Megakaryoblastic Leukemia and Down's Syndrome: A Review
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Abstract:
Megakaryoblastic leukemia and transient leukemia in Down's syndrome have been reviewed using case reports from the literature and our own experience at the Hospital for Sick Children. The following conclusions have been reached: (1) approximately 20% of leukemia (excluding transient leukemia) in Down's syndrome is acute megakaryoblastic leukemia; (2) approximately 20% of all leukemia in Down's syndrome is transient leukemia; (3) transient leukemia in Down's syndrome is acute megakaryoblastic leukemia; (4) recurrence of acute megakaryoblastic leukemia occurs in 20% of the cases of transient leukemia; and (5) the incidence of acute megakaryoblastic leukemia in Down's syndrome is estimated to be 400 times that in normal children. These observations suggest that a specific form of leukemia, namely acute megakaryoblastic leukemia, has a remarkable association with Down's syndrome.Keywords:
Acute megakaryoblastic leukemia
Chronic leukemia
Objective: The literatures on the treatment of acute leukemia and chronic leukemia with Chinese medicine were collected from CNKI dated from January 1979 to April 2012.The syndrome types,prescriptions and drugs were summarized through frequency analysis so as to probe into the laws of the differentiation of syndrome and treatment of the disease in Chinese medicine.Methods: The types of syndromes,formulae and drugs were collected,standardized and made out statistics to observe the using frequency,percentage and category.Chi-square test was applied to compare the drug-using differences between acute leukemia and chronic leukemia.Results: There were 28 syndrome types in acute leukemia and 21 in chronic leukemia,and the exuberant of toxic-heat,insufficiency of qi and blood and deficiency of qi and yin were common syndromes in both leukemia.Syndrome factor analysis showed that fire,heat,blood stasis and internal phlegm was the common excessive pathogenesis in both leukemia;The deficiency of Qi,Yin,Blood and Yang was the common deficient pathogenesis;leukemia mainly involved three viscera including the spleen,kidney and liver.There were 354 prescriptions in acute leukemia and 229 in chronic leukemia collected,among which,220 and 90 were set prescriptions,134 and 139 were prescriptions formed by self-experience respectively in acute leukemia and chronic leukemia.The formulae of tonifying,heat-clearing and treating blood disorders were used more in set prescriptions in both acute leukemia and chronic leukemia.There were totally 264 and 246 kinds of drugs respectively applied in acute and chronic leukemia,classified into 42 category and further combined into 19 category in which tonic drug,removing blood stasis and heat-clearing drugs were used mostly in both leukemia.Chi-square test results showed that hemostatic and dampness-clearing drugs were more used in acute leukemia than in chronic leukemia,while activating blood circulation was more used in chronic leukemia than in acute leukemia(P0.05).Conclusion: The analysis on the syndromes types,the characteristics of formulae and drugs for leukemia in Chinese medicine provided the references for the syndrome differentiation and treatment at present.
Chronic leukemia
Pathogenesis
Blood stasis
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Megakaryoblastic leukemia and transient leukemia in Down's syndrome have been reviewed using case reports from the literature and our own experience at the Hospital for Sick Children. The following conclusions have been reached: (1) approximately 20% of leukemia (excluding transient leukemia) in Down's syndrome is acute megakaryoblastic leukemia; (2) approximately 20% of all leukemia in Down's syndrome is transient leukemia; (3) transient leukemia in Down's syndrome is acute megakaryoblastic leukemia; (4) recurrence of acute megakaryoblastic leukemia occurs in 20% of the cases of transient leukemia; and (5) the incidence of acute megakaryoblastic leukemia in Down's syndrome is estimated to be 400 times that in normal children. These observations suggest that a specific form of leukemia, namely acute megakaryoblastic leukemia, has a remarkable association with Down's syndrome.
Acute megakaryoblastic leukemia
Chronic leukemia
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Acute megakaryoblastic leukemia
Trisomy
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Children with Down syndrome (DS) have a marked increase in susceptibility to Acute Megakaryoblastic Leukaemia (DS-AMKL) and the closely linked neonatal preleukaemic syndrome, Transient Myeloproliferative Disorder (DS-TMD). The distinct stages of DS-TMD and DS-AMKL provide an excellent tractable model to study leukaemogenesis. This review focuses on recent studies describing clinical, haematological and biological features of DS-AMKL and DS-TMD. The findings from these studies suggest that mutations in the key haemopoietic regulator GATA1 (GATA binding protein 1) in DS-AMKL and DS-TMD may be useful in diagnosis and assessing minimal residual disease. These findings raise the possibility of population-based screening strategies for DS-TMD and the development of treatment to eliminate the preleukaemic TMD clone to prevent DS-AMKL. Advances in our understanding of perturbed haemopoiesis in DS, the role of GATA1 and of cooperating mutations are also discussed. These findings have implications for leukaemia biology more broadly given the frequency of acquired trisomy in other human leukaemias.
Acute megakaryoblastic leukemia
GATA1
Trisomy
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AbstractA case is presented of a child with mosaic Down syndrome, who presented at birth with a transient leukemia and later progressed to megakaryoblastic leukemia. Evidence is presented that both leukemias were of megakaryoblastic lineage and evolved from a trisomic hematopoietic precursor. This case is unique in the poor course of the initial transient neonatal leukemia with improvement following chemotherapy. It also highlights the form of leukemia and associated myelodysplasia that occurs in children with Down syndrome. This form of leukemia and transient leukemia are interrelated and are unique to children with Down syndrome.Key Words: Down syndrometransient leukemiamegakaryoblastic leukemiamosaicism
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Acute megakaryoblastic leukemia
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Children with Down syndrome (DS) have a 10- to 20-fold increased risk of developing acute leukemia. An estimated 10% of newborns with DS develop Transient Myeloproliferative Disease (TMD) or Transient Leukemia (TL), a clonal accumulation of megakaryoblasts that resolves spontaneously within months. Acute megakaryoblastic leukemia (AMKL) develops in approximately 20% of cases of TMD/TL by 4 years of age. Both the blasts of AMKL and TMD/TL in DS harbor somatic mutations of GATA1, an essential transcriptional regulator of megakaryocytic differentiation. The distinct phenotypes of megakaryoblastic leukemia in DS are a unique biological model of the incremental process of leukemic transformation. Pediatr Blood Cancer 2007;49:1066–1069. © 2007 Wiley-Liss, Inc.
Acute megakaryoblastic leukemia
GATA1
Blood cancer
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