Leucemias Mielomonocitica Juvenil: Aspectos...
Transcript of Leucemias Mielomonocitica Juvenil: Aspectos...
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Luiz Fernando Lopes
Leucemias Mielomonocitica Juvenil: Aspectos atuais
Diagnosticar e tratar : Dois desafios na mesma doença
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Declaração de Conflito de Interesse
Declaro que não possuo conflito de interesse na (s) categoria (s) abaixo:•Consultoria: •Financiamento de Pesquisa: •Honorário: •Patentes e Royalties:•Afiliação na Diretoria de uma Entidade ou Conselho: •Discussão de uso de drogas Off-label:
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Síndrome Mielodisplásica
RA 1RAEB 8RAEB-t 1cmmL 3MDS2d 1
Lopes, LF . Ped Hematol / Oncol, 1999; 16:347
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Síndrome Mielodisplásica em PediatriaProposta para Classificação
Hann 1992 Gardner & Haas 1992 Schwartz &Cohen 1993
Altman 1993Juvenile CML RAInfantile monosomy RARS RARA RAEB RARSRAEB RAEB-t CMMLDown Synd. Monosomy 7 syndrome RAEBLeukemoid reaction Juvenile CML RAEB-tMyelodysplasia with JCML in traformation Juvenile CMLEosinophiliaAtypical CML Ph-negative CML Familial CMLFamilial CML CMMLEssential thrombicythemia ChronicmonocyticleukemiaPolycythemia rubra veraSecondary myelodysplasia
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Antonio Machado y Ruiz (1875-1939)
“Caminante, son tus huellasel camino, y nada más;caminante, no hay camino,se hace camino al andar...”
1997
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Classificação de 96 crianças com SMD
RA RCMD RARS RAEB RAEB-t CMML Down S CON.ABN.
SEC. MDS
Non class.
Total
FAB 8 46 6 14 74
WHO 2 7 22/24 14 4 73
PEDIATRICHasle
9 41 5 14 8 5 82
FRENCH 7 40 5 13 10 5 80
Mario Jose Aguiar de Paula - Tese-2004-FAP
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2000 21 114 64
2002 41 176 96
# centros total SMD
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Survival in Advanced MDS without SCTor HSCT after > 6 Months of Intensive Therapy
121086420
1.0
.8
.6
.4
.2
0.0years
P
p=0.31, SE=0.07 N=65, 35 events
EWOG-MDS unpublished-meeting S.Paulo 2003
Months60483624120
Cum
Sur
viva
l(%
)
1,0
,8
,6
,4
,2
0,0
GCB-SMD-Ped- Meeting S.Paulo 2003
GCB-SMD-PEDGRUPO COOPERATIVO BRASILEIROSÍNDROME MIELODISPLÁSICA EM PEDIATRIA
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• I. Mielodisplasia / Doença Mieloproliferativa • Leucemia mielomonocitica Juvenil (LMMJ)• Leucemia mielomonocitica Crônica (LMMoC) • Leucemia mieloide crônica BCR-ABL negative
(LMC Ph-)• II. Síndrome Down (SD)
• Mielopoiése anormal transitória (TAM)• Leucemia Mielóide em SD
• III. Síndrome Mielodisplasica (SMD)• Citopenia refratária (CR)
• (Sp blastos <2% e MO blastos <5%)• Anemia refratária com excesso de blastos (AREB)
• (SP blastos 2-19% ou MO blastos 5-19%)• AREB em transformação (RAEB-t)
• (SP ou MO blastos 20-29%)
Hasle H et.al., Leukemia 2002, 17: 277-282
New Pediatric Classification
2003
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Annual incidence per million - children till 14 years1,8 SMD– 1,2 JMML– 0,9 LMA S. Down
Media Age (years)- 6,8 MDS– 1.8 JMML
Sex Distribution – equal MDS – > boys JMML
SMD e LMMJ
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Acquired or Congenital Abnormalities Constitutional? Associated with MDS or LMMJ
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Current diagnostic criteria for juvenilemyelomonocytic leukemia
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Classificaçao WHO 2016
I- Clinical and hematologicfeatures(all 4 featuresmandatory)
II- Genetic studies(1 findingsufficient)
III- For patients without geneticfeatures, besides the clinicalhematologic features listedunder I, the following criteriamust be fulfilled
• Peripheral blood monocytecount > 1x10 9 /L
• Somatic mutation in PTPN11*or KRAS*or NRAS*
• Monosomy 7 or any otherchromosomal abnormality orst least 2 of the followingcriteria:
• Blast percentage in peripheralblood and bone marrow<20%
• Clinical diagnosis of NF1 orNF1 mutation
• Hemoglobin F increased for age
• Splenomealy • Gerline CBL muation and lossof heterozygosity of CBL**
• Myeloid or erythroidprecursors on peripheralblood smear
• ABSENCE OF Philadelphia choromosome (BCR/ABL1 rearrangement)
• GM-CSF hypersensitivity in colony assay
• Hyperphosphorylation ofSTAT5
Diagnostic Criteria for Juvenile Myelomonocytic Leukemia(modified by Locatelli &Niemeyer,2014)
*Germline mutation(indicating Noonan sydrome)need to be excluded**Ocasional cases with heterozygous splice site mutations
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Alerta
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• 21 months - male - lymphonodomegaly, hepatosplenomegaly. • Hb 9.9 g/dl, platelet 90 x 10³/μL, WBC 61 x 10³/µL (24% PMNL, 16% monocytes) • BM smears hypercellular M:E ratio of 7: 1 • normal karotype. • Fetal hemoglobin was 3.2%, with 23% F cells• LMMJ ?
EBV Studies
• Recent acquired infection (↑VCA-IgG, antibody EA and absent EBNA antibodies).
• Subsequently, antibodies to EBNA + ↑VCA-IgG, EA - remained elevated for over 3 years.
• Persistent Epstein-Barr Virus Infection
EBV Herrod HG et al., 1983
Silvia LuporiniSJCampos 2011
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Concomitant active viral infection and JMML
• Kirby M et al., 1990: JMML differentiation from infantile cytomegalovirus infection
• Toyoda H et al., 2004 – JMML with concomitant cytomegalovirus infection
• Vallero S et al., 2009 - JMML and active Parvovirus B19 infection at diagnosis
• Prabhu SB et al., 2010 - JMML Presenting With Coexistent Cytomegalovirus Infection
Silvia LuporiniSJCampos 2011
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The possibility that viruses contribute to MDS pathogenesis by stimulating pre-exiting malignant
clones
Van den Berg, H et al., 1999 - MDS (Refractory anemia) and HIV/AIDS
Manabe A et al., 2004 - monoclonal integration of the EBV genome in LMMJ
Kishore J, Mukhopadhyay C , 2004 - case of pure red cell aplasia in a 45-year-old female for last 7 years due to chronic persistent parvovirus B19
infection to myelodysplasia after 4 years
Silvia LuporiniSJCampos 2011
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NAPNucleo Apoio ao
Pesquisador
CPOMCentro PesquisaOnco Molecular
HOSPITAL ClinicaTMO
BARRETOS- Hospital de cancer infantojuvenil
2013Contatos:[email protected]@hcbinfantil.com.brPhone:17-3321-5400 ramal 5493
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Hospital de Câncer Infantotjuvenil Barretos
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MieloproliferativasMaria Lucia de Martino Lee- UNIFESP
Morfologia
Citometria de FluxoAnita Frisanco Oliveira- Barretos
Eduardo Caetano- Barretos
Elizabeth Xisto Souto-H.Brigadeiro- SP
Irene Lorand-Metze- UNICAMP
Lígia Niéro-Melo- UNESP
Maria Claudia Zerbini-USP- SP
Nydia Bacal- H.A.Einstein- SP
Rafael Dezen Gaiolla-UNESP
TerapêuticaAdriana Seber-H.S.Camilo-SP
Neysimelia Villela- Barretos
Roseane Gouveia-H.S.Camilo- SP
EpidemiologiaLuiz Fernando Lopes- BarretosGlaucia Regina C. Murra- BarretosBeatriz Pereira Martins- Barretos
Genética clínicaHenrique Galvão- Barretos
Genética MolecularDaniel O.Vidal- Barretos
Maria de Lourdes Chauffaille-
UNIFESP
CitogenéticaElvira D.R. P. Velloso-USP- SP
Simone Jacovaci Colleta- Barretos
Genética
Falencias MedularesCelia Martins Campanaro –F.M.Jundiaí
Marlene Garanito –USP- SP
Silvia Luporini-Sta Casa- SP
2016
2016- 6 comitês -28 membrosCoordenador – Dr Luiz Fernando Lopes
Serviços de ReferenciaBenigna – Belo Horizonte
Isis Magalhaes- Brasília
Viviany Viana - Fortaleza
NAP-Barretos Diego Alves- Barretos
Gabriela Bernal Salvador
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Morfologia/Citometria de Fluxo
Irene Lorand-Metze Elizabeth Xisto Souto Lígia Niéro Maria Claudia Zerbini
Nydia BacalRafael Dezen Gaiolla
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Elvira Deolinda R.P. Velloso
Mieloproliferativas
Maria Lucia Lee
Terapêutica/TMO
Citogenética
Adriana Seber Roseane Gouveia
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Henrique Galvão Oncogenetica
Glaucia Murra Enfermeira Coordenadora SMD
Neysimelia VillelaTMO
Anita FrisancoMorfologia
Diego AlvesBanco de Dados
Equipe de Barretos
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Daniel VidalBiologia Molecular
Aline TansiniCitometria Fluxo
Simone Jacovaci ColletaCitogenetica
Eduardo CaetanoHemopatologia
Equipe de Barretos
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2000 21 114 64
2002 41 176 96
2004 55 233 120
2016(Setembro)
149 572 228
# centros total SMD
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7
4966
5786
265
19 47 6631
64
228
26
96132
88
150
492
0
100
200
300
400
500
600
1985-1996 1997-2001 2002-2006 2007-2011 2012-2016(September)
Conclusive Diagnosis (1985-2016 -September)
MDS Not
MDS
Total
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Total = 572
0
50
100
150
200
250
1985-1996 1997-2001 2002-2006 2007-2011 2012-2016
26 - 4,5%
99- 17,3%
139 - 24,3%
95 - 16,6%
213- 37,2
Total of cases registred / period
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0
10
20
30
40
50
60
70
80
90
100
I - 1985-1996
II - 1997-2001
III - 2002-2006
IV - 2007-2011
V - 2012-2016
84,2%
95,7% 98,5%
98,7%
100%
42,1%
68% 67,1%
58%
87,5%
21%
68%
65,6%
41,9%
79,6%
Exames Diagnósticos Realizados
Citologia
Histologia
Citogenética
0
10
20
30
40
50
60
70
80
I - 1985-1996II - 1997-2001III - 2002-2006IV - 2007-2011V - 2012-2016
0% 0% 0%6,4%
78,1%
0% 0% 0% 0%
68,7%
0% 0% 0% 0%
26,5%
Exames Diagnósticos Realizados
Imunofeno
Molecular
Genética Clinica
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0
10
20
30
40
50
60
70
80
90
100
Citologia Histologia Citogenética
84,2
42,1
21
95,7
6868
98,5
67,1 65,6
98,7
58
41,9
100
87,5
79,6I - 1985-1996
II - 1997-2001
III - 2002-2006
IV - 2007-2011
V - 2012-2016
Exames Diagnósticos Realizados%
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Total = 572
Case Distribution MDS x Non MDSGCB-SMD-PED
265 - 46,3%
228- 39,7%
58 - 10,1%
22 - 3,7%
Case Distrinution MDS x Non MDSGCB-SMD-PED
Not MDS
MDS
On Going
Inconclusive
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LMA – 52
Hipo – 36 Agranulocitose Amegacariocitose Congênita Anemia Carencial Anemia Diseritropoética/ Congênita Anemia Megaloblástica Anemia Sideroblástica Anemia Hemolítica Hemopatia Primária Hipoplasia de Mo Mielofibrose Leucopenia/ Neutropenia Idiopática Trombocitemia Púrpura Trombocitopênica Idiopática (PTI)
MO reacional – 36
Casos não SMD Dçs Hereditarias– 18
Anemia Aplástica – 20
LLA – 09
MO Hiper – 11
Fanconi Síndrome Wiskott Aldrich Síndrome Pearson Doença de Gaucher Blackfan Diamond Neutropenia Familial Talassemia Síndrome Kostmann
Leishmania Pavovirus Vírus EB Hepatite HIV Toxocariase
LMC– 5
Outras – 71
Infecção – 7
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0
10
20
30
40
50
60
70
80
90
100
RC RAEB RAEB-t JMML MDS / AML Notappropriate
31 - 15,4%
98 - 43,2%
18 - 7,9%
48- 21,1%
14 - 6,2% 14- 6,2%
Classificação HASLE 2002N (%)
Total= 228
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1997-2001%
2002-2006%
2007-2011%
2012-2016%
RC 14,8 9,0 19,3 21,8
RAEB/ RAEB-t 53,1 66,6 45,1 32,8
JMML 12,7 16,6 29,0 31,25
Others (Constitutional/ 2nd MDS)
19,1 7,5 6,4 14,0
76 6
14
25
44
14
21
6
11 9
20
95
2
9
0
5
10
15
20
25
30
35
40
45
50
1997-2001 2002-2006 2007-2011 2012-2016
RC
RAEB/RAEB-t
JMML
Others
(N= 47) (N= 66) (N= 31) (N= 64)
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36
100
18
47
1
147 5
15,8
43,9
7,9
20,6
0,46,1 3,1 2,2
SMDN %
TOTAL:228
Congresso HEMO/Florianópolis, 2016
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Meses 1985- 2016Total:228Congresso HEMO/Florianópolis, 2016
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Congresso HEMO 2016Time (months)
706050403020100-10
Cum
Sur
viva
l
1,2
1,0
,8
,6
,4
,2
0,0
MDS
other MDS
JMML
RAEB-t
RAEB
RA
1985 – 2002 ♯ 96 1997 – 2016 ♯ 193
Congresso SMD-SP 2003
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p:0,496
Congresso HEMO/Florianópolis, 2016
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Nº %
1997 a 2006 114 50,02007 a 2016 95 41,7TOTAL 209 91,7Não se aplica 19 8,3TOTAL 228 100,0
Período
Congresso HEMO/Florianópolis, 2016
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p:0,008
Total N # Eventos
1997 a 2006 105 72
2007 a 2016 88 28
TOTAL 193 100
Congresso HEMO/Florianópolis, 2016
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p:0,064 p:0,024
Total N #Eventos
SMD 1997 a 2006 76 522007 a 2016 50 15Total 126 67
LMMJ 1997 a 2006 16 132007 a 2016 27 10Total 43 23
Congresso HEMO/Florianópolis, 2016
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Cursos itinerantes
Brasília
Novembro 2002
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Manaus – Junho 2008
Cursos itinerantes
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Salvador – Outubro 2014
Cursos itinerantes
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SMD: 5 Reuniões de Morfologia em BarretosAbril – Novembro 2015
• 4 hematologistas/patologistas convidados por reunião
• Morfologistas – GCB-SMD-PED:
Maria Lucia Lee
Claudia Zerbini
Ligia Niero
Rafael Gaiolla
Elizabeth Xisto
Irene Lorand-Metze
• Equipe Barretos :
Aline Tansini - flow cytometry;
Daniel Vidal - Molec Biol;Simone Jacovaci Colleta – Cytogenetics;Eduardo Caetano – Phatology;
Henrique Galvão – Genetic;
Anita Frisanco - Morphology;
Neisymelia Vilella- BMT
www.cure4kids.org sala Brasil
Ultima quinta feira do mes 14 hs (11 am Memphis)
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SMD - Reuniões de morfologia em Barretos
Belo Horizonte - MG• Mitiko Murao• Frederico de Melo
São Paulo - SP•Claudia Oliveira•Rafael Baceiro•Lilian Cristofani•José Eduardo Bernardes
Porto Alegre - RS• Elissandra Machado• Adriana Santini
Fortaleza - CE• Viviany Viana• Carlos Gustavo Hirth
Rio de Janeiro - RJ• Anna Beatriz Willeme• Ana Paula Bueno
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Surpresas e alegrias !!!
Primeira criança identificada com SMD 1989
Outubro 2016Enfermeira P.I., aos 36 anos
1989P.I., aos 9 anos
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Praça XV – Dudu Gentil / Vila Nossa Senhora do Desterro-Fundação 1662
Obrigado!!