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Year 2012, Volume: 34 Issue: 3, 365 - 368, 14.05.2012

Abstract

References

  • Weinstein IM. Idiopathic myelofibrosis: historical review, diagnosis and management. Blood Rev 1991; 5: 98.
  • Tefferi A, Spivak JL. Polycythemia vera: Scientific advances and current practice. Semin Hematol 2005; 42: 206-20.
  • Guardiola P, Esperou H, Cazals-Hatem D, Ifrah N, Jouet JP, Buzyn A, Sutton L, Gratecos N, Tilly H, Lioure B, Gluckman E. Allogeneic bone marrow transplantation for agnogenic myeloid metaplasia. French Society of Bone Marrow Transplantation. Br J Haematol 1997; 98: 1004.
  • Chen SW, Hwang WS, Tsao CJ, Liu HS, Huang GC. Hydroxyurea and splenic irradiation-induced tumour lysis syndrome: a case report and review of the literature. J Clin Pharm Ther 2005; 30: 623-5.
  • Weinmann M, Becker G, Einsele H, Bamberg M. Clinical indications and biological mechanisms of splenic irradiation in chronic leukaemias and myeloproliferative disorders. Radiother Oncol 2001; 58: 235-46.
  • Slanina J, Vondraczek A, Wannenmacher M. Symptomatic irradiation therapy of the spleen in advanced osteomyelosclerosis. Dtsch Med Wochenschr 1986; 111: 1144-50.
  • Paulino AC, Reddy SP. Splenic irradiation in the palliation of patients with lymphoproliferative and myeloproliferative disorders. Am J Hosp Palliat Care 1996; 13: 32-5.
  • McFarland JT, Kuzma C, Millard FE, Johnstone PA. Palliative irradiation of the spleen. Am J Clin Oncol 2003; 26: 178-83.
  • Parmentier C, Charbord P, Tibi M, Tubiana M. Splenic irradiation in myelofibrosis. Clinical findings and ferrokinetics. Int J Radiat Oncol Biol Phys 1977; 2: 1075.
  • Cihan YB, Yokuş O, Mutlu H. Kronik miyeloproliferatif hastalığıolan yaşlılarda gelişen semptomatik splenomegalide palyatif radyoterapinin rolü. Turkish Journal of Geriatrics 2012; 15: 34-9.

İdiyopatik myelofibrozis tanılı olguda adaptif splenik radyoterapi

Year 2012, Volume: 34 Issue: 3, 365 - 368, 14.05.2012

Abstract

Özet

İdiyopatik myelofibrosis  kronik myeloproliferatif bir hastalıktır. Kronik myeloproliferatif hastalıklar myeloid hücre büyümesi ile ilişkili klonal kök hücre bozukluklarıdır. Kemik iliği yetersizliğine bağlı görülen ekstramedüller hematopoez ve artmış eritropoez sonucu masif splenomegali gelişir, bu da anemi ve abdominal rahatsızlığa yol açarak morbiditeye sebep olur. Splenik radyoterapi masif splenomegaliye bağlı görülen abdominal ağrı, anemi ve trombositopeni gibi semptomları palyasyon amacıyla önerilmektedir. Bu çalışmada idiyopatik myelofibrozis tanısı ile takip edilen 59 yaşındaki erkek olgu değerlendirildi. Masif splenomegaliye bağlı narkotik analjezik kullanımı gerektiren şiddetli ağrı, hipersplenizmin neden olduğu ciddi trombositopeni ve tekrarlayan transfüzyon ihtiyacı sonucu palyasyon amacı ile haftada 2 fraksiyon toplamda 6 fraksiyon olacak şekilde haftalık artan dozlarda splenik RT uygulandı. Tedavi sonrası olgunun abdominal ağrısında %100 palyasyon sağlandığı görüldü. Splenik RT, semptomatik splenomegalinin palyatif tedavisinde etkin bir tedavi modalitesi olarak göz önünde bulundurulmalıdır.

Anahtar sözcükler: İdiyopatik myelofibrozis, splenomegali, splenik radyoterapi

 

Abstract

Idiopathic myelofibrosis  is a chronic myeloproliferative disorder. Chronic myeloprolipherative disorders are clonal stem cell disorders associated with myeloid cell growth  . Extramedullary hematopoiesis in the bone marrow deficiency and increased erythropoiesis resultin  massive splenomegaly which then lead to anemia and abdominal discomfort  which are major causes of morbidity in these cases. Splenic radiotherapy  is recommended for palliation of symptoms such as abdominal pain, anemia and thrombocytopenia  due to massive splenomegaly. In this study, a 59-year-old male patient with a diagnosis of idiopathic myelofibrosis was evaluated. Splenic radiotherapy was applied to him in 2 fractions per week with increasing doses for a total of 6 fractions  as he had  massive splenomegaly, severe pain requiring narcotic analgesic use, and had repeated  need for transfusion caused by severe thrombocytopenia due to hypersplenism His abdominal pain palliation was achieved by 100% after treatment. . Splenic radiotherapy should be considered as an effective treatment modality for palliative treatment of symptomatic splenomegaly.

Keywords: Idiopathic myelofibrosis, splenomegaly, splenic radiotherapy

References

  • Weinstein IM. Idiopathic myelofibrosis: historical review, diagnosis and management. Blood Rev 1991; 5: 98.
  • Tefferi A, Spivak JL. Polycythemia vera: Scientific advances and current practice. Semin Hematol 2005; 42: 206-20.
  • Guardiola P, Esperou H, Cazals-Hatem D, Ifrah N, Jouet JP, Buzyn A, Sutton L, Gratecos N, Tilly H, Lioure B, Gluckman E. Allogeneic bone marrow transplantation for agnogenic myeloid metaplasia. French Society of Bone Marrow Transplantation. Br J Haematol 1997; 98: 1004.
  • Chen SW, Hwang WS, Tsao CJ, Liu HS, Huang GC. Hydroxyurea and splenic irradiation-induced tumour lysis syndrome: a case report and review of the literature. J Clin Pharm Ther 2005; 30: 623-5.
  • Weinmann M, Becker G, Einsele H, Bamberg M. Clinical indications and biological mechanisms of splenic irradiation in chronic leukaemias and myeloproliferative disorders. Radiother Oncol 2001; 58: 235-46.
  • Slanina J, Vondraczek A, Wannenmacher M. Symptomatic irradiation therapy of the spleen in advanced osteomyelosclerosis. Dtsch Med Wochenschr 1986; 111: 1144-50.
  • Paulino AC, Reddy SP. Splenic irradiation in the palliation of patients with lymphoproliferative and myeloproliferative disorders. Am J Hosp Palliat Care 1996; 13: 32-5.
  • McFarland JT, Kuzma C, Millard FE, Johnstone PA. Palliative irradiation of the spleen. Am J Clin Oncol 2003; 26: 178-83.
  • Parmentier C, Charbord P, Tibi M, Tubiana M. Splenic irradiation in myelofibrosis. Clinical findings and ferrokinetics. Int J Radiat Oncol Biol Phys 1977; 2: 1075.
  • Cihan YB, Yokuş O, Mutlu H. Kronik miyeloproliferatif hastalığıolan yaşlılarda gelişen semptomatik splenomegalide palyatif radyoterapinin rolü. Turkish Journal of Geriatrics 2012; 15: 34-9.
There are 10 citations in total.

Details

Primary Language Turkish
Journal Section Case Reports
Authors

Ayfer Ay

Mehmet Eren

Publication Date May 14, 2012
Published in Issue Year 2012Volume: 34 Issue: 3

Cite

AMA Ay A, Eren M. İdiyopatik myelofibrozis tanılı olguda adaptif splenik radyoterapi. CMJ. December 2012;34(3):365-368.