] 10/27 (37%) 0/8 (0%) 0/8 (0%) 1/5 (20%) 0/3 (0%) Serum IL-4 >N (pg/mL)[low *] 4/24 (17%) 1/11 (9%) 1/6 (17%) 1/5 (20%) 1/3 (33%) Serum tryptase level >N (ng/mL)[low *] 1/24 (4%) 2/7 (29%) 4/8 (50%) 1/5 (20%) 0/3 (0%) Table I Characteristics of patients included in the study M-HES, Myeloproliferative variant of HES; WBC, white blood cell. Parameter IHES F/P+ CEL F/P- CEL M-HES L-HES3 Organ involvement  Cardiac 15/51 (29%) 3/16 (19%) 2/10 (20%) 4/8 (50%) NA Pulmonary 16/51 (31%) 2/16 (13%) 0/10 (0%) 1/8 (12%) NA Cutaneous 8/51 (16%) 0/16 (0%) 0/10 (0%) 1/8 (12%) 2/3 (66%) Splenomegaly 7/51 (14%) 11/16 (69%) 6/10 (60%) 4/8 (50%) 2/3 (66%) Hepatomegaly 4/51 (8%) 6/16 (38%) 4/10 (40%) 2/8 (25%) 1/3 (33%) Neurologic 4/51 (8%) 1/16 (6%) 2/10 (20%) NA NA Gastrointestinal 5/51 (10%) 0/16 (0%) 1/10 (10%) 1/8 (12%) NA Others Lymph nodes Lymph nodes No No Lymph nodes Constitutional symptoms (fever, weakness, weight loss, sweats, cough) Yes Yes Yes Yes Yes Steroids treatment  Response to monotherapy with steroids (%) 53 0 33 (steroids + HU) 12 100 CR (n) 27/51 NA NA NA NA PR (n) 18/51 NA 2/6 1/8 3/3 NR (n) 6/51 12/12 4/6 5/8 NA Current treatment and disease status 35 in PR/CR on maintenance dose, 10 in CR off therapy, 6 resistant 16 in CR on imatinib 2 in PR on HU + steroids, 1 in CR after AlloSCT, 7 died 2 in CR on imatinib, 4 in PR on steroids + HU, 2 died 2 in PR on steroids 1 in CR after ABCT (NHL transformation) [low *] Significant differences in laboratory findings between HES and CEL IHES, Idiopathic HES; M-HES, myeloproliferative variant of HES.