A 58-year-old white Chinesewoman has presented at her localGP with unexpected bruising.The patient has raised that when younger, she worked in afactory which manufactured benzene, she has lived in the UK for around 14years. Other symptoms that she is showing: -unexpectedweight loss -painin upper left flank -nightsweats -fatigue Physical examination showed splenomegaly explaining for thepain in the upper left flank. The other symptoms; fatigue, bruising could beresult anemia. The symptoms shown are inline with chronic leukemia but furthertesting is needed.CBC and DC will be recommended to test for any abnormalitiesin the blood. The results from the CBC showed leucocytosis andthrombocytosis.
The differential count showed a slight increase in neutrophils,lymphocytes, monocytes, basophils, andeosinophils, reinforcing the diagnoses of leucocytosis. Symptoms with theincrease of blasts cells but staying >10 would suggest the patient is in thechronic phase of CML. However further test should be carried out. Bone marrow samples are need needed to be taken.
From image3 you can see that the patients bone marrow is hypercellular,and shows low levels of fat. Observing the biopsy you can see the increasednumber of white blood cells compared to red. Megakaryocyte cells are dysplasticin nature with blasts cells making up around 8% of all cells.
Enlarged Spleen – Pain in upper left flank, is typical ofCML RBC was not consideredto be outside of reference range, however,would be considered on the ‘low’ side, which would explain for the fatigue – anemia. Leucocytosis and thrombocytosis are characteristic of CML. Blasts cells under 10 in the peripheral blood would hinttowards the chronic phase of CML. Peripheral blood smear obtained from the patient is again common in CML. Again the patient had8% blasts cells in the bone marrow which would indicate CML. On detection of the Philadelphia chromosome abnormality (Ph)and/or the BCR-ABL1 oncogene through further testing – karyotyping, FISH andPCR can CML be confidently diagnosed. FISH and karyotyping showed the presence of Ph Chromosome and BCR-ABL1 gene.
Administration of tyrosine kinase inhibitors (TKI):First line treatment: Imatinib– 400mg/daily.Second line treatment: Nilotinib 300mg twice/daily orDasatinib 100mg/daily.Third line treatment: Hematopoietic stem celltransplantation. The patient is in the chronic phase of the disease. Imatinib as first lineresponse showed an 82% complete cytogenetic response rate at 6 years. If intolerance of first-linetreatment occurs, Dasatinib has shown increased rates of complete hematologicresponse at 3, 6, 12 and 18 months compared to Nilotinib.Stem cell transplant should only be offered if first andsecond line treatments fail. Hematologic, cytogenetic and molecular response is monitoredto ensure treatment is affected.
Hematologic is measured every 2 weeks, until CHR is achieved– platelet = <450 x 109/L & WBC = <10 x 109/L. Cytogenetic is measured every 6 months until a CCR is achieved– Ph + Chromosome is 0%. Molecular response is measured every 3 months until MCyR isachieved – the transcript isnon-quantifiable NCR-ABL <0.10. All responses should be achieved once, and again confirmedat 2 different points in the treatment. The patient showed tobe responding to treatment, with partial hematologic, the cytogenetic and molecular response at 6 monthsfollow up. However first-line treatmentshould be continued.
At 12 months the patient again showed a completehematologic, cytogenetic and molecular response to treatment. Hematologic, cytogenetic and molecular responses are stillmonitored to ensure the patient does not relapse.