Medical Archive

Incidence

Peak incidence of 65 years of age.

Etiology

Classification

??(WHO) : blast > 20% (FAB?? 30%? ??)
??? 80% AML / ??? 80% ALL

??????/??????: ?? ??? ??! – AML : 016-821-1517 

WHO 2016 Classification of Myeloid Neoplasms with Germline Predisposition

• Myeloid neoplasms with germline predisposition without a preexisting disorder or organ dysfunction
  • Acute myeloid leukemia with germline CEBPA mutation 
  • Myeloid neoplasms with germline DDX41 mutation 
• Myeloid neoplasms with germline predisposition and preexisting platelet disorders
  • Myeloid neoplasms with germline RUNX1 mutation  
  • Myeloid neoplasms with germline ANKRD26 mutation
  • Myeloid neoplasms with germline ETV6 mutation 
• Myeloid neoplasms with germline predisposition and other organ dysfunction
  • Myeloid neoplasms with germline GATA2 mutation 
  • Myeloid neoplasms associated with bone marrow failure syndromes 
  • Myeloid neoplasms associated with telomere biology disorders 
  • Myeloid neoplasms associated with Noonan syndrome 
  • Myeloid neoplasms associated with Down syndrome 

WHO 2016 Classification of Acute Myeloid Leukemia and Related Neoplasms

• Acute myeloid leukemia (AML) with recurrent genetic abnormalities
  • AML with t(8;21)(q22;q22); RUNX1-RUNX1T1 
  • AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11 
• Acute promyelocytic leukemia with PML-RARA 
  • AML with t(9;11)(p21.3;q23.3); MLLT3-KMT2A 
  • AML with t(6;9)(p23;q34.1); DEK-NUP214 
  • AML with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM 
  • AML (megakaryoblastic) with t(1;22)(p13.3;q13.3); RBM15-MKL1 
  • Provisional entity: AML with BCR-ABL1 
  • AML with mutated NPM1 
  • AML with biallelic mutations of CEBPA 
  • Provisional entity: AML with mutated RUNX1 
• AML with myelodysplasia-related changes
  • Therapy-related myeloid neoplasms 
• AML, not otherwise specified (NOS)
  • AML with minimal diwerentiation 
  • AML without maturation 
  • AML with maturation 
  • Acute myelomonocytic leukemia 
  • Acute monoblastic/monocytic leukemia 
  • Pure erythroid leukemia 
  • Acute megakaryoblastic leukemia 
  • Acute basophilic leukemia 
  • Acute panmyelosis with myelofibrosis 
• Myeloid sarcoma 
• Myeloid proliferations related to Down syndrome
  • Transient abnormal myelopoiesis (TAM) 
  • Myeloid leukemia associated with Down syndrome

2017 European LeukemiaNet Risk Stratification by Genetics for Acute Myeloid Leukemia

Favorable	t(8;21)(q22;q22); RUNX1-RUNX1T1  inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11  Mutated NPM1 without FLT3-ITD or with FLT3-ITD low(c)  Biallelic mutated CEBPA
Intermediate	Mutated NPM1 and FLT3-ITD high(c)  Wild type NPM1 without FLT3-ITD or with FLT3-ITD low(c) (w/o adverse-risk genetic lesions)  t(9;11)(p21.3;q23.3); MLLT3-KMT2A d  Cytogenetic abnormalities not classified as favorable or adverse
Adverse	t(6;9)(p23;q34.1); DEK-NUP214  t(v;11q23.3); KMT2A rearranged  t(9;22)(q34.1;q11.2); BCR-ABL1  inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM(EVI1)  �5 or del(5q); �7; �17/abn(17p)  Complex karyotype, e monosomal karyotype f  Wild type NPM1 and FLT3-ITD high(c)  Mutated RUNX1 g  Mutated ASXL1 g  Mutated TP53 h

Clinical features

AML
??, ????? ??. ?? ????, 
??, Myeloid sarcoma(chloroma)
M3? DIC
M4,5? ????, ??, ?? ??
M7? ?? type? ?? ?? 5? ??? ??.
Usually MPO+, except monocytic & megakaryoblastic leukemia

Genetic findings

Immunophenotypic findings

Prognostic factors

#??? hyperleukocytosis(>100,000)? early CNS bleeding, pul. leukostasis
#????(CR)? ?? ????? CR??? ??? ?? ??.

Down SD: AML? 30? ? ??? ?? good. drug toxicity? ?? reduction ??

Clinical presentation

Symptoms

Physical findings

Hematologic findings

Treatment

AML: CyAn+1st SCT
APML(M3): ATRA+An+SCT
ALL: VPDL+2nd SCT

Retinoic acid syndrome (ATRA ???)
??? ????? ???? ??. ?? induction? ????.
?? 3? ? ??, ????, ??, ??, � ? Glucocorticoid, CTx(An), supportive Tx

AML: M phase ?- ??, ???? ??
ALL: G0/G1 phase ? – ???? ??

PBS	Neutrophil >1,000/?l, plt >100,000/?l, ?? ???, NO BLAST
BM	Cellularity >20%, blast < 5%, NO AUER ROD
	NO EXTRAMEDULLARY LEUKEMIA
	RT-PCR, FISH?? ???/??? ???? ??

????(CR)? ?? – CR ??? consolidation Tx.

2. ??: ????? ???? BM > CNS, ?? 
3. ??? ?? ? ??? ?????? sepsis

Induction chemotherapy

Postremission therapy

Supportive care

??
Packed RBC ?? Hb>8g/dL ??
??? ?? ??? ?? (GVHD ??)
??????? (??? ?? ??? ??)

Treatment for refractory or relapsed AML

Treatment of acute promyelocytic leukemia