ISSN: 1300-7777 E-ISSN: 1308-5263
Severe Myelotoxicity Associated with Thiopurine S-Methyltransferase*3A/*3C Polymorphisms in a Patient with Pediatric Leukemia and the Effect of Steroid Therapy [Turk J Hematol]
Turk J Hematol. 2014; 31(4): 399-402 | DOI: 10.4274/tjh.2013.0082  

Severe Myelotoxicity Associated with Thiopurine S-Methyltransferase*3A/*3C Polymorphisms in a Patient with Pediatric Leukemia and the Effect of Steroid Therapy

Burcu Fatma Belen1, Turkız  Gursel1, Nalan Akyurek2, Meryem Albayrak3, Zühre  Kaya1, Ülker  Kocak1
1Gazi University Faculty Of Medicine, Department Of Pediatric Hematology, Ankara, Turkey
2Gazi University Faculty Of Medicine, Department Of Pathology, Ankara, Turkey
3Kırıkkale University Faculty Of Medicine, Department Of Pediatric Hematology, Ankara, Turkey

Myelosuppression is a serious complication during treatment of acute lymphoblastic leukemia and the duration of myelosuppression is affected by underlying bone marrow failure syndromes and drug pharmacogenetics caused by genetic polymorphisms. Mutations in the thiopurine S-methyltransferase (TPMT) gene causing excessive myelosuppression during 6-mercaptopurine (MP) therapy may cause excessive bone marrow toxicity. We report the case of a 15-year-old girl with T-ALL who developed severe pancytopenia during consolidation and maintenance therapy despite reduction of the dose of MP to 5% of the standard dose. Prednisolone therapy produced a remarkable but transient bone marrow recovery. Analysis of common TPMT polymorphisms revealed TPMT *3A/*3C.

Keywords: Myelosuppression, Thiopurine S-methyl transferase, Acute leukemia


Pediatrik Bir Lösemi Olgusunda Tiyopurin S-Metiltransferaz *3A/*3C Polimorfizmi ile İlişkili Ağır Miyelotoksisite-Steroid Tedavisinin Etkisi

Burcu Fatma Belen1, Turkız  Gursel1, Nalan Akyurek2, Meryem Albayrak3, Zühre  Kaya1, Ülker  Kocak1
1Gazi Üniversitesi Tıp Fakültesi, Pediatrik Hematoloji Anabilim Dalı, Ankara
2Gazi Üniversitesi Tıp Fakültesi, Patoloji Anabilim Dalı, Ankara
3Kırıkkale Üniversitesi Tıp Fakültesi, Pediatrik Hematoloji Anabilim Dalı, Ankara

Miyelosupresyon, akut lenfoblastik lösemi tedavisinde görülen ciddi bir komplikasyon olup, miyelosupresyon süresi kemik iliği yetmezlik sendromlarından veya genetik polimorfizmin yol açtığı ilaç farmakogenetiğinden etkilenmektedir. Merkaptopürin metabolizasında yer alan Thiopurin s-metil transferaz (TPMT) enziminin azalmış aktivitesine neden olan polimorfizmleri artmış kemik iliği toksisitesine yol açar. Burada, 15 yaşında TPMT *3A/*3C ve MTHFR polimorfizmleri saptanan ve konsolidasyon/ idame tedavisi boyunca MP’nin standart dozun %5’inde yoğun miyelosupresyon görülen ve steroid tedavisi ile geçici düzelme saptanan bir T-ALL olgusu sunulmaktadır.

Anahtar Kelimeler: Miyelosupresyon, Thiopurin S-metil transferaz, Akut lösemi


Burcu Fatma Belen, Turkız  Gursel, Nalan Akyurek, Meryem Albayrak, Zühre  Kaya, Ülker  Kocak. Severe Myelotoxicity Associated with Thiopurine S-Methyltransferase*3A/*3C Polymorphisms in a Patient with Pediatric Leukemia and the Effect of Steroid Therapy. Turk J Hematol. 2014; 31(4): 399-402

Corresponding Author: Burcu Fatma Belen, Türkiye


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