Abstract
Myelofibrosis kemik iliğinin hiposellülaritesi, fibroz elemanlarının artımı ve myeloid metaplazi olarak bilinen ekstramedüller hematopoesisi yansıtan hepatosplenomepali ile karakterize bir klinik tablodur. Daha çok yetişkinlerin bir hastalığı olan primer myelofibrosis çocuklarda nadir olarak görülmekte ve bu yaş grubunda bildirilen olguların büyük bir kesimini sekonder myelofibrosis oluşturmaktadır. 1,25 Dihidroksikolekalsiferolün (1,25 DHCC) kemik mineralizasyonundaki etkisi kadar hematopoetik doku üzerindeki etkisi de dikkati çekmektedir. D vitamini eksikliği ile birlikte olabilen myelofibrosis olguları giderek artan sayıda bildirilmektedir. D vitamini eksikliğine bağlı raşitzme sekonder myelofibrosis tanısı alan ve tüm bulguları D vitamini tedavisi ile düzelen on iki aylık bir olgu takdim edildi.
References
- 1. Arlet PH Nicodeme R A Done D Foumnier D Delsol G Talle Y : Clinical evidence for 1,25 Hydroxychole calciferol action in myelofibrosis, Lancet 1 : 1013, 1984.
- 2. Boxer : Anemia in primary hyperparatlhıyroidism. Arch Intern Med. 137 : 588- 90, 1977.
- 3. Brovall C Mitchell H Saral R Santos GV Cıvın CI : Acute myelofibrosis in the child. J. Pediatr. 103 : 91, 1083.
- 4. Cooperg AA Singer OP : Reversibl myelofibrosis due to vitamin D deficiency rickets, Can. Med. Assoc. J, 94 : 392-95, 1966.
- 5. Editorial : Vitamin D and the Iymphomedullary system. Lancet 1 : 1105-9, 1884.
- 6, Manopolas S Deftos LJ : The vitamin D endocrine system and the hematolymphopoeietiç Tissue, Ann. Intern, Med. 100 : 144-6, 1984,
- 7. lLau S Ramsay NKC Smit li CM KacKenna R Kersey JH : Spontaneous resolution of severe childhood myelofibrosis. J. Pediatr. 98 : 585-88, 1981.
- 8. Say B Berkel İ : Idiopathic myelofibrosis in an infant. J. Pediatr 64 : 5680-85, 1964.
Abstract
Myelofibrosis which is known as myeloid metaplasia is characterized by hypocellularity and fibrosis of the bone marrow. The disease is almost always associated with on enlarged liver and spleen asa result of exatramedullary hemotopoesis, Idiopathic myelofibrosis is a disorder and is relatively move İreguent in adults. In children secondary myelofibrosis accounts most of the cases. The importance of 1,25 DHCC on mineral metabloism is well known, and it is also known that calcitriol is effective on hematopoetic system. Rickets and secondary myelofibrosis according to vitamin D deficiency has besn reported in increasing number.This report a patient with rickets and secondary myelofibrosis according to vitamin D deficiendey whose finding are reversible by vitamin D treatment is described.
Keywords
myelofibrosis secondary to rickets Vitamin D deficiency Myelofibrosis is hypocellularity of the bone marrow
References
- 1. Arlet PH Nicodeme R A Done D Foumnier D Delsol G Talle Y : Clinical evidence for 1,25 Hydroxychole calciferol action in myelofibrosis, Lancet 1 : 1013, 1984.
- 2. Boxer : Anemia in primary hyperparatlhıyroidism. Arch Intern Med. 137 : 588- 90, 1977.
- 3. Brovall C Mitchell H Saral R Santos GV Cıvın CI : Acute myelofibrosis in the child. J. Pediatr. 103 : 91, 1083.
- 4. Cooperg AA Singer OP : Reversibl myelofibrosis due to vitamin D deficiency rickets, Can. Med. Assoc. J, 94 : 392-95, 1966.
- 5. Editorial : Vitamin D and the Iymphomedullary system. Lancet 1 : 1105-9, 1884.
- 6, Manopolas S Deftos LJ : The vitamin D endocrine system and the hematolymphopoeietiç Tissue, Ann. Intern, Med. 100 : 144-6, 1984,
- 7. lLau S Ramsay NKC Smit li CM KacKenna R Kersey JH : Spontaneous resolution of severe childhood myelofibrosis. J. Pediatr. 98 : 585-88, 1981.
- 8. Say B Berkel İ : Idiopathic myelofibrosis in an infant. J. Pediatr 64 : 5680-85, 1964.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Pediatric Metabolism Diseases |
| Journal Section | Research Article |
| Authors | |
| Publication Date | June 30, 1991 |
| Published in Issue | Year 1991 Volume: 44 Issue: 2 |