慢性嗜伊紅性白血球增高症 - tsim.org.tw

1 ( chronic or idiopathic hypereosinophilic syndrome ) 23 prednisolone 1/3 1/6 ( Chronic hypereosinophilic syndrome ) ( Eosinophilia ) ( Steroid ) ( chronic or idiopathic hypereosinophilic syndrome, HES ) ( involve ) 1975 Chusid (1) 1500/ l (2) (3) ( presumptive signs and symptoms of organ involvement ) 1 25-75% ( blastic transformation ) 1 84% ( ST-T ) 40% X ( infiltrative process ) 30% 27% 20% BUN SGPT SGOT 1 2, 3, 4 HES 5,6 HES ( chronic eosinophilic leukemia )

2 4 4 Wintrobe's 1964 Hilderbrand 7 idiopathic eosinophilia X-ray 5 HES HES 6 1,2 1998 Oliver HES 4 1. ( chronic benign HES ) 2. ( HES with organ involvement ) 3. ( chronic eosinophilic leukemia, CEL ) 4. ( CEL with blastic transformation )( ) ( ) HES 1,2 1,2 HES Chusid 14 HES 10 4 2 4 1 1 Flaum 2 hypodense 8 eosinophilic myelocyte ( myeloblast ) 1 ( )

3 4 9 HES 2, 3, 4 3 2, 3, 10 hydroxyurea10 vincristine11 6-mercaptopurine12 etoposide13 cyclosporin A14 alpha 9, 15-17 18 23 6 1/3 4 Oliver 4 I. A. 1. 1500/mm3 2. 3. B. 1. 2. (1) 25% (2) myeloblasts 5% (3) naphthol chloroacetate II.

4 1. 2. myeloblasts 30% 23 87 8 6 86 9 1 9900/mm3 Neutrophil/ Lymphocyte 92%/8% 120/80 mmHg 76 20 C H3 ( H3 cell counter, Beyer, New York, USA ) l gm/dl MCV 9500 / l neutrophil / lymphocyte / monocyte / eosinophil / basophil / / / 66% / Luc ( Luc H3 peroxidase 1-3% ) 247000/ l gm/dl ( gm/dl ) gm/dl mg/dl ( mg/dl ) Widal Weil-Felix X 87 9 4 11870/ l 87 10 2 13660/ l 4037-7174/ l eosinophilic myelocyte eosinophilic metamyelocyte 10% eosinophilic band form eosinophilic segment form 14% ( mast cell disease ) 19 87 10 28 ng/ml ( ng/ml ) B12 279 pg/ml ( 200-950 pg/ml ) 18 16 ( 46,XY ) (45,XY,-6 ) ( 45,XY,-17 ) HTLV-I+II ANA EBV-VCA IgM C3 g/L ( g/L ) leukocyte alkaline phosphatase ( LAP ) score 135 ( 110-190 )

5 C4 g/L ( g/L ) IgA g/L ( g/L ) IgG g/L ( g/L ) IgM g/L ( g/L ) IgE IU/ml ( 250 IU/ml ) 87 12 21 gm/dl 88 1 6 B12 pg/ml IgE 1915 IU/ml IgG IgA g/L B12 1 ml 88 2 10 / l gm/dl MCV 11820/ l neutrophil/lymphocyte/monocyte/eosinophi l / basophil ( Luc ) 180x103 / l 87 8 6 88 2 10 4000-7000 / l ( ) mg / kg prednisolone ( 30mg ) gm/dl 1 mg/kg ( 60mg ) 3214/ l 1959 / l 58 1244/ l 831 / l 10 prednisolone 20 mg 10 mg 10mg qod 5mg qod 88 7 15 1310 / l ( ) gm/dl HES megakaryocyte B12 2 LAP score 4 (myeloproliferative varients ) IgE ( non-malignant varient ) Flaum ( ) 2 ( ) 3 2,3 HES Chusid Oliver ( )

6 4 ( endocardial fibrosis ) HES 2 2 1 1 2 myeloid dyspoiesis hypersegmentation 1 basophilia 200 /mm3 myeloblasts progranulocytes 2 metamyelocytes myelocytes 1 1 2 megakaryoctes 1 myelofibrosis 2 myeloid dyspoiesis 2 basophilia 1 2 myeloblasts-progranulocytes 5 2 2 B12 1 LAP 1 (chronic eosinophilic leukemia, CEL ) CEL naphthol chloroacetate esterase ( ) HES HES 30 4 4 ( ) 3 1.

7 A. 5 B. 5 C. 11mm 4 3 D. 3 E. 3 F. 2 2. A. 5 B. 3 C. 2 3. A. X 3 B. X 2 C.

8 2 4. A. 2 B. 2 C. 2 5. A. 2 6. A. 1 B. 1 3. A. 2 B. 1 5. A. 3 (random loss ) CEL random loss 4 clonal DNA Chusid 1 Flaum 2 2 HES B12 LAP score 3 LAP score 87 10 28 B12 ( 88 1 6 ) B12 ( Vit B12 )

9 B12 gm/dl IgE HES B12 LAP score IgE 2 1 Oliver ( antieosinophil therapy ) (1) (2) 11 (1) (2) 7174-4037/ l (3) ( 45,XY,-6 ) ( 45,XY,-17 ) (4) steroid (5) prednisolone prednisolone 1 mg/kg 5mg qod 1500/ml alpha-interferon alpha-interferon 9, 17 hydroxyurea 18 Oliver Chusid Oliver Toxocara canis, Trichinella spirella Ascaris 20 21 22-26 MJ, Dale DC, West BC, Wolff SM.

10 The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine 1975; 54: 1-27. MA, Schooley RT, Fauci AS, Gralinick HR. A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. I. hematologic manifestations. Blood 1981; 58: 1012-20. RT, Flaum MA, Gralnick HR, Fauci AS. A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. clinical manifestations. Blood 1981; 58: 1021-6. JW, Deol I, Morgan DL, Tonk VS. Chronic eosinophilic leukemia and hypereosinophilic syndromes: proposal for classification, literature review and report of a case with a unique chromosomal abnormality. Cancer Genet Cytogenet 1998;107: 111-7. JD, Rowley JD, Plapp F, Golomb HM, Ultmann JE. Chromosomal aneuploidy in a patient with hypereosinophilic syndrome. Evidence for a malignant disease. Am J Med 1977; 63: 1010-4.