CD34 positive-microgranular variant of acute promyelocytic leukemia in a child

Abstract

Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia (AML) in which abnormal promyelocytes predominate. APL is rare in children (approximately 10% of childhood AML) and is characterized by a higher incidence of hyperleukocytosis, an increased incidence of microgranular morphology, the presence of balanced t(15;17)(q22;q11.2-12) translocation, and more frequent occurrence of the PML-RARα isoforms bcr 2 and bcr 3 compared to adults. The cytomorphology of microgranular variant blasts is obviously different from AML M3 blasts; these cells have a nongranular or hypogranular cytoplasm or contain fine dust-like cytoplasmic azurophil granules that may not be apparent by light microscopy. This case report emphasizes the importance of a high index of suspicion for the diagnosis of APL, the hypogranular variant in particular. They are responsive to differentiation therapy with all trans-retinoic acid and complete remission in seen in >80% cases.

A Leucemia Mieloide Aguda Poderia ter se Apresentado Ainda Pior? “Causa Incomum de Trombose Multiarterial Concomitante”

“…No entanto, em casos raros, a LPA pode apresentar um forte imunofenótipo positivo para CD34. 4 Por outro lado, em casos de forte positividade para CD34, os subtipos de LMA não-LPA devem ser considerados no diagnóstico diferencial. Em nosso paciente com múltiplas tromboses catastróficas, CID não foi considerado como FIB, aPTT, TP e INR foram todos considerados normais.…”

“…However, in rare cases, APL can present a strong CD34 positive immunophenotype. 4 On the other hand, in cases of strong CD34 positivity, non-APL AML subtypes should be considered in the differential diagnosis. In our patient with multiple catastrophic thromboses, DIC was not considered as FIB, aPTT, PT, and INR were all found to be normal.…”

Co existence of acute promyelocytic leukemia and autoimmune haemolytic anaemia in a girl – A diagnostic and therapeutic challenge

“…APL is a rare leukemia in pediatric age group representing 10% of all APML [6]. It is associated with PML-RARα or its variants.…”