• MPN-type
– significant weight loss – drenching nigh sweats – left upper quadrant pain from significant splenomegaly
Morphology (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
Occasionally, overexpression of CD56, aberrant expression of CD2, and decreased expression of HLA-DR, CD13, CD15, and CD36 may be observed.
Histopathology
relatively insensitive as compared with cytochemistry or flow cytometry
the most reliable markers : CD168R, CD163
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.
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.
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
（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
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.
Morphology (BM)
hypercellular in over 75% of cases
normalcellular and hypocellular also occur
dysgranulopoiesis, dyderythropoiesis, micromegakaryocytes amally lobated nuclei (in up to 80% of patients)
(WHO classification of myeloid neoplasms)
Diagnosis
Clinical manifestation
• MDS-type
– Fatigue and dyspnea due to anemia – susceptibility to infections – rarely bleeding