Physical examination on final admission revealed dullness on percussion and fine crackles on auscultation in the lower lung fields, and also cervical, axillary, and inguinal lymphadenopathy, scleral and sublingual jaundice, and bilateral pitting leg edema. Abdominal examination revealed no masses, no hepatosplenomegaly, and no evidence of ascites.

Admission laboratory tests showed white blood cells 18.10×10⁹/L (normal: 4.80–10.80×10⁹/L), hemoglobin 100 g/L (normal: 120–160 g/L), platelets 325×10⁹/L (normal: 140–440×10⁹/L), sodium 135 mmol/L (normal: 136–147 mmol/L), and potassium 3.4 mmol/L (normal: 3.5–5.0 mmol/L). Liver function tests revealed total bilirubin 103.7 μmol/L (normal: 1.7–18.9 μmol/L), direct bilirubin 69 μmol/L (normal: 1.7–8.6 μmol/L), aspartate aminotransferase 225 U/L (normal: 10–31 U/L), alanine aminotransferase 217 U/L (normal: 10–31 U/L), alkaline phosphatase 615 U/L (normal: 53–141 U/L), gamma glutamyl transferase 478 U/L (normal: 7–50 U/L), and lactate dehydrogenase 434 U/L (normal: 110–210 U/L). Serology was negative for Epstein–Barr virus, cytomegalovirus, and hepatitis A, B, and C viruses. Screening for alcohol and acetaminophen in the blood was negative. Testing for Wilson disease, hemochromatosis, and α1-antitrypsin deficiency was unrevealing.

Imaging of the abdomen by computed tomography (ct) and ultrasonography revealed massive retroperitoneal lymphadenopathy, a normal liver and spleen, and no evidence of hepatic vascular obstruction. There was also no evidence of gallstones nor of biliary obstruction, the latter confirmed by magnetic resonance cholangiopancreatography. Doppler ultrasound of the legs showed no deep-vein thrombosis. Imaging of the chest by ct revealed areas of consolidation consistent with pneumonia, and a course of broad-spectrum antibiotics was started, with resolution of the patient’s respiratory symptoms.

Liver biopsy revealed cholestasis, moderate steatosis, and in the portal spaces, prominent small lymphocytic infiltrates that co-expressed CD20, Bcl2, CD5, CD43, and faintly CD23, consistent with a diagnosis of cll (Figure 1). There was no evidence of progression to a large B-cell lymphoma, thus ruling out a Richter transformation. A diagnosis of liver failure because of leukemic infiltration was made, and the patient was started on fludarabine, cyclophosphamide, and rituximab on hospital day 13.

Based on a review of the current literature, only three cases of liver failure have so far been associated with cll. Greer et al. ⁴ described a case of acute liver failure attributable to organ infiltration by a T-cell cll. The patient’s liver failure was unresponsive to chemotherapy, and he died of gastrointestinal bleeding and hepatorenal failure on hospital day 8. Shehab et al. ⁵ reported a case of acute liver failure caused by infiltration of a B-cell cll which had undergone a Richter transformation to a diffuse large B-cell lymphoma. Finally, Costa et al. ⁶ described a case of chronic liver failure that occurred over the span of 3 months because of hepatic venous outflow obstruction secondary to B-cell cll infiltration of the liver. In the latter two cases, a course of chemotherapy resulted in reversal of liver failure and prompt resolution of symptoms.

Unlike the abovementioned cases, our patient had a cll of B-cell origin without any evidence of Richter transformation on biopsy. Our case also differs from the Costa case because the liver failure was acute, not chronic, and not secondary to hepatic venous obstruction. Furthermore, unlike the cases presented by Shehab and Costa, our patient’s symptoms were unresponsive to chemotherapy.

Usually, cll tends to be an indolent disease with chronic infiltration of the liver. However, in the setting of a sudden and rapid clinical deterioration, histologic transformation of the cll to a large B-cell lymphoma should be suspected. This transformation is heralded by sudden clinical deterioration, characterized by increasing lymphadenopathy, worsening of systemic symptoms, extranodal involvement, and elevated serum lactate dehydrogenase ²³. Although the patient presented here had the symptoms of a Richter transformation, liver biopsy failed to support such a diagnosis.