Congenital acute myeloid leukemia: A rare diagnostic enigma case report with review of literature

Abstract

Recently, few studies have shown that imatinib induces tumor regression in patients with unresectable or advanced PVNS. [12] Imatinib is a tyrosine kinase inhibitor which blocks macrophage colony stimulating factor receptors, which is expressed in high levels in most of mononuclear and multinucleated stromal cells and are thought to be responsible for tumor formation. [13] The use of imatinib in this indication is based on the observation of the presence of a t(1;2) translocation in bulk of the tumors, leading to the over expression of active colony-stimulating factor 1, which attracts inflammatory cells that dominate in PVNS. [12,14] No prospective studies on use of imatinib in PVNS are available as of now. [15] Financial support and sponsorship Nil.

Extreme Hyperleukocytosis in an Extremely Preterm Infant

“…Incidence is reported to be 1 in 5 million [8]. The doubling time of leukemic cells leads to clinically evident disease after birth or shortly after that [8]. No elevated blasts were found in our patient's blood smear and, in addition to the normal chromosomal test, prompted us to rule out CL and TMD.…”

“…CL is extremely rare, usually diagnosed at birth or within 1month of life. Incidence is reported to be 1 in 5 million [8]. The doubling time of leukemic cells leads to clinically evident disease after birth or shortly after that [8].…”

Lactante menor con leucemia congénita y enfermedad de injerto contra huésped postrasplante

“…La mayoría de los casos de leucemia neonatal y congénita son de origen mieloide, comparado con el resto de las leucemias en edad pediátrica, donde predomina la estirpe linfoide. 7,8 El protocolo incluye quimioterapia intensiva multiagente y tratamiento de soporte. El trasplante de progenitores hematopoyéticos es una opción adicional en pacientes de alto riesgo; 9 en la paciente de este estudio la carga tumoral al diagnóstico (más de 50,000 leucocitos/mm 3 ), edad a la manifestación, ausencia de traslocaciones de pronóstico favorable [t(8;21), inv(16) o t(16;16)] y reacción deficiente al primer ciclo de quimioterapia 10 se asociaron con factores de alto riesgo de falla al tratamiento o recidiva hematológica; por tanto, en estos casos debe mejorarse el enfoque del trasplante, pues provocaría un desenlace sombrío después de haber recibido quimioterapia convencional de inducción y consolidación.…”