UNIVERSITAS AIRLANGGA



Detail Article

Indonesian Journal of Clinical Pathology and Medical Laboratory

ISSN 0854-4263

Vol. 20 / No. 1 / Published : 2013-01

Order : 13, and page :58 - 63

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Original Article :

{(talasemia-β di penderita pengidap leukemia limfoblastik akut (lla)}

Author :

  1. I Dewa Gede Ugrasena*1
  2. Mia Ratwita Andarsini*2
  3. Mangihut Rumiris*3
  4. Maria Christina Shanty Larasati*4
  5. Bambang Permono*5
  1. Dosen Fakultas Kedokteran
  2. Dosen Fakultas Kedokteran
  3. Dosen Fakultas Kedokteran
  4. Mahasiswa Fakultas Kedokteran
  5. Dosen Fakultas Kedokteran

Abstract :

Thalassemias are a heterogeneous group of genetic disorders. β-thalassemia is due to impaired production of beta globin chains, which leads to a relative excess of alpha globin chains. The abnormalities of hemoglobin synthesis are usually inherited but may also arise as a secondary manifestation of another disease, most commonly hematological neoplasm. In this article, two cases of acquired β-thalassemia in children with ALL focusing on the diagnosis and the possible relationship between the two hematological diseases are presented. The first case was a four (4) year old boy with ALL-L1 type at the maintenance phase of chemotherapy, suffering from anemia with Hb 8.0 g/dL, WBC 22.600/mm3 and  platelet count of 200.000/mm3, peripheral blood smear revealed anisocytosis, polychromasia, hypochromia, basophilic stippling, and normoblasts. The result of Hb electrophoresis was as follows,  Hb A 54.9 %, Hb F 29.4 %, Hb E  13.4 % and Hb A2: 2.3 %. The patient was diagnosed as ALL-L1 type and β-Thalassemia. The second case, was a 13 year old girl with ALL-L1 type in remission after chemotherapy, she suffered from anemia with Hb 6.7 g/dL, WBC 12,400/mm3, platelet count  200,000/mm3, and peripheral blood smear showed anisocytosis, hypochromia, normoblasts, myelocytes and basophilic stippling. The results of Hb electrophoresis were:  Hb F 0.41%, Hb A1c 0.78%, Hb A2 2.95% with the conclusion of a β-thalassemia trait, this patient was then diagnosed as  ALL-L1 type remission + β-thalassemia trait. The case reviewers assumed that the acquired β-thalassemia in these patients were due to the altered expression of globin chain. The mechanism of this syndrome might be the acquisition of α  mutation affecting RNA or proteins involved in α-globin gene regulation and resulting in the reduction of the (α/β)-globin biosynthetic ratios, or/and associated with chemotherapy-inducement.     Talasemia adalah kelompok kelainan genetik pembentukan hemoglobin. Di talasemia-β hasilan rantai β terhambat, sehingga menyebabkan rantai α meningkat. Kelainan pembentukan hemoglobin bersifat diturunkan, tetapi dapat muncul sebagai manifestasi sekunder dari beberapa penyakit, pada umumnya berupa keganasan hematologis. Tujuan laporan dua kasus talasemia-β di penderita LLA ini, menitikberatkan hal terkait tata diagnosis dan kemungkinan hubungan kedua kelainan hematologis tersebut. Kasus pertama ialah seorang anak laki-laki berusia 4 tahun dengan LLA-L1 sedang menjalani kemoterapi tingkat rumatan, penderita mengalami anemia dengan Hb 8,0 g/dL, WBC 22.600/mm3, platelet 200.000/mm3, hapusan darah tepi didapatkan anisositosis, polikromasia, hipokromia, basophilic stippling, normoblast. Hasil Hb electrophoresis: Hb A 54,9 %, Hb F 29,4 %, Hb E 13,4 % dan Hb A2 2,3 %. Penderita didiagnosis LLA-L1 dan talasemia-β. Kasus kedua, anak perempuan berusia 13 tahun dengan LLA-L1 remisi pasca kemoterapi, penderita mengalami anemia dengan Hb 6,7 g/dL, WBC 12.400/mm3, platelet 200.000/mm3, hapusan darah tepi didapatkan anisositosis, hipokromia, normoblast, mielosit, basophilic stippling. Hasil Hb elektrophoresis: Hb F 0,41%, Hb A1c 0,78%, Hb A2 2,95%. Penderita didiagnosis LLA-L1 remisi dan talasemia-β trait. Dalam kasus ini dapat didugakan bahwa talasemia-β di penderita LLA terjadi karena perubahan ekspresi rantai globin dengan mekanisme perpindahan RNA atau pengaturan gen globin yang menyebabkan berkurangnya angka banding biosintesis (α/β)-globin, atau dan dihubungkan dengan dipicu kemoterapi yang diberikan.

Keyword :

β-thalassemia, children, acute lymphoblastic leukemia,


References :

Benz JE,(2000) Treatment of Thalassemia New York : Churchill Livingstone





Archive Article

Cover Media Content

Volume : 20 / No. : 1 / Pub. : 2013-01
  1. (lipid Ratio In Myocardial Infarction)
  2. (the Synergic Effect Of Chloroquine And N-acetyl Cysteine In Decreasing Parasitemia And Erythrocyte Malondyaldehyde Level In Balb/c Mice Infected With Plasmodium Berghei)
  3. Serum Albumin In Liver Cirrhosis
  4. Urine 5 Hidroxyindolacetic (5-hiaa) Acid In Colorectal Cancer
  5. Microrganisms And Sensitivity Tests Of Diabetic Foot
  6. Protein Disulfide Isomerase Family A Member 4 (pdia4) In Metastatic Breast Cancer
  7. Apo B / Apo A-i Ratio In Acute Coronary Syndrome
  8. Pneumocystis Pneumonia (pcp) In Hiv And Aids Patients With Pulmonary Symptom
  9. Ckmb Activity And Its Ckmb Mass As Well As Cardiac Troponin-1 In Acute Coronary Syndrome
  10. Platelet Count In Pre Eclampsia Patients
  11. Fusion Of Gen Breakpoint Cluster Region Abelson Kinase (bcr-abl) And Routine Haematological Test
  12. Secondary Iron Overload Related With Transferrin Saturation And Ferritin
  13. {(talasemia-β Di Penderita Pengidap Leukemia Limfoblastik Akut (lla)}
  14. Workload Analysis In Laboratory Installation

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