(优选)慢性粒单核细胞白血病诊治进展

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.
Occasionally, overexpression of CD56, aberrant expression of CD2, and decreased expression of HLA-DR, CD13, CD15, and CD36 may be observed.
Histopathology
Morphology (BM)
hypercellular in over 75% of cases
normalcellular and hypocellular also occur
dysgranulopoiesis, dyderythropoiesis, micromegakaryocytes and megakaryocytes with abnormally lobated nuclei (in up to 80% of patients)
relatively insensitive as compared with cytochemistry or flow cytometry
the most reliable markers : CD168R, CD163
(WHO classification of myeloid neoplasms)
Diagnosis
Clinical manifestation
• MDS-type
– Fatigue and dyspnea due to anemia – susceptibility to infections – rarely bleeding
CMML-1 (BM)
CMML-2(BM)
Representative peripheral blood and BM smears
distinction between promonocytes and abnormal monocytes may be problematic
Immunophenotype
grnulocytic proliferation an increase in erythroid precursors
mild to moderate increase in the amount of reticulin fibres (30%)
Immunohistochemistry on tissue sections
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.
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.
3 JMML 4 MDS/MPN, U (RARS-T, refractory
anemia with ringed sideroblasts associated with thrombocytosis)
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.
• MPN-type
– significant weight loss – drenching nigh sweats – left upper quadrant pain from significant splenomegaly
Morpholo百度文库y (PB)
PB monocytes usually range from 2 to 5 × 109/L, but may exceed 80 × 109/L.
monocytic proliferation can be difficult to appreciate (cytochemistry and immunohistochemistry)
Abnormal monocytes have
Monocytosis with morphologicadllyenser chromatin, nuclear
（优选）慢性粒单核细胞白血 病诊治进展
Contents
1 Definition 2 Diagnosis 3 Risk stratification 4 Therapeutic options
Definition
WHO Classification of MDS/MPN
1 CMML
2 Atipical CML, BCR-ABL1 negative