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 ?
Kirsten Hawksey, who "idolised" her 15-month-old daughter, Penelope, was diagnosed with acute promyelocytic leukaemia on November 27.
Advances in therapies for acute promyelocytic leukaemia.
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".
Maths teacher Paul McGeary was diagnosed with relapsed blood cancer, Acute Promyelocytic Leukaemia (APML), earlier this year, having been in remission from an initial diagnosis in November 2014.
Fusion between a novel Krnppel-like zinc finger gene and the retinoic acid receptor-alpha locus due to a variant t(11; 17) translocation associated with acute promyelocytic leukaemia.
The product is marketed in Japan for acute promyelocytic leukaemia, a form of AML characterised by a different genomic alteration of RARA (gene encoding RARa).
Acute promyelocytic leukaemia, APL, is probably one of the best-understood malignancies and the only one where highly efficient oncogene-targeted therapies (retinoic acid and arsenic) actually cure the disease.
It was set up by Melissa Ginns and Ian Mustard following the loss of Ian's 16-year-old daughter Abbie from acute promyelocytic leukaemia in 2013.
On November 4 she was diagnosed with acute promyelocytic leukaemia, became unconscious just 12 hours later and died soon after.
Funded by the charity Leukaemia & Lymphoma Research, the scientists have spent five years studying data from 231 people diagnosed with acute promyelocytic leukaemia (APL).
Doctors diagnosed him as having acute promyelocytic leukaemia, which is a rare form of the disease and stops blood clotting.

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