Acquired ß-Thalassemia in Children With Acute Lymphoblastic Leukemia (ALL)  

Oleh:

Ugrasena, I Dewa Gede ; Andarsini, Mia Ratwita ; Rumiris, Mangihut ; Larasati, Maria Christina Shanty ; Permono, Bambang

Jenis: Article from Journal - ilmiah nasional - terakreditasi DIKTI
Dalam koleksi: Indonesian Journal of Clinical Pathology and Medical Laboratory vol. 20 no. 01 (Nov. 2013), page 58-63.
Topik: ß-thalassemia; children; acute lymphoblastic leukemia;

Ketersediaan

Perpustakaan FK Nomor Panggil: I01.K Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

Thalassemias are a heterogeneous group of genetic disorders. ß-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 ß-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 ß-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 ß-thalassemia trait, this patient was then diagnosed as ALL-L1 type remission + ß-thalassemia trait. The case reviewers assumed that the acquired ß-thalassemia in these patients were due to the altered expression of globin chain. The mechanism of this syndrome might be the acquisition of a mutation affecting RNA or proteins involved in a-globin gene regulation and resulting in the reduction of the (a/ß)-globin biosynthetic ratios, or/and associated with chemotherapy-inducement.

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