A Case of Burkitt's Lymphoma in a Child Presenting with Obstructive Jaundice and Requiring Surgical Intervention

Introduction

Burkitt's lymphoma is a high-grade B non-Hodgkin lymphoma (NHL) with a high proliferative index and is exquisitely sensitive to chemotherapy. Jaundice is a rare presentation of NHL and has been reported in approximately 0.4 to 3% of pediatric cases.¹ In most instances, hyperbilirubinemia occurs due to external compression of biliary system or infiltration of hepatic parenchyma. Hence jaundice usually resolves with administration of chemotherapy and surgical drainage is rarely required.

We report the case of a 6-year-old boy, with abdominal Burkitt's lymphoma presenting with marked obstructive jaundice and discuss associated challenges in administering optimum chemotherapy as well as need for surgical intervention.

Case Report

A 6-year-old male child presented with a 2-month history of intermittent fever, yellowish discoloration of eyes, high-colored urine, and distension of the abdomen. He was presumptively treated for viral hepatitis with indigenous medicines. Abdominal ultrasound scans followed by needle aspiration were suggestive of a malignancy. Thereafter the child was referred to us for further management.

On examination, the child was conscious, oriented, deeply icteric, pale, emaciated (weight < 5th percentile for age), and hemodynamically stable. There were no lymphadenopathy, pedal edema, skin rash, or signs of liver failure. Systemic examination revealed a distended abdomen with a firm, nontender mass in the epigastric region extending to the right hypochondrium and hypogastric region. Rest of the examination was unremarkable.

The patient was thoroughly investigated with laboratory and imaging studies (Table 1). Computed tomography (CT) of the abdomen revealed a large nodal mass in the region of the pancreatic head causing common bile duct obstruction (Fig. 1).

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Fig. 1 (A) Computed tomography of the abdomen showing large lobulated mass near pancreatic head, causing abrupt cutoff of distal common bile duct. (B) Computed tomography of abdomen showing dilated intrahepatic biliary radicals post first cycle of chemotherapy. (C) Serum bilirubin trend during treatment. (D) Percutaneous biliary drainage, catheter could not be negotiated beyond common bile duct. COP, cyclophosphamide, vincristine, prednisolone; ERCP, endoscopic retrograde cholangiopancreatogram; PTBD, percutaneous transhepatic biliary drainage; RCOP, rituximab, cyclophosphamide, vincristine, prednisolone.

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A transduodenal endoscopic biopsy confirmed the diagnosis, and after staging workup, a final diagnosis of primary abdominal Burkitt's lymphoma, stage 3 (Murphy's staging), group B (LMB) was made.^2,3,4 Tumor lysis prophylaxis was initiated, and chemotherapy as per LMB-96 protocol was planned. The chemotherapy schedule for group B disease includes cytoreductive phase COP (cyclophosphamide, vincristine, prednisolone), followed by two cycles each of COPADM (COP, Adriamycin, and methotrexate) and CYM (cytarabine and methotrexate) and one cycle of maintenance COPADM. In view of the elevated serum bilirubin, doses of vincristine, Adriamycin, and methotrexate were withheld as per the guidelines of the protocol and the NCCN (National Comprehensive Cancer Network).⁵ Because of compromise in the chemotherapy schedule, rituximab was added to the initial three cycles (R-COP and R-COPADMx2) and CYM was substituted with CYVE (cytarabine, etoposide).

Clinically, the patient responded well to treatment; however, high levels of serum bilirubin persisted. Two attempts at endoscopic retrograde cholangiopancreatogram (ERCP) were unsuccessful after which percutaneous biliary drainage (PBD) was done. The catheter could not be negotiated beyond the CBD. This reduced the bilirubin from 19 to 7.2 mg/dL and enabled us to give consolidation therapy. Response evaluation after three cycles of chemotherapy revealed complete response. However, the clinical course of the patient was quite turbulent with multiple episodes of febrile neutropenia during which the hepatopathy worsened. After completion of chemotherapy courses, hyperbilirubinemia and biliary dilatation persisted. Two months later, a side-to-side choledochoduodenostomy was performed. Presently the child is 4 years off therapy and disease free, with normal liver functions.

Discussion

Jaundice is a known though uncommon initial presentation of pediatric NHL.⁶ Various postulated mechanisms include direct involvement of the liver, extrinsic lymph nodal compression, intrahepatic cholestasis, and toxic hepatitis due to drugs and or hemolysis. The porta-hepatis and hepatopancreaticoduodenal regions, where the bile ducts are less mobile, are particularly vulnerable to compression and obstructive jaundice.¹ In this case as well, extrahepatic biliary obstruction was due to enlarged lymph nodes at the porta hepatis. The transaminitis might have been due to drugs administered to the patient prior to diagnosis and/or possible infiltration by lymphoma. This along with episodes of febrile neutropenia during chemotherapy treatment may have led to infection/inflammation in the biliary system, subsequently culminating in healing by fibrosis and development of bile duct stricture.

Burkitt's lymphoma in children has excellent cure rates, and chemotherapy is the mainstay of treatment. The presence of hepatopathy poses a challenge to the treating oncologists as most of the indicated cytotoxic agents require omission or dose modification. Rituximab is a chimeric monoclonal antibody against the protein CD20 present on the surface of B-NHL cells. When used in combination with multiagent chemotherapy, it has been found to be efficacious in its treatment.⁷ Its safety profile in patients with deranged liver function prompted us to add it to our compromised chemotherapy schedule.⁸

Burkitt's lymphoma is a chemotherapy sensitive disease; hence, jaundice was expected to resolve with the initiation of antilymphoma therapy. However, persistent hepatopathy necessitated a biliary drainage procedure in parallel. As two attempts at ERCP failed, PBD was done, which dramatically brought down the serum bilirubin, enabling delivery of consolidation chemotherapy in optimum doses. Various techniques for biliary decompression include external-internal drain, stent placement, or laparotomy at presentation. Although these treatments may relieve the obstruction and associated symptoms, they may cause crucial delay in definitive treatment. In a disease, as aggressive as Burkitt's lymphoma, it is equally important to avoid delay and initiate antilymphoma therapy urgently. Establishing the cause of obstructive jaundice is very critical as it determines the approach to management and influences the ability to deliver optimum therapy. In a retrospective review of 35 adult lymphoma patients with obstructive jaundice, serum bilirubin normalized in 85% of those who underwent ERCP or PBD.⁹ Choledochoduodenostomy was done after the completion of all planned therapy to prevent cholestatic liver damage.

Conclusion

Obstructive jaundice is a rare initial presentation of NHL. If the serum bilirubin does not rapidly normalize with treatment, it is important to proceed with biliary drainage. Early decompression allows for optimum delivery of curative chemotherapy and prevents chronic liver damage.