Chronic Myeloid Leukemia Presenting with Hyperleukocytosis and Coagulopathy, Complicated by Gout and Renal Mass
Introduction/Background/Significance: Hyperleukocytosis (WBC >100,000/μL), typically seen in acute leukemia, can also occur in chronic myeloid leukemia (CML) and is associated with high morbidity and mortality[1, 2]. Here, we present a rare case of chronic-phase CML (CP-CML) with a renal mass and hyperleukocytosis complicated by leukostasis, disseminated intravascular coagulation (DIC), acute gout flare, and secondary infection.
Materials and Methods/Case Presentation/Objective: A 59-year-old male with gout and a 15-pack-year smoking history presented with blurry vision, left thigh, and right chest wall pain and swelling. Labs revealed marked leukocytosis (WBC:436×103/μL, ANC:227×103/μL, metamyelocytes:5%, basophils:0%), immature-like cells on smear, anemia (Hb:9g/dL, MCV:105.6fL), normal platelets (PLT:314×103/μL), elevated LDH (1900U/L) and uric acid (13.2mg/dL), prolonged APTT/PT, low haptoglobin, and normal fibrinogen. Imaging showed splenomegaly (20cm), a calcified right renal mass (4×3cm), and large hematomas in the chest wall and thigh.
He was started on hydroxyurea and rasburicase, but WBC rose to 673×103/μL with leukostasis symptoms and DIC, prompting leukapheresis. Flow-cytometry showed 2% CD34+ cells with left-shifted granulocytosis; FISH confirmed BCR-ABL translocation. Dasatinib was started for presumed accelerated-phase CML (CML-AP). Bone marrow (BM) biopsy confirmed CML-CP with 100% cellularity, 2% blasts, mild fibrosis, and BCR-ABL Mbcr (p210) transcript at 51% IS; Sokal score was 1.3 (high-risk).
After dasatinib treatment, he developed pancytopenia (WBC:0.54×109/L, RBC:2.35×106U/L, PLT:17x103/μL), leading to a one-week treatment hold. He also had a severe polyarticular gout flare, confirmed by arthrocentesis, with tenosynovitis and tophaceous arthritis, and treated with prednisone and colchicine.
A week after his discharge, he returned with left great toe pain with purulent discharge, diagnosed with gout flare with superimposed soft tissue infection, and was treated with empiric antibiotics, colchicine, and prednisone. MRI was inconclusive for osteomyelitis. Aspiration revealed urate crystals; culture grew E.faecium. He improved with the treatment and was discharged with urology follow-up for possible nephrectomy of the renal mass.
Results/Description/Main Outcome Measure(s)
This case illustrates a rare presentation of CML-CP with hyperleukocytosis, typically seen in acute leukemias and reported in CML case reports[1, 3, 4], with its complications—DIC, leukostasis, and leukemic retinopathy[2, 5]. In such cases, leukapheresis is used to quickly reduce the WBC count[1], as in our case. Although renal tumors can trigger paraneoplastic DIC[6], the resolution of coagulopathy with WBC control pointed toward leukostasis as the cause.
Despite suggestive signs of advanced disease[7], BM confirmed CML-CP. Tyrosine-kinase inhibitors (TKI) remained the cornerstone of treatment[8] and led to a rapid response. Gout, though rarely reported in CML[8], posed treatment challenges due to superimposed infection and immunosuppression.
Conclusions: This case demonstrates that hyperleukocytosis and DIC can occur in CML-CP, mimicking advanced disease. Despite concerning clinical features, BM confirmed CML-CP, highlighting the importance of molecular confirmation. Rapid cytoreduction and TKI therapy were effective, though complications like gout flare, infection, and a renal mass added management challenges.
References
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8. Jabbour E. Am J Hematol. 2024;99(11):2191-2212.
