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Metaplazia mieloida cu

mielofibroza

Dr. Ana Manuela Crisan


Clinica de Hematologie si Transplant Medular
Institutul Clinic Fundeni
WHO classification of myeloid malignancies

1. Acute myeloid leukemia (AML) and related precursor neoplasms

2. Myeloproliferative neoplasms (MPN)


2.1. Classic MPN
2.1.1. Chronic myelogenous leukemia, BCR-ABL1 positive (CML)
2.1.2. Polycythemia vera (PV)
2.1.3. Primary myelofibrosis (PMF)
2.1.4. Essential thrombocythemia (ET)
2.2. Nonclassic MPN
2.2.1. Chronic neutrophilic leukemia (CNL)
2.2.2. Chronic eosinophilic leukemia, not otherwise specified (CEL-NOS)
2.2.3. Mastocytosis
2.2.4. Myeloproliferative neoplasm, unclassifiable (MPN-U)
ET

3. Myelodysplastic syndromes (MDS)


3.1. Refractory cytopenia with unilineage dysplasia (RCUD)
3.1.1. Refractory anemia (ring sideroblasts < 15% of erythroid precursors)
3.1.2. Refractory neutropenia
3.1.3. Refractory thrombocytopenia
3.2. Refractory anemia with ring sideroblasts (RARS; dysplasia limited to
erythroid lineage and ring sideroblasts 15% of bone marrow erythroid precursors)
3.3. Refractory cytopenia with multi-lineage dysplasia (RCMD; ring sideroblast count
does not matter)
3.4. Refractory anemia with excess blasts (RAEB)
3.4.1. RAEB-1 (24% circulating or 59% marrow blasts)
3.4.2. RAEB-2 (519% circulating or 1019% marrow blasts or Auer rods
present)
3.5. MDS associated with isolated del(5q)
3.6. MDS, unclassifiable
ET

4. MDS/MPN
4.1. Chronic myelomonocytic leukemia (CMML)
4.2. Atypical chronic myeloid leukemia, BCR-ABL1 negative
4.3. Juvenile myelomonocytic leukemia (JMML)
4.4. MDS/MPN, unclassifiable
4.4.1. Provisional entity: refractory anemia with ring sideroblasts associated with
marked thrombocytosis (RARS-T)

5. Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of


PDGFRA,c PDGFRB,c or FGFR1c
5.1. Myeloid and lymphoid neoplasms with PDGFRA rearrangement
5.2. Myeloid neoplasms with PDGFRB rearrangement
5.3. Myeloid and lymphoid neoplasms with FGFR1 abnormalities
ET

MPN reprezinta boli clonale dobindite ale celulei STEM


pluripotenta, caracterizate prin proliferarea uneia sau mai multor
linii celulare.
Dovada clonalitatii este confirmata de prezenta aceleiasi mutatii in
toate celulele mieloide cu origine in CSP.
Proliferarea continua poate afecta una, doua sau trei linii celulare
concomitent sau secvential.
Procesul de maturare si diferentiere sunt aparent normale dar
proliferarea este continua.
Evolutia:
-initial: proliferare multilineara si metaplazie mieloida hepato-splenica
-ulterior: mielofibroza si leucemie acuta
Bone marrow stem cell
Clonal abnormality

Granulocyte Red cell Megakaryocytes Reactive


precursors precursors fibrosis

Chronic myeloid Polycythaemia Essential Myelofibrosis


leukemia rubra vera thrombocytosis
(PRV) (ET)
10% 10%

70%
AML 30%
1951- BMPC:
PV, TE, MFI, LMC

2005- JAK2 V617F 1960- cromoz Ph


PV, TE, MFI 1985- BCR-ABL

Ci comune de semnalizare sunt uzilizate de JAK2 i BCR/ABL


Somatic mutations in MPNs -
chronology

Kralovi Nangal
cs et Pikman Klamp
et al, fl et al, ia et al,
al, 2013
2005 2006 2013

JAK2 MPL CALR


V617F
(2005) (2006) (2013)
Mutatiile in MPN Ph (-)

Gena PV TE PMF

JAK2 97% 50-60% 50-60%

CALR 3-10% 3-10%

MPL 33% 33%

Unmutated 10-15% 10-15%


JAK2,CALR,
MPL
JAK2 V617F pozitiv sau negativ ?

PV

TE

MFI
MMM: evolutie
Faza de proliferare=> insuficienta medulara
1=> 15 ani

MMM : tratament
Substitutie ME
Androgeni
Interferon
Anagrelida
Thalidomida

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