acute promyelocytic leukaemia

acute promyelocytic leukaemia

A subtype of leukaemia that comprises 10% of AML, which has a poor prognosis in children. It is characterised by a retinoic acid receptor alpha translocation (RARA) (t(15;17) and PML-RARA gene rearrangement) and is uniquely responsive to all trans retinoic acid (ATRA), which drives the leukaemic cells to maturation.

Clinical findings
Fatigue, weakness and dyspnea due to anaemia; bruisability and bleeding due to low platelets; fever, infections due to leukopaenia, DIC, and splenomegaly.
 
Management
Induction with ATRA, followed by conventional chemotherapy, commonly an anthracycline plus cytarabine.

a·cute pro·my·e·lo·cyt·ic leu·ke·mi·a

(ă-kyūt' prō'mī-ĕ-lō-sit'ik lū-kē'mē-ă)
The disorder presenting as a severe bleeding disorder, with infiltration of the bone marrow by abnormal promyelocytes and myelocytes, low plasma fibrinogen, and defective coagulation.
Synonym(s): acute promyelocytic leukaemia.

a·cute pro·my·e·lo·cyt·ic leu·ke·mi·a

(ă-kyūt' prō'mī-ĕ-lō-sit'ik lū-kē'mē-ă)
Leukemia as a severe bleeding disorder, with infiltration of the bone marrow by abnormal promyelocytes and myelocytes, a low plasma fibrinogen level, and defective coagulation.
Synonym(s): acute promyelocytic leukaemia.
References in periodicals archive ?
Acute Promyelocytic Leukaemia (APL) is an acute leukaemia characterised by rearrangements involving the PML and RARA loci.
Hana was diagnosed with relapsed acute promyelocytic leukaemia, M3, last year at the SKMC after being initially taken to Al Mafraq hospital on May 23, 2017, for bruises over the face and extremities.
Another form of leukaemia known as acute promyelocytic leukaemia (APL) was once a fatal diagnosis.
Flow cytometry was done on peripheral blood/bone marrow aspirate of the patients suspected to have Acute Promyelocytic Leukaemia. 2-4 ml of venous blood was collected in EDTA.
The product is indicated for induction of remission and consolidation in patients with Acute Promyelocytic Leukaemia (APL) who are refractory to, or have relapsed from, retinoid and anthracycline chemotherapy, and whose APL is characterised by the presence of the t(15;17) translocation or PML/RAR-alpha gene expression.
The t(15;17) translocation of acute promyelocytic leukaemia fuses the retinoic acid receptor alpha gene to a novel transcribed locus.
Ribeiro, "How I treat children and adolescents with acute promyelocytic leukaemia," British Journal of Haematology, vol.
Kirsten Hawksey, who "idolised" her 15-month-old daughter, Penelope, was diagnosed with acute promyelocytic leukaemia on November 27.
Acute Promyelocytic Leukaemia (APL) and Translocation (15;17) in APL
The cause of death was given as asphyxiation, due to a respiratory tract haemorrhage, methotrexate (the oral chemotherapy drug) toxicity, renal failure, acute promyelocytic leukaemia (APL) Coroner Anita Bhardwaj recorded a narrative verdict, ruling the "administering of methotrexate had caused Robyn's death".
Grimwade, "Acute promyelocytic leukaemia with t(11;17)(q23;q12-21) and a good initial response to prolonged ATRA and combination chemotherapy," British Journal of Haematology, vol.

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