Clinical Gastroenterology and Hepatology
Volume 8, Issue 2 , Pages e15-e16, February 2010

Acute Lymphoblastic Leukemia Presenting as Painless Jaundice

  • Darryn Potosky

      Affiliations

    • Division of Gastroenterology and Hepatology, Department of Medicine, University of Maryland School of Medicine, Baltimore, Maryland
    • ,
  • William Twaddell

      Affiliations

    • Department of Pathology, University of Maryland School of Medicine, Baltimore, Maryland
    • ,
  • Sandeep Khurana

      Affiliations

    • Division of Gastroenterology and Hepatology, Department of Medicine, University of Maryland School of Medicine, Baltimore, Maryland

published online 15 July 2009.

Article Outline

 

A 52-year-old woman was admitted with fatigue and jaundice. Two weeks before admission she developed malaise and dyspnea on exertion. Initially, her primary care provider prescribed azithromycin for presumed bronchitis. Her symptoms did not improve, and she subsequently developed jaundice, prompting further evaluation.

She denied any significant alcohol use or known liver disease in herself or family members. She had a prior cholecystectomy for symptomatic cholelithiasis. Physical exam was notable only for jaundice and the lack of any stigmata of chronic liver disease. Laboratory data revealed pancytopenia with a white blood cell count of 0.7 K/μL (4.5–11), hemoglobin 10.3 g/dL (11.9–15.7), and a platelet count of 18 K/μL (153–367). Manual differential showed 78% lymphocytes and 22% neutrophils with no abnormal-appearing cells. Liver enzymes were also abnormal with an AST of 690 U/L (10–41), ALT 639 U/L (15–54), alkaline phosphatase 199 U/L (38–126), total bilirubin 16.5 mg/dL (0.4–1.5), and albumin 3.0 g/dL (3.5–5.2). Acetaminophen level was undetectable. Viral studies revealed immunity to hepatitis A and B and absence of infection with hepatitis C. Liver tests obtained 4 months prior by her primary care provider were entirely normal.

Magnetic resonance venography of the liver and magnetic resonance cholangiopancreatography were normal. Serologic tests indicated prior exposure to cytomegalovirus, herpes simplex virus, and Epstein–Barr virus. Bone marrow and liver biopsy were performed. Bone marrow sections showed a markedly hypercellular marrow with extensive blasts. Flow cytometric analysis showed that the blasts were positive for CD 10 (early B-cell marker), CD 19, CD 20 (B-cell marker), CD 38, CD 79a, and TdT (DNA polymerase expressed solely in premature lymphocytes), a phenotype consistent with the diagnosis of acute precursor B-lymphoblastic leukemia. Liver histology revealed atypical portal infiltrates with monotonous-appearing lymphocytes expanding outside sinusoidal tracts (Figure A). Immunostains were positive for CD 10 (Figure B), CD 20, and TdT (Figure C), confirming hepatic infiltration of acute precursor B-lymphoblastic leukemia as the cause of the patient's jaundice and abnormal liver tests.

The patient was treated with intravenous steroids, resulting in a dramatic improvement in her bilirubin and normalization of her transaminases. She was subsequently given standard induction chemotherapy, achieving a complete marrow remission. She ultimately underwent allogeneic stem cell transplantation and died of complications caused by graft-versus-host disease.

Hepatic involvement of hematologic malignancies is primarily described in autopsy series of patients with chronic lymphoma.1 In case reports and descriptive series, hepatic involvement of leukemia is rare at presentation, even in patients with abnormal liver tests. It is even more uncommon to have hepatic dysfunction at diagnosis, and fulminant failure is rarely seen. In patients specifically with acute lymphoblastic leukemia, malignant infiltration presenting as acute hepatitis is almost solely described in the pediatric literature.2 To our knowledge, there is only one reported adult case3; however, no reported cases (pediatric or adult) have biopsy confirmation, relying on exclusion of other causes for diagnosis. Last, diagnosis is essential, because normalization of liver function is required for standard induction chemotherapy because many agents used are metabolized by the liver.

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References 

  1. Walz-Mattmuller R, Horny HP, Ruck P, et al. Incidence and pattern of liver involvement in haematological malignancies. Pathol Res Pract. 1998;194:781–789
  2. Kelleher JF, Monteleone PM, Steele DA, et al. Hepatic dysfunction as the presenting feature of acute lymphoblastic leukemia. J Pediatr Hematol Oncol. 2001;23:117–121
  3. Aoki CA, Bowlus CL, Rossaro L. An adult case of acute lymphoblastic leukaemia presenting as hepatic dysfunction. Dig Liv Dis. 2005;37:206–210

 Conflicts of interest The authors disclose no conflicts.

PII: S1542-3565(09)00655-7

doi:10.1016/j.cgh.2009.07.007

Clinical Gastroenterology and Hepatology
Volume 8, Issue 2 , Pages e15-e16, February 2010