β-THALASSEMIA MAJOR

Background: Â-thalassemia is a common single genetic disorder in Pakistanwith about 8% gene frequency and roughly 10 million carriers. Growth impairment leadingto short stature in thalassemic patients is an important cause of morbidity. Objectives: Todetermine the frequency of short stature in children with muti-transfused â-Thalassemia major.Study Design: Descriptive cross sectional study. Place and Duration of Study: PediatricDepartment, Allied Hospital, Faisalabad from December 2015 to May 2016. Patient & Methods:Ninety multi-transfused â-thalassemia major patients diagnosed by hemoglobin electrophoresisbetween 06 to 10 years of age of either gender were included. Patients with â-thalassemiamajor with a concomitant chronic illness like congenital heart disease, tuberculosis, celiacdisease and immunodeficiency and those with familial short stature as determined by historyand mid-parental height were excluded. Results: Out of 90 cases, 56.67% (n=51) werebetween 6-8 years of age while 43.33% (n=39) between 9-10 years of age, mean + SD was7.85+1.50 years, 51.11%(n=46) male and 48.89%(n=44) were females. Frequency of shortstature in children with â-thalassemia major receiving multiple transfusion was 41.11% (n=37)while 58.89% (n=53) had normal stature. Conclusion: The frequency of short stature is highamong â-thalassemic multi-transfused children. It is recommended that every patient withâ-Thalassemia major, should be sort out for short stature. However, surveillance of growth anddevelopment in these patients is important.

THE RISK FACTOR OF ALLOANTIBODY FORMATION IN THALASSEMIA PATIENTS RECEIVING MULTIPLE TRANSFUSION (Faktor Kebahayaan Terbentuknya Aloantibodi pada Pasien Talasemia yang Menerima Transfusi Darah Berulang)

Untuk kelangsungan hidup pasien talasemia intermediet dan mayor, memerlukan transfusi darah secara teratur. Transfusi berulangini berpeluang membentuk aloantibodi yang dapat menyebabkan kebahayaan hemolitik. Maka transfusi berulang akan memperberathemolitik karena pada pasien talasemia sudah ada proses tersebut. Tujuan penelitian ini adalah untuk mengetahui berbagai faktorkebahayaan untuk terbentuknya aloantibodi pada pasien talasemia yang mendapat transfusi darah berulang khusus di RSUP Fatmawati,Jakarta. Cara meneliti ini menggunakan rancangan potong lintang. Subjek penelitian adalah semua pasien talasemia yang mendapattransfusi darah berulang di RSUP Fatmawati Jakarta yang memenuhi patokan kesertaan. Sebanyak 81 subjek diikutkan dalam penelitianini. Data pada penelitian ini di analisis secara statistik dengan uji Chi Kuadrat. Hasil menguji secara Chi Kuadrat menunjukkan: kelamin,suku, diagnosis, selang transfusi darah, jenis darah, reaksi yang terkait, riwayat keluarga, kadar Hb. Kadar feritin dan golongan darahbukan merupakan faktor kebahayaan untuk terbentuknya aloantibodi, sedang faktor usia, jumlah kantong darah yang ditransfusikan,keberadaan komplikasi akibat transfusi darah dan lama masa waktu menerima darah transfusi, merupakan faktor kebahayaan untukterbentuknya aloantibodi pada pasien talasemia yang mendapat transfusi berulang di RSUP Fatmawati.

Red cell allo- and autoimmunisation in transfused sickle cell and cancer patients in Kenyatta National Hospital, Nairobi, Kenya

Background: Currently, no data are available on the prevalence of red blood cell (RBC) antibody formation amongst Kenyan patients with multiple transfusion needs, such as patients with sickle cell disease (SCD) or haematological malignancies (HM) and solid (SM) malignancies.Objectives: We determined the prevalence and specificities of RBC alloantibodies and autoantibodies in two patient groups with recurrent transfusion demands at Kenyatta National Hospital, Nairobi, Kenya.Method: Between February and August 2014, 300 samples from SCD, HM and SM patients were collected and screened for alloantibodies. Samples from 51 healthy blood donors were screened for irregular antibodies and phenotyped.Results: Amongst the 228 patients with viable samples (SCD, n = 137; HM, n = 48; SM, n = 43), the median transfusion frequency was two to three events per group, 38 (16.7%) were RBC immunised and 32 (14.0%) had a positive direct antiglobulin test. We identified specific alloantibodies in six patients (2.6%). Four of these six were SCD patients (2.9%) who had specific RBC alloantibodies (anti-Cw, anti-M, anti-Cob, anti-S); amongst HM patients one had anti-K and one had anti-Lea. RBC autoantibody prevalence was 3.1% (7/228). Amongst the healthy blood donors, the Ror, ccD.ee and R2r, ccD.Ee phenotypes accounted for 82% of the Rhesus phenotypes and all were Kell negative.Conclusion: The numbers of transfusions and the rates of RBC alloantibodies are low and the most important RBC alloantibody-inducing blood group antigens are relatively homogeneously distributed in this population. A general change in the Kenyatta National Hospital pre-transfusion test regimen is thus not necessary. The current transfusion practice should be reconsidered if transfusion frequencies increase in the future.