Successful Treatment of Congenital Acute Myeloid Leukemia (AML-M6) in a Premature Infant

Abstract

Congenital acute myeloid leukemia (AML), and especially AML-M6 is a rare disease with a poor prognosis. Moreover, reports of treatment outcome of congenital AML-M6 in premature infants are not available. We report the first treated case of congenital AML-M6 in a premature girl, who received a full AML protocol. She presented with blueberry-muffin spots, anemia, high white blood cell count, and serious cardiopulmonary distress. Peripheral blood smears showed AML-M6 blasts. After treatment with a sequential low-dose cytarabine after birth and full-dose AML treatment according to the MRC-12 protocol at the age of 2 months, she now is in continuous complete remission for 4 years.

Neonatal leukaemia

“…Overall, central nervous system (CNS) infiltration is reported in around 50% of all cases and manifests with a bulging fontanelle, papilloedema and retinal haemorrhages as well as a reduced level of consciousness (which can also be due to leucostasis associated with hyperleucocytosis). Babies with hyperleucocytosis can also have respiratory distress with hypoxia and acidosis, cardiac failure and renal failure (Weis et al , ; Heikenheimo et al , ; Ferguson et al , ; Van Dongen et al , ).…”

Successful Treatment of Congenital Erythroleukemia With Low-Dose Cytosine Arabinoside

“…This is the first case described of a patient with congenital erythroleukemia who has achieved a durable remission with low‐dose chemotherapy alone. The only other reported survivor achieved hematological remission after two doses of low‐dose Ara‐C, but relapsed at eight weeks of age and subsequently received standard AML therapy . The remaining five reported cases died of disease without receiving cytotoxic therapy, reflecting the need for early initiation of therapy and further highlighted by the successful outcome in our patient.…”

“…Acute myeloid leukemia (AML) occurs more frequently than acute lymphoblastic leukemia during the first four weeks of life, with acute monoblastic leukemia accounting for approximately 50% of cases, followed by acute myelomonocytic leukemia in 20% . Acute erythroleukemia is extremely rare, with six reported cases …”

Prenatal Findings in Congenital Leukemia: A Case Report

“…Leukemic fetuses, especially those without association of Down syndrome, usually require postnatal chemotherapy. Although several reports described successful chemotherapy against leukemia during the early neonatal period [15][16][17][18] , these neonates were in relatively stable conditions before initiating the chemotherapy. Since most of the leukemic fetuses are likely to be already deteriorated in utero, the rescue might be difficult even if the prenatal diagnosis was established and the delivery was expedited before the fetal demise.…”