University of Khartoum

Clinical and Hematological Characteristics of Acute Promyelocytic Leukaemia and Its Outcome

Clinical and Hematological Characteristics of Acute Promyelocytic Leukaemia and Its Outcome

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Title: Clinical and Hematological Characteristics of Acute Promyelocytic Leukaemia and Its Outcome
Author: Hassan, Nusiba Mohammed Abd Allah
Abstract: Background: Acute Promyelocytic Leukaemia (APL) is a distinct type of Acute Myeloid Leukaemia, classified as M3 by French-American-British classification (FAB), and leukemia with recurrent chromosomal abnormality by World Health Organization (WHO). It has unique clinical and morphological characteristics, and a unique cytogenetic abnormality t(15,17), which make patients respond to All-Trans-Retinoic Acid therapy (ATRA). The aim was to study the clinical, hematological, molecular and immunophenotypic characteristics of APL , to classify these patients into risk groups according to WBCS and platelets count (Sanz score ), and to determine the outcome of this disease in correlation with risk stratification . Materials and Methods: Retro prospective, descriptive hospital based study was carried on fifty seven cases of APL at Radioisotope Center of Khartoum (RICK) over a period of two and a half years. Patients data was collected from laboratory records and patients files, some data was completed by phone through designed questionnaire, complete blood count, bone marrow and molecular tests were reviewed. Molecular test was done by RT-PCR, coagulation tests for only 25 patients, flowcytometery was done for 18 patients. The outcome and causes of death were collected from their hospital medical records. Results: The mean age was 27 years ± 2.5 years , two peaks of age at 12 years and 25 years were observed with slight female predominance (ratio was 1:1.4), one third of patients came from western states. Fever and bleeding were the main presenting symptoms accounting for 68.4% and 66.7% respectively. Splenomegaly, hepatomegaly and lymphoadenopathy were rare presenting sign . The mean WBCS was 19.5×109/L ± 2.5×109/L, HB 7.4g/dl ± 0.3g/dl and platelets were 33.5×109/L ± 4.07×109/L, and all patients showed abnormal promyelocytes with Sudan Black B positivity and depression of hematopoietic activity in bone marrow material. Morphologically 53 patients had hypergranular and 3 had hypogranular promyelocytic leukemia.INR was high in 57% of patients . flowcytometeric finding include high side scatter and high expression of myeloperoxidase, CD33 and CD13 and negative expression of CD34 and HLA. PML-RARA fusion gene was positive in 90% of patients . Almost half of patients were in the high risk group. More than half of patients died, mainly during induction phase due to hemorrhage . Most of the early deaths occurring in the high and intermediate risk groups indicating direct correlations (P -value = 0.018). Conclusion: APL is a serious type of leukemia carrying high mortality and morbidity, it has a distinct clinical, morphological and molecular finding that can be used for early diagnosis, and high mortality rate correlated with high risk groups. Death occur mainly during the induction due to hemorrhage which necessitates good management.
Description: 98 Pages
URI: http://khartoumspace.uofk.edu/123456789/25395


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