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Case Report | DOI: https://doi.org/10.31579/2690-4861/074
1 Graduate, Ayub Medical College, Pakistan.
2 Resident Physician, Staten Island University Hospital, New York, NY, USA.
*Corresponding Author: Madeeha Subhan Waleed, Graduate, Ayub Medical College, Pakistan.
Citation: M S Waleed, W Sadiq. (2020) A Rare Case of CML with Abnormally High TLC Count. International Journal of Clinical Case Reports and Reviews. 4(1); DOI: 10.31579/2690-4861/074
Copyright: © 2020 Madeeha Subhan Waleed, This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Received: 06 October 2020 | Accepted: 19 October 2020 | Published: 28 October 2020
Keywords: neoplastic transformation; hemopoietic stem cells; TLC; CML
CML is a neoplastic transformation of the primitive hemopoietic stem cells.CML presents with increased TLC count and low lap score.Diagnosis is made using peripheral smear,flow cytometry or bone marrrow biopsy.The incidence of this disease has remained stable for the last decade.We present to you a young male with CML and abnormally high WBC count of 175 000/uL ,low LAP score and anemia.Philadelphiahia chromosome (9:22 translocation) is positive.
Introduction
Chronic myelogenous (CML) or chronic granulocytic leukemia (CGL) is a malignancy of the white blood cells.it accounts for 15% of the adult leukemias.in severe cases.it has three phases i.e chronic, accelerated and the blast phase.Blast crisis is defined as the presence of ≥ 30% marrow blasts [1]. The translocation of 9,22 chromosome leads to a fusion protein bcr-abl tha is an active tyrosine kinase .chronic myeloid leukemia (CML) patients receive imatinib which is a tyrosine kinase inhibitors as first-line therapy achieve good cytogenetic and molecular responses [2,3].
Case Presentation
A 39 year old previously healthy male presented to the hospital with weightloss,left sided chest discomfort and tightness for two months.These symptoms started gradually.The pain was burning in character which didnot radiate anywhere.There were no aggravating factors.It was intially relieved by pain killers but then slowly it increased to the point where he decided to seek medical attention.There was no associated shortness of breath,sputum production,cough or hemoptysis.Moreover,he also complained of generalized fatigue,malaise low grade fever for 2 months.He lost about 7 kgs during this period.On review of system there was no headache,visual changes,vomiting,urinary or bowel problems. Regarding his social history he is an electrician ,non-alcoholic and nonsmoker but eats naswar(sun dried tobacco leaves).On examination, he had no edema,pallor,petechia,pallor,clubbing or lymphadenopathy.Cardiovascular and respiratory exam was unremarkable.On abdominal exam,there was no tenderness.The spleen was palpable two finger breadth below the costal margin and the bowel sounds were hypoactive.It was decided to admit him and appropriate laboratory investigations were ordered.The patient was started on ceftriaxone and vancomycin since he was febrile.
Laboratory Workup: Complete blood picture:
Peripheral smear:
There was marked leuckocytosis with bimodal peak of neutrophils and immature granulocytes.Anemia with rbc morphology showing anisocytosis ,hypochromia,pokilocytosis with pencil cells. Platelets were adequate.Peripheral film was suggestive of chronic myeloid Leukemia.
ESR: 76/1st hour.
Blood metabolic profile:
Blood metabolic profile:
Chest Xray: Normal study Philidalpheia chromosome was positive. Urine R/E: Normal LAP score: 02 Diagnosis was confirmed with flow cytometry.
Discussion
CML occurs in 7% to 15% of all leukemias in adults with approximately 1 to 1.5 cases per 100,000 population [4, 5]. Asymptomatic cases of CML have increased from 15% to about 40% of all cases.A European study published in 2007 estimates the CML incidence to be 1-2 cases per 100,000 people every year [6].The activity of leukocyte alkaline phosphatase is reduced in nearly all patients at diagnosis [7].According to Medscape total WBC count 20,000-60,000 cells/μL in CML with mildly increased basophils and eosinophils while in our patient it was 175,000/uL. The most common feature of CML is an increased WBC count, usually above 25,000/µL [8]. Some patients have wide cyclic variations in their WBC count, with peak counts every few days or separated by up to 70 days [9]. The WBC differential usually shows granulocytes in all stages of maturation, from blasts to mature granulocytes that look morphologically normal.Most patients have mild anemia on diagnosis.
Conclusions
Patient suffering from CML may have a subtle presentation.Blood work up will often lead to diagnosis of CML.They may present with TLC count greater than 100,000 which is usually a feature of Acute Leukemia.Extreme leukoctytosis can cause leukostasis which itself contribute to additional complications.Imitanib should be started in bcr-abl positive patients to bring wbc count in the normal range and the response should be monitored as some patients might require leukaphereis.As CML is one of the four main types of leukemia that affects and accounts for 15 % of all the leukemias worldwide approximately,so proper workup and therapy should be started immediately to decrese mortality and morbidty.