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A rare case of acute lymphoblastic leukaemia with hemophilia A

DOI: 10.1186/1824-7288-35-40

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Abstract:

Acute lympphoblastic leukaemia accounts for around 77% of childhood leukemia. Hemophilia is a congenital bleeding disorder characterized by deficiency of coagulation factor VIII or IX out of which 85% is due to factor VIII deficiency and 15% due to factor IX deficiency. The coincidence of these two diseases together is rare which has led to challenges in developing treatment strategies.12 yr old boy known case of hemophilia (factor VIII deficiency) diagnosed at 6 month of age presented with intermittent fever (on and off) of 2 month without chills and rigors, significant weight loss of 3 kilograms in 1 one month, generalized weakness of 1 month and joint swelling right knee of 7 days duration. The swelling was spontaneous associated with restriction of movements. There was no family history of malignancy or bleeding disorders.On examination, the anthropometric measurements were appropriate for age. There was pallor, petechiae and ecchymoses on his legs, along with gingival bleeding, and haematoma on right knee. Systemic examination revealed hepatosplenomegaly. Peripheral blood counts revealed hemoglobin levels of 7.6 g%, white cell counts of 41 × 109/L (blasts 85%), and platelet counts of 100 × 109/L. After receiving 40 U/kg FVIII concentrates, bone marrow aspiration was performed, which showed lymphoblast of 85%. His immunophenotype consisted of CD3 87%, CD7 99%, CD8 10%, CD5 3%, CD10 98%, CD19 18%, CD22 20% and HLA DR negative The cells were negative with MPO staining. RT-PCR for BCR abl negative. A hepatitis viruses screening test found the patient to be hepatitis B and C negative. HIV serological test was negative and cytogenetic test revealed 46, XY. Coagulation screening showed a prolonged activated partial thromboplastin time (APTT) (test 46 seconds, control 28 seconds), with normal prothrombin time. The ultrasound scan of abdomen revealed hepatosplenomegaly, Echocardiography and Xray chest was normal.After admission to our center, the patient received 30 U/k

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