Acute Megakaryoblastic Leukemia in Children: Diagnosis and Management Challenges in Resource-Poor Countries

Abstract

Acute Megakaryoblastic Leukemia (AMKL) is a rare subtype of acute myeloid leukemia classified as AML M7 by the French-American-British (FAB) Leukemia Cooperative Study Group. We reviewed the records of children aged less than 18 years with AMKL during the period from January 2003 to December 2010. The diagnosis of AMKL was established on the basis of the FAB criteria and confirmed by immunophenotyping. Eight cases were assigned for this period. The incidence rate of AMKL was 5.5 % of AML. The median age at the time of diagnosis was 1 year. The karyotype presented translocation (1, 22) in two cases. One girl had Down syndrome (DS). Treatment included two inductions based on daunorubicin and cytosine arabinoside (7+3) followed by two consolidations with high dose of cytosine arabinoside (16 g/m 2 ). Complete remission (CR) was achieved in 3 patients (43%). One patient underwent allogeneic stem cell transplant (SCT) and is still enjoying continuous CR 5 years after treatment. Other patients died from failure or relapse. In this small series from a single institution, reported incidence of AMKL is underestimated probably due to the difficulties of diagnosis. The prognosis was poor, particularly because of unavailability of intensified chemotherapy treatment and allogeneic SCT.

Outcome of pediatric acute myeloid leukemia (AML) in low- and middle-income countries: a systematic review of the literature

“…Relapse rates varied from 10% to 83% (n=15 studies), but most relapse rates were between roughly 30% and 45% [20,21,30,[37][38][39][40]42,44,45]. The highest reported relapse rate was 83%, however the majority of patients in this study had an etoposide-omitted and cytarabine-reduced induction regimen, or received a palliative regimen [29].…”

“…The majority of the studies were retrospective cohort studies (n=26), either single center (n=23) or multicenter (n=3) [42][43][44], whereas only one study had a prospective design [45]. One study included only non-DS AMKL patients [31], and one study reported on non-DS AMKL, DS-AMKL, and non-AMKL patients [21].…”

“…AMKL patients were evaluated in two studies with considerably different sample sizes (80 non-DS AMKL patients [31] vs. 7 non-DS AMKL patients and 1 DS-AMKL patient [21]). In the smaller study [21], the CR rate was low (43%), the ED rate was high (57%), two-third of the patients relapsed, and only one patient was alive at 5 years after diagnosis.…”

“…AMKL patients were evaluated in two studies with considerably different sample sizes (80 non-DS AMKL patients [31] vs. 7 non-DS AMKL patients and 1 DS-AMKL patient [21]). In the smaller study [21], the CR rate was low (43%), the ED rate was high (57%), two-third of the patients relapsed, and only one patient was alive at 5 years after diagnosis. In the other study [31], the CR rate was relatively high (71%), the ED and TRM rates were high (20% and 14%, respectively), one-third of the patients relapsed, and the 2-year pEFS and pOS were 45% and 53%, respectively.…”

SALL4 Gene Expression in Acute Myeloid Leukemia

“…Cumulative Overall Survival of AML Patients Included in the Study leukemia is 10 times more prevalent in adults than in children, it accounts for more than 30% of all childhood cancers. Compared to other types of leukemia, AML is the most deadly (Cherkaoui et al, 2014). The NCI Cancer Registry presented the cancer incidence rates at national and regional level of Egypt in the period of time between 2008 and 2011 based upon results of National Cancer Registry Program (NCRP).…”