CMML诊治进展
江苏省人民医院 血液科 洪鸣
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Contents
1 Definition 2 Diagnosis 3 Risk stratification 4 Therapeutic options
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Definition
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WHO Classification of MDS/MPN
1 CMML
The monocytes generally are mature, but can exhibit abnormal granulation or unusual nuclear lobation or chromatin patten. (abnormal monocytes)
Dysgranulopoiesis is present in most cases.
grnulocytic proliferation an increase in erythroid precursors mild to moderate increase in the amount of reticulin fibres (30%)
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Immunohistochemistry on tissue sections
monocytic proliferation can be difficult to appreciate (cytochemistry and immunohistochemistry)
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Abnormal monocytes have
Monocytosis with morphologicadllyenser chromatin, nuclear
normal monocytes (PB)
convolutions and folds and a
more greyMCiysotohnpolcacystymesticwoaibtphnlonarumcslaemlaitrie.asn(dPB)
Promonocytes typically have a light-gray cytoplasm with a few lilac-colored granules and a stippled nuclear chromatin.
CMML-1 (BM)
CMML-2(BM)
Representative peripheral blood and BM smears distinction between promonocytes and ab.normal monocytes may be problematic
Immunophenotype
The PB and BM cells usually express CD33 and CD13, with variable expression of CD14, CD68, CD64.
An increased percentage of CD34+ cells has been associated with early transformation to acute leukemia.
• MPN-type
– significant weight loss – drenching nigh sweats – left upper quadrant pain from significant
splenomegaly
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Morphology (PB)
PB monocytes usually range from 2 to 5 × 109/L, but may exceed 80 × 109/L.
2 Atipical CML, BCR-ABL1 negative
3 JMML
4 MDS/MPN, U (RARS-T, refractory
anemia with ringed sideroblasts associated with thrombocytosis)
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Definition
A clonal hematopoietic stem cell disorder that is characterized by the presence of an absolute monocytosis (>1×109/L) in the peripheral blood and the presence of myelodysplastic and myeloproliferative features in the bone marrow.
(WHO classification of myeloid neoplasms)
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Diagnosis
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Clinical manifestation
• MDS-type
– Fatigue and dyspnea due to anemia – susceptibility to infections – rarely bleeding
relatively insensitive as compared with cytochemistry or flow cytometry
the most reliable markers : CD168R, CD163
Occasionally, overexpression of CD56, aberrant expression of CD2, and decreased expression of HLA-DR, CD13, CD15, and CD36 may be observed.
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Histopathology
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Morphology (BM)
hypercellular in over 75% of cases
normalcellular and ellular also occur
dysgranulopoiesis, dyderythropoiesis, micromegakaryocytes and megakaryocytes with abnormally lobated nuclei (in up to 80% of patients)