Page 343 - Concise Pathology for Exam Preparation ( PDFDrive )
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328    SECTION II  Diseases of Organ Systems





                                                                                 Stab form









                                                                                 Metamyelocyte




                                                                                 Neutrophil
                                                                                 Myelocyte



                     FIGURE 12.7.  PBS showing a markedly raised leukocyte count with granulocyte precursors
                     ranging from myeloblasts, myelocytes and metamyelocytes to mature neutrophils. Segmented
                     neutrophils and myelocytes predominate.



                     Laboratory	diagnosis:
                       •  Peripheral	smear (Fig. 12.7)
                         -  Normocytic normochromic anaemia
                         -  Total  leukocyte  count  is  markedly  raised,  typically  between  100  3  10 and
                                                                                         9
                           300 3 10 /L.
                                    9
                         -  Granulocyte precursors ranging from myeloblasts, myelocytes and metamyelo-
                           cytes  to  mature  neutrophils  are  seen.  Segmented  neutrophils  and  myelocytes
                           predominate.
                         -  Myeloblasts are less than 10%.
                         -  Increase in basophils and eosinophils is observed.
                         -  Platelets are normal or increased.
                       •	 Bone	marrow
                         -  Hypercellular bone marrow with marked proliferation of all granulocytic elements
                         -  Twenty to thirty percent of patients show mild bone marrow fibrosis in late stages.
                       •	 Philadelphia	chromosome	(Ph) is positive in more than 95% of cases, in all three
                         phases. This is a reciprocal translocation between the long arms of chromosome 9 and
                         chromosome 22 (t9; 22).
                       •	 Leukocyte	alkaline	phosphatase	(LAP)	score is very low, usually less than 5 (normal
                         20–100).
                       2.  Accelerated phase of CML: CML may transform itself to a blastic phase with or with-
                        out going through an accelerated phase. Features of accelerated phase are the following:
                         (a)  Progressive anaemia
                         (b)  Increase in splenic size
                         (c)  Increase in total leukocyte count with an increase in circulating immature cells
                           (blast cells 10–19% in the peripheral blood/or bone marrow)
                          (d)  Peripheral blood basophilia (. 20%)
                                                                                            3
                         (e)  Persistent thrombocytosis (. 1,000,000/mm ) or thrombocytopenia (, 100,000/mm )
                                                             3
                           not responsive to therapy
                         (f)  Cytogenetic evidence of clonal evolution (cytogenetic changes in addition to Ph
                           chromosome, eg, trisomy 8, etc.)






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