Severe lactic acidosis, hypertriglyceridemia, and extensive axial skeleton involvement in a case of disseminated Burkitt’s lymphoma

Abstract

A 12-year-old boy presented with fever for 3–4 months, abdominal pain, weakness, and mild lower-back pain for 15 days. He had tachycardia, tachypnea (46/min), pallor, edema-feet, and tender hepatomegaly (10 cm below right costal margin). There was no splenomegaly or lymphadenopathy. Rest of the systemic examination was normal. Investigations showed anemia (7.9 g/dL), leucocytosis (19.8 9 10/L, 88% polymorphs), normal platelets (260 9 10/L), hypoalbuminemia (2.4 g/dL), normal AST/ ALT (56/91 U/L), normal blood sugar levels, hyperuricemia (12.3 mg/dL) and elevated alkaline phosphate (346 U/L), lactate dehydrogenase (1956 U/L), ferritin (2973 ng/mL), and fasting serum triglycerides (651 mg/dL). Blood gas showed metabolic acidosis (pH 7.25, pO2 82 mmHg, pCO2 14 mmHg, HCO3 5.2 mmol/L, Base excess 17.1) and elevated levels of serum lactate (21 mmol/L). Computerized tomographic scan revealed hepatomegaly with multiple contrast enhancing nodules, bilateral renal enlargement and multiple lytic areas in skull, humerus, scapula, ribs, vertebrae, pelvis, and femur (Fig. 1). Liver biopsy showed cells containing scanty cytoplasm and pleomorphic hyperchromatic nuclei, with dense chromatin and inconspicuous nucleoli arranged in sheets of small and large clusters (Fig. 2). The tumor cells were positive for surface IgM, CD45, CD20, CD10, Ki-67; negative for cytokeratin, CD3 and terminal deoxynucleotidyl transferase; and MIB 1 index was 99.5%, consistent with Burkitt’s lymphoma (BL). Uninvolved liver parenchyma showed micro and macrovesicular steatosis. Bone marrow revealed nodular infiltration by malignant cells having high nucleo-cytoplasmic ratio, vacuolar nucleus, and prominent nucleoli. Immunohistochemistry markers were consistent with BL. Cerebrospinal fluid examination revealed no malignant cells. Epstein–Bar virus IgM and IgG were negative. Retroviral serology was negative. The child was started on MCP842 protocol for non-Hodgkin lymphoma (NHL). With aggressive bicarbonate therapy and following chemotherapy, the lactic acidosis resolved. The child developed hypocalcemia, hyperphosphatemia, azotemia and hyperuricemia, which were managed conservatively. Subsequently, the child developed febrile neutropenia with catheter-related blood-stream acinetobacter infection, which was managed as per standard protocols. The child had a successful recovery. Appendicular and facial bone involvement has been well described in BL. Only one case of scapular involvement has been reported [1]. Osteolytic lesions predominantly in the axial skeleton in our patient were noteworthy. Such widespread involvement of skull, humerus, scapula, ribs, K. Kulkarni L. S. Arya Division of Pediatric Oncology, Apollo Center for Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi, India