We report two cases of biphenotypic acute leukaemia diagnosed in Hospital Universiti Kebangsaan Malaysia (HUKM), their clinical, haematological characteristics and response to chemotherapy. Both patients are middle-aged ladies who presented with hepatosplenomegaly and high white cell count, mainly composed of blast cells. Their bone marrow aspirations were hypercellular comprising of more than 90% heterogenous blast cells. Cytochemical analyses show more than 3% positivity towards peroxidase, with smaller blasts showing block positivity towards PAS. Immunophenotypically, the blasts showed dual expression of CD 33 and CD 19, CD 19 and CD34, intra CD22, intra TdT and intraMPO. One of the patients showed presence of the Philadelphia chromosome on cytogenetic analysis which was confirmed by Fluorecsence In Situ Hybridisation (FISH). Molecular analysis also showed presence of the BCR-ABL fusion protein. Both patients were given combination chemotherapy consisting of daunorubicin and cytosine arabinoside.However, the patient with positive Philadelphia chromosome BCR-ABL did not achieve morphological remission after induction chemotherapy. In view of the poor prognosis of this disease, both the patients were planned for upfront peripheral blood stem cell transplantation.
Biphenotypic Acute Leukemia: A Report of Two Cases
Author
Abstrak
Kami melaporkan dua kes leukemia akut bifenotipik yang didiagnos di Hospital Universiti Kebangsaan Malaysia (HUKM), ciri-ciri klinikal, hematologi dan tindakbalas penyakit ini terhadap kemoterapi. Kedua-dua pesakit adalah wanita pertengahan umur yang menunjukkan pembengkakan hati dan limpa serta bilangan sel darah putih yang tinggi. Sum-sum tulang menunjukkan kepadatan sel dimana 90% terdiri dari sel-sel leukemia pelbagai saiz. Analisa sitokimia menunjukkan lebih daripada 3% adalah positif terhadap ujian peroksidase dan sel-sel leukaemia yang lebih kecil menunjukkan positiviti secara blok terhadap ujian periodic acid-schiff (PAS). Ujian imunofenotip menunjukkan ko-ekspresi dwi penanda sel CD 33 dan CD 19, CD 19 dan CD34, intra CD22, intra TdT dan intraMPO. Analisa sitogenetik daripada salah seorang pesakit tersebut menunjukkan kehadiran kromosom Philadelphia yang disahkan dengan ujian Fluorescence In Situ Hybridisation (FISH). Analisa molekular juga menunjukkan kehadiran protein fusi BCR-ABL. Kedua-dua pesakit menerima rawatan kemoterapi yang terdiri daripada daunorubicin dan cytosine arabinoside. Namun begitu, pesakit yang mempunyai kromosom Philadelphia BCR-ABL tidak berjaya mencapai remisi morfologi selepas kemoterapi induksi. Memandangkan prognosis buruk pesakit leukemia ini, pemindahan sel-sel stem periferi dirancangkan untuk kedua-dua pesakit ini.
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Related Images
 table1.jpg "TABLE 1: Laboratory findings in two cases of Biphenotypic Acute Leukemia (BAL)")
 image1.jpg "FIGURE 1: Bone marrow aspiration of case 1 showing a heterogenous population of large (red arrow) and small (blue arrow) blasts cells. (MGG stain x 400)")
 image2.jpg "FIGURE 2: Bone marrow aspiration of case 2 showing a heterogenous population of large (red arrow) and small (blue arrow) blast cells. (MGG stain x 400)")
 image3.jpg "FIGURE 3: Immunophenotyping of case 1 showed co-expression CD 19 and CD33, CD 13 and CD10, HLA DR and CD34, intra22 and intraMPO of gated cells.")
 image4.jpg "FIGURE 4: FISH analysis of case 2 showed presence of BCR-ABL fusion gene")
 image5.jpg "FIGURE 5: Molecular analysis by RT-PCR of case 2 showed presence of major BCR-ABL fragment.")
