Prevalence and outcomes of thrombotic and hemorrhagic complications in pediatric acute promyelocytic leukemia in a tertiary Brazilian center

Disseminated intravascular coagulation (DIC) is characterized by the intravascular activation of coagulation with loss of localization arising from different causes, and is diagnosed using scoring systems which rely upon the presence of an underlying disorder compatible with DIC alongside hemostatic derangements such as low platelet count, prolonged prothrombin time, and elevated fibrinogen degradation products. DIC is common in patients with acute leukemia, with prevalence ranging from 17 to 100% in acute promyelocytic leukemia (APL) and 8.5 to 25% in acute lymphoblastic leukemia (ALL) and non-APL acute myeloid leukemia (AML). The pathophysiology is complex and varies between the leukemia subtypes, and is not fully reflected by the laboratory markers currently used to classify DIC. Similarly, the clinical consequence of DIC in acute leukemia also varies across the types of leukemia. DIC is primarily associated with bleeding in APL, while thrombosis is the dominant phenotype in ALL and non-APL AML. The cornerstone of managing DIC is the treatment of the underlying disease, as exemplified by the important role of early administration of all-trans retinoic acid in APL. Other aspects of management focus on supportive care aimed at minimizing the risk of bleeding, via transfusion of blood products. The use of blood products is more liberal in APL, due to the hemorrhagic phenotype and unacceptably high rates of early hemorrhagic death. This review will focus on the pathophysiology, risk factors, clinical implications, and the management of DIC in patients across the spectrum of acute leukemias.

show abstract

“…Risk factors for thrombosis are leukocytosis, FLT3-ICD mutations, CD2 expression, and CD15 expression. 21,22…”

Section: Pathophysiologymentioning

confidence: 99%

Management of Disseminated Intravascular Coagulation in Acute Leukemias

Cate

Leader

2021

Hamostaseologie

View full text Add to dashboard Cite

show abstract

“…In DIC caused by LM3 the levels of protein C, protein S and, antithrombin III are normal. 1,6 In the initial phase of APL, most patients present with bleeding in the form of bruises, petechiae, epistaxis, and menorrhagia. Hence, the main complication and cause of premature death in clinical trials are hemorrhagic complications in the central nervous system and lungs, mainly during the first 15 days after diagnosis.…”

Section: Thrombosis and Bleeding In Aplmentioning

confidence: 99%

“…To avoid this, it is recommended to provide timely transfusion support, where platelets, fibrinogen and, other coagulation factors are administered prophylactically. 1,2 And as in adults, a high leukocyte count (> 10.0x10 / L) is considered a risk factor for premature death secondary to hemorrhage in pediatric patients. 1 Despite prophylaxis, the rates of premature death from bleeding events remain the same.…”

Section: Thrombosis and Bleeding In Aplmentioning

confidence: 99%

“…1,2 And as in adults, a high leukocyte count (> 10.0x10 / L) is considered a risk factor for premature death secondary to hemorrhage in pediatric patients. 1 Despite prophylaxis, the rates of premature death from bleeding events remain the same. 6 In the study carried out by de Albuquerque-Antunes et al, 1 it was determined that 89% of the patients had hemorrhagic manifestations, mainly mucocutaneous bleeding.…”

Section: Thrombosis and Bleeding In Aplmentioning

confidence: 99%

“…Acute promyelocytic leukemia (APL) causes between 5% and up to 15% of all myelocytic leukemias in pediatric age, although the highest incidence is observed in Latin American countries, such as Brazil, Mexico, Peru, and Venezuela, where it is reported higher than 20%; but in general terms, it is considered rare in children. [1][2][3] Specifically, the morphology corresponds to the M3 or M3v (hypogranular variant) subtype of the Franco-American-British (FAB) classification. 95% of APL cases are characterized by a translocation between chromosome 15 and 17 t (15; 17) (q22; q21) and the fusion between the PML gene and the retinoic acid receptor alpha gene in chromosome 1, giving rise to the formation of the PML-RARa protein.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Update on acute promyelocytic leukemia in the pediatric population

Ruiz-Rodriguez¹,

ObandoMadrigal²,

Mateus-Vargas³

2021

HTIJ

View full text Add to dashboard Cite

Acute promyelocytic leukemia (APL) has shown differences in incidence around the world, it has a characteristic molecular, pathogenic and clinical development mechanism. Despite being a low incidence acute myeloid leukemia (AML) compared to other AML and having a good response to therapy, it continues to be of interest due to the toxicities of its current treatment, to mutations that have shown resistance to those drugs, and to the few possibilities of alternative treatment with all-trans retinoic acid (ATRA) and anthracyclines in the pediatric population. Furthermore, the description of new mutations that originate APL produced by LPA generates new interest in studies that may shed light on their effects on the disease’s incidence, prognoses, and response to treatment. This article aims to review different studies that describe findings of APL in terms of incidences, treatments, side effects, survival, especially in the pediatric population. Different studies propose the use of ATRA - arsenic trioxide (ATO) as the first line of treatment, however, few have been carried out in the pediatric population and the use of ATO is not recommended in children under 5 years of age. On the other hand, the differentiation syndrome (DS) has been d

show abstract

Idarubicin/tretinoin

2021

Reactions Weekly

View full text Add to dashboard Cite

Prevalence and outcomes of thrombotic and hemorrhagic complications in pediatric acute promyelocytic leukemia in a tertiary Brazilian center

Cited by 7 publications

References 16 publications

Management of Disseminated Intravascular Coagulation in Acute Leukemias

Management of Disseminated Intravascular Coagulation in Acute Leukemias

Update on acute promyelocytic leukemia in the pediatric population

Idarubicin/tretinoin

Contact Info

Product

Resources

About