acute promyelocytic leukaemia

(redirected from acute progranulocytic 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.
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