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A case of leukocytosis with hepatosplenomegaly

Published:August 15, 2016DOI:https://doi.org/10.1016/j.ejim.2016.08.008

      1. Case description

      A 51-year-old man with hypertension presented with left upper quadrant (LUQ) pain, fatigue, and 10 kg weight loss over 6 months. He had no fever or lymphadenopathy. Physical examination was significant for hepatomegaly palpable 6 cm below the coastal margin and spleen palpable 12 cm below the costal margin. Laboratory evaluation revealed a white blood cell (WBC) count of 206.3 × 103/uL, with 34% neutrophils, 9% blasts, 11% eosinophils, 18% basophils, 8% metamyelocytes, and 18% myelocytes. Hemoglobin level was 9.4 g/dL and platelet count was 455,000/uL. A peripheral blood smear was obtained (Fig. 1).
      What is the diagnosis?

      2. Diagnosis

      The peripheral blood smear shows neutrophilic leukocytosis with granulocytes at different stages of maturation including myeloblasts, myelocyte, metamyelocytes, bands, and mature neutrophils. This is characteristic of chronic myeloid leukemia (CML). Bone marrow biopsy confirmed the diagnosis with conventional cytogenetics showing Philadelphia chromosomes.
      CML is a myeloproliferative neoplasm characterized by translocation t(9;22) giving rise to a constitutively-activated tyrosine kinase BCR-ABL which drives aberrant WBC proliferation. CML leads to production of mature functioning WBCs and hence these patients typically do not present with infections and instead have fatigue and symptoms of splenomegaly e.g. early satiety, or LUQ pain, particularly when bending down e.g. tying their shoes. Differential diagnoses include leukemoid reaction, other myeloproliferative disorders, chronic myelomonocytic leukemia and proliferative myelodysplastic syndromes. Diagnosis is confirmed with detection of Philadelphia chromosomes, which however may be negative in 10–15% cases [
      • Jabbour E.
      • Kantarjian H.
      Chronic myeloid leukemia: 2014 update on diagnosis, monitoring, and management.
      ]. Advent of tyrosine kinase inhibitors over the last 15 years have led to dramatic improvement in outcomes for patients with CML who can now have a near normal life-expectancy [
      • Sasaki K.
      • Strom S.S.
      • O'Brien S.
      • et al.
      Relative survival in patients with chronic-phase chronic myeloid leukaemia in the tyrosine-kinase inhibitor era: analysis of patient data from six prospective clinical trials.
      ].
      The patient was initially treated with hydroxyurea and allopurinol for the hyperleukocytosis and then started on imatinib 400 mg daily.

      Contributors

      All authors provided care for the patient, wrote, and revised the manuscript.

      Funding information

      This report received no specific funding.

      Conflict of interests

      There are no competing interests.

      Informed consent

      Written consent to publication was obtained.

      Ethics committee approval

      Not applicable.

      References

        • Jabbour E.
        • Kantarjian H.
        Chronic myeloid leukemia: 2014 update on diagnosis, monitoring, and management.
        Am J Hematol. 2014; 89: 547-556
        • Sasaki K.
        • Strom S.S.
        • O'Brien S.
        • et al.
        Relative survival in patients with chronic-phase chronic myeloid leukaemia in the tyrosine-kinase inhibitor era: analysis of patient data from six prospective clinical trials.
        Lancet Haematol. 2015; 2: e186-e193