Hemoglobin H Disease in Turkey: Experience from Eight Centers

Bu çalışmada, ülkemizdeki hemoglobin H (HbH) hastalığı sorununu irdelemek, bölgelere göre dağılımını tespit etmek ve sonuç olarak HbH hastalığının ülkemiz için önemini vurgulamak amaçlandı. Sekiz hemoglobinopati merkezinden toplam 273 hasta çalışmaya dahil edildi. Merkezler arasında en fazla bildirimi 232 hasta ile Antakya Hemoglobinopati Merkezi sağladı. Kalan 7 merkezden toplam 41 HbH hastası bildirildi. Eş zamanlı olarak PubMed'den Türkiye'den yayınlanan ya da Türk HbH hastalarının dahil edildiği makaleler araştırıldı ve sonuçta 198 HbH hastasını içeren toplam 16 yayına ulaşıldı. Bu çalışma, ülkemizde HbH hastalığı sorununu araştıran bir ön çalışmadır. Çalışmamıza Antakya'dan oldukça fazla hasta bildiriminin olması HbH hastalığı yönünden dikkatimizi bu bölgeye yöneltmemize neden olacaktır. Sağlık Bakanlığı önderliğinde oluşturulacak kayıt sistemleri ile tüm HbH'li hastalar kayıt altına alınmalı ve böylece HbH hastalığının ülkemiz için önemi vurgulanmalıdır.

Anahtar Kelime:

Konular: Hematoloji

Türkiye'de Hemoglobin H Hastalığı: Sekiz Merkezin Deneyimi

Öz:

The purpose of this study was to research the problem of hemoglobin H (HbH) disease, to reveal the distribution patterns among different health centers, and to emphasize the importance of this disease for Turkey. A total of 273 patients were included from 8 hemoglobinopathy centers. The Antakya Hemoglobinopathy Center reported 232 patients and the remaining 7 centers reported 41 patients. PubMed was also searched for published articles related to Turkish patients with HbH disease, and we found 16 articles involving a total of 198 HbH patients. Most of the patients were reported from Antakya; thus, special attention should be paid to this region. This is a preliminary study to investigate the extent of the problem of HbH disease and it emphasizes the need for hematology associations or the Ministry of Health to record all cases of HbH disease in Turkey.

Anahtar Kelime:

Konular: Hematoloji

Belge Türü: Makale Makale Türü: Diğer Erişim Türü: Erişime Açık

Chui DH, Fucharoen S, Chan V. Hemoglobin H disease: not necessarily a benign disorder. Blood 2003;103:791-800.
Steinberg MH, Forget BG, Higgs DR, Weatherall DJ. Disorders of Hemoglobin. 2nd ed. Cambridge, Cambridge University, 2009.
Kulaç İ, Ünal Ş, Gümrük F. Brilliant cresyl blue staining for screening hemoglobin H disease: reticulocyte smear. Turk J Hematol 2009;26:45.
Canatan D, Oğuz N, Güvendik İ, Yıldırım S. The incidence of alpha- thalassemia in Antalya-Turkey. Turk J Haematol 2002;19:433-434.
Ozsoylu S, Malik SA. Incidence of alpha thalassemia in Turkey. Turk J Pediatr 1982;24:235-244.
Fei YJ, Kutlar F, Harris HF, Wilson MM, Milana A, Sciacca P, Schiliro G, Masala B, Manca L, Altay C. A search for anomalies in the zeta, alpha, beta, and gamma globin gene arrangements in normal black, Italian, Turkish, and Spanish newborns. Hemoglobin 1989;13:45-65.
Arcasoy A. Türkiye'de Thalassemia Taşıyıcı Sıklığı. Ankara, Turkey: Ankara Thalassemia Derneği, 1991 (in Turkish).
Kılınç Y. Hemoglobinopathies in Turkey. Turk J Hematol 2006;23:214-216.
Polat G, Yuregir GT, Aksoy K. Detection of deletional alpha thalassemia in Cukurova. Ann Med Sci 1998;7:14-17.
Celik MM, Gunesacar R, Oktay G, Duran GG, Kaya H. Spectrum of ?-thalassemia mutations including first observation of (FIL) deletion in Hatay Province, Turkey. Blood Cells Mol Dis 2013;51:27-30.
Aldemir Ö, İzmirli M. Hataydaki ?-talasemi genotipleri ve ?-talasemi genotip frekansı. Abant Med J 2014;3:233-236 (in Turkish).
Kılınç Y, Kümi M, Gürgey A, Altay Ç. Adana bölgesinde doğan bebeklerde kordon kanı çalısması ile alfa talasemi, G6PD enzim eksikliği ve HbS sıklığının araştırılması. Doğa Tıp ve Ecz Der 1986;10:162 (in Turkish).
Aksoy M, Erdem S. Haemoglobin H disease study of an Eti-Turk family. Acta Genet Stat Med 1968;18:12-22.
Ozsoylu S, Isik K. Haemoglobin H disease in a Turkish family. Scand J Haematol 1973;10:54-58.
Orkin SH, Old J, Lazarus H, Altay C, Gurgey A, Weatherall DJ, Nathan DG. The molecular basis of alpha-thalassemias: frequent occurrence of dysfunctional alpha loci among non-Asians with Hb H disease. Cell 1979;17:33-42.
Altay C, Gurgey A, Tuncbilek E. Hematological evaluation of patients with various combinations of alpha-thalassemia. Am J Hematol 1980;9:261-267.
Aksoy M, Kutlar A, Kutlar F, Harano T, Chen SS, Huisman TH. Hemoglobin H disease in two Turkish females and one Iranian newborn. Hemoglobin 1985;9:373-384.
Kutlar F, Gonzalez-Redondo JM, Kutlar A, Gurgey A, Altay C, Efremov GD, Kleman K, Huisman TH. The levels of zeta, gamma, and delta chains in patients with Hb H disease. Hum Genet 1989;82:179-186.
Fei YJ, Oner R, Bozkurt G, Gu LH, Altay C, Gurgey A, Fattoum S, Baysal E, Huisman TH. Hb H disease caused by a homozygosity for the AATAAA-- >AATAAG mutation in the polyadenylation site of the alpha 2-globin gene: hematological observations. Acta Haematol 1992;88:82-85.
Yüregir GT, Aksoy K, Çürük MA, Dikmen N, Fei YJ, Baysal E, Huisman TH. Hb H disease in a Turkish family resulting from the interaction of a deletional ?-thalassemia-1 and a newly discovered poly A mutation. Br J Haematol 1992;80:527-532.
Cürük MA, Dimovski AJ, Baysal E, Gu LH, Kutlar F, Molchanova TP, Webber BB, Altay C, Gürgey A, Huisman TH. Hb Adana or ?2(59)(E8)Gly-->Asp?2, a severely unstable ?1-globin variant, observed in combination with the -(?)20.5 KB?-thal-1 deletion in two Turkish patients. Am J Hematol 1993;44:270-275.
Oner C, Gürgey A, Oner R, Balkan H, Gümrük F, Baysal E, Altay C. The molecular basis of Hb H disease in Turkey. Hemoglobin 1997;21:41-51.
Oner C, Oner R, Birben E, Balkan H, Gümrük F, Gürgey A, Altay C. HB H disease with homozygosity for red cell G6PD deficiency in a Turkish female. Hemoglobin 1998;22;157-160.
Durmaz AA, Akin H, Ekmekci AY, Onay H, Durmaz B, Cogulu O, Aydinok Y, Ozkinay F. A severe ? thalassemia case compound heterozygous for Hb Adana in ?1 gene and 20.5 kb double gene deletion. J Pediatr Hematol Oncol 2009;31:592-594.
Cürük MA. Hb H (?4) disease in Cukurova, southern Turkey. Hemoglobin 2007;31:265-271.
Ünal Ş, Gümrük F. The hematological and molecular spectrum of alpha thalassemias in Turkey: the Hacettepe experience. Turk J Hematol 2015;32:136-143.
Karakas Z, Koc B, Temurhan S, Elgun T, Karaman S, Asker G, Gencay G, Timur Ç, Yıldırmak ZY, Celkan T, Devecioglu Ö, Aydın F. Evaluation of alpha thalassemia mutations in cases with hypochromic microcytic anemia: Istanbul perspective. Turk J Hematol 2015.
Gurgey A, Altay C, Beksaç MS, Bhattacharya R, Kutlar F, Huisman TH. Hydrops fetalis due to homozygosity for alpha-thalassemia-1, -(alpha)-20.5 kb: the first observation in a Turkish family. Acta Haematol 1989;81:169- 171.

APA	ünal s, Oktay G, ACIPAYAM C, ILHAN G, GALİ E, CELKAN T, BAY A, Malbora B, akar n, Oymak Y, TOPTAŞ T (2016). Hemoglobin H Disease in Turkey: Experience from Eight Centers. , 56 - 59.
Chicago	ünal selma,Oktay Gönül,ACIPAYAM Can,ILHAN GÜL,GALİ Edip,CELKAN TÜLIN TIRAJE,BAY Ali,Malbora Baris,akar nejat,Oymak Yeşim,TOPTAŞ Tayfur Hemoglobin H Disease in Turkey: Experience from Eight Centers. (2016): 56 - 59.
MLA	ünal selma,Oktay Gönül,ACIPAYAM Can,ILHAN GÜL,GALİ Edip,CELKAN TÜLIN TIRAJE,BAY Ali,Malbora Baris,akar nejat,Oymak Yeşim,TOPTAŞ Tayfur Hemoglobin H Disease in Turkey: Experience from Eight Centers. , 2016, ss.56 - 59.
AMA	ünal s,Oktay G,ACIPAYAM C,ILHAN G,GALİ E,CELKAN T,BAY A,Malbora B,akar n,Oymak Y,TOPTAŞ T Hemoglobin H Disease in Turkey: Experience from Eight Centers. . 2016; 56 - 59.
Vancouver	ünal s,Oktay G,ACIPAYAM C,ILHAN G,GALİ E,CELKAN T,BAY A,Malbora B,akar n,Oymak Y,TOPTAŞ T Hemoglobin H Disease in Turkey: Experience from Eight Centers. . 2016; 56 - 59.
IEEE	ünal s,Oktay G,ACIPAYAM C,ILHAN G,GALİ E,CELKAN T,BAY A,Malbora B,akar n,Oymak Y,TOPTAŞ T "Hemoglobin H Disease in Turkey: Experience from Eight Centers." , ss.56 - 59, 2016.
ISNAD	ünal, selma vd. "Hemoglobin H Disease in Turkey: Experience from Eight Centers". (2016), 56-59.

APA	ünal s, Oktay G, ACIPAYAM C, ILHAN G, GALİ E, CELKAN T, BAY A, Malbora B, akar n, Oymak Y, TOPTAŞ T (2016). Hemoglobin H Disease in Turkey: Experience from Eight Centers. Turkish Journal of Hematology, 33(1), 56 - 59.
Chicago	ünal selma,Oktay Gönül,ACIPAYAM Can,ILHAN GÜL,GALİ Edip,CELKAN TÜLIN TIRAJE,BAY Ali,Malbora Baris,akar nejat,Oymak Yeşim,TOPTAŞ Tayfur Hemoglobin H Disease in Turkey: Experience from Eight Centers. Turkish Journal of Hematology 33, no.1 (2016): 56 - 59.
MLA	ünal selma,Oktay Gönül,ACIPAYAM Can,ILHAN GÜL,GALİ Edip,CELKAN TÜLIN TIRAJE,BAY Ali,Malbora Baris,akar nejat,Oymak Yeşim,TOPTAŞ Tayfur Hemoglobin H Disease in Turkey: Experience from Eight Centers. Turkish Journal of Hematology, vol.33, no.1, 2016, ss.56 - 59.
AMA	ünal s,Oktay G,ACIPAYAM C,ILHAN G,GALİ E,CELKAN T,BAY A,Malbora B,akar n,Oymak Y,TOPTAŞ T Hemoglobin H Disease in Turkey: Experience from Eight Centers. Turkish Journal of Hematology. 2016; 33(1): 56 - 59.
Vancouver	ünal s,Oktay G,ACIPAYAM C,ILHAN G,GALİ E,CELKAN T,BAY A,Malbora B,akar n,Oymak Y,TOPTAŞ T Hemoglobin H Disease in Turkey: Experience from Eight Centers. Turkish Journal of Hematology. 2016; 33(1): 56 - 59.
IEEE	ünal s,Oktay G,ACIPAYAM C,ILHAN G,GALİ E,CELKAN T,BAY A,Malbora B,akar n,Oymak Y,TOPTAŞ T "Hemoglobin H Disease in Turkey: Experience from Eight Centers." Turkish Journal of Hematology, 33, ss.56 - 59, 2016.
ISNAD	ünal, selma vd. "Hemoglobin H Disease in Turkey: Experience from Eight Centers". Turkish Journal of Hematology 33/1 (2016), 56-59.