Primary hepatic Burkitt’s lymphoma as firstmanifestation of HIV–AIDS in a hepatitis Bseropositive adult: when defences failAntonio Jose Reyes,1,2 Kanterpersad Ramcharan,3,4 Samuel Aboh,2 Wesley Greaves5

1Department of Medicine,Neurology Unit, San FernandoTeaching Hospital, SanFernando, Trinidad and Tobago2Department of InfectiousDisease Unit/Medicine, SanFernando Teaching Hospital,San Fernando, Trinidad andTobago3Department of Medicine,San Fernando TeachingHospital, San Fernando,Trinidad and Tobago4Department of Medicine,Surgi-Med Clinic, SanFernando, Trinidad and Tobago5Department of Pathology,San Fernando TeachingHospital, San Fernando,Trinidad and Tobago

Correspondence toDr Kanterpersad Ramcharan,Department of Medicine, SanFernando Teaching Hospital,San Fernando, Trinidad andTobago, West Indies;kramcharan79@yahoo.com

Accepted 11 October 2016

To cite: Reyes AJ,Ramcharan K, Aboh S, et al.BMJ Case Rep Publishedonline: [please include DayMonth Year] doi:10.1136/bcr-2016-217620

DESCRIPTIONA previously well 32-year-old heterosexual manpresented with progressive wasting syndrome, jaun-dice, night sweats, vomiting and abdominal painfor 1 month. There was no history of recreationaldrug use or exposure to hepatotoxins. Examinationshowed normal vital signs, a body mass index of18 kg/m2, generalised muscle atrophy, oral thrushand no lymphadenopathy with an enlarged liver14 cm below the costal margin at the midclavicularline. The physical examination was otherwisenormal.Serology for HIV enzyme-linked immune sorbent

assay (ELISA), Western blot immunoassay tests andhepatitis B surface antigen were all seropositiveconfirming new onset HIV–AIDS with hepatitis Bcoinfection. Extensive investigations for other dis-eases were negative and relevant results are shown(table 1). The HIV viral load was high at 300 392RNA copies/mL and the CD4+ T-cell count waslow at 116 cells/mL (reference values 410–1590).Serum creatinine was also high at 4.2 mg/dL andblood urea nitrogen was high at 43 mg/dL (refer-ence value 3–20 mg/dL) suggesting renal dysfunc-tion (table 1).On ultrasonography, the liver measured 22.5 cm

with multiple heteroechoic lesions in both lobes,with the largest lesion being 7.8×5.3 cm in diam-eter (figure 1). CT scan of the chest, abdomen andpelvis showed multiple hypovascular hepatic lesionsof 2.3×1.7 cm in size in axial and coronal views(figure 2A, B). Ultrasound-guided biopsy withhistopathology using routine H&E staining andimmunohistochemistry confirmed Burkitt’s lymph-oma (figure 3A–E).Diagnosed as primary hepatic Burkitt’s lymph-

oma (PHBL) stage I B and HIV-associatednephropathy as the first manifestation of HIV–AIDS with hepatitis B seropositivity, he was clas-sified as Child-Pugh class A and was treated withblood transfusions, highly active antiretroviraltherapy (HAART) and renal replacement therapy(table 2). Two months later, clinical improvementensued with the HIV viral load dropping to38 567 RNA copies/mL, and a CD4+ T-cellcount increased to 258 cells/mL. His refusal offurther care led to discharge for follow-up but hewas readmitted 3 weeks later with severe uraemia(serum creatinine 11.2 mg/dL and urea 108 mg/dL)

Table 1 Medical investigations

Blood test ResultReferencerange

WCC 1.3×109/L 4.5–11.0×109/L

HGB 3.2 g/dL 14.0–17.5 g/dL

MCV 83.2 fL/red cell 80–96 fL/red cell

Platelets count 49×103/mL 156–373×103/mL

Serum potassium 7.1 mmol/L 3.5–5.1 mmol/L

Serum sodium 117 mmol/L 135–145 mmol/L

Serum creatinine 4.2 mg/dL 0.5–1.2 mg/dL

BUN 43 mg/dL 3–20 mg/dL

Uric acid 18 mg/dL 2.5–8 mg/dL

Alanine aminotransferase 152 IU/L 20–60 IU/L

Aspartate aminotransferase 48 IU/L 5–40 IU/L

γ-glutamyl transpeptidase 219 IU/L 8–61 IU/L

Lactate dehydrogenase 997 IU/L 105–333 IU/L

Alkaline phosphatase 403 IU/L 40–129 IU/L

Total bilirubin 7.3 mg/dL 0.2–1.3 mg/dL

Direct bilirubin 6.9 mg/dL 0.0–0.4 mg/dL

Albumin 2.6 g/dL 3.5–5.5 g/dL

Albumin-corrected calcium 18.94 mg/dL 9.6–11.2 mg/dL

CRP 11.1 mg/dL 0.0–1.0 mg/dL

β-2-microglobulin 3.1 mg/L <2 mg/L

International normalisedratio

1.30 0.5–1.1

ELISA for HIV Reactive Non-reactive orreactive

Western blot test for HIV Positive Positive or negative

HIV viral load on admission 300,392 RNAcopies/mL

HIV viral load 2 monthsfrom admission

38 567 RNAcopies/mL

CD4+ T-cell count onadmission

116 cells/mL 410–1590 cells/mL

CD4+ T-cell count 2 monthsfrom admission

258 cells/mL 410–1590 cells/mL

EBV IgG antibodies >200 Positive: >22

EBV IgM antibodies 0.1 Negative: ≤0.8Hepatitis B surface antigen Positive Positive or negative

Hepatitis C IgG antibodies Negative Positive or negative

Hepatitis C IgM antibodies Negative Positive or negative

PCR for viral infections Tests notobtained

Negative or positive

Lumbar puncture for CSFanalysis

Consent wasdeclined

Normal or abnormal

Bone marrow aspirationbiopsy

Consent wasdeclined

Normal or abnormal

BUN, blood urea nitrogen; CRP, C reactive protein; CSF,cerebrospinal fluid; EBV, Epstein-Barr virus; ELISA, enzyme-linkedimmune sorbent assay; HGB, haemoglobin; MCV, mean corpuscularvolume; WCC, white cell count.

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and prolonged international normalised ratio (2.1). He claimedadherence to HAART. Renal tract ultrasonography then showedfeatures in the right (figure 4A) and left kidney (figure 4B) ofgrade III HIV-associated nephropathy without hydronephrosis.

The patient deteriorated (table 2) and died from a massiveupper gastrointestinal bleed. A postmortem was not obtained.

The aetiology of PHBL is also unknown. Recent reports havedescribed cases of PHBL in patients with hepatitis C or B virusinfection, Epstein-Barr virus (EBV), HIV or other patients withimmune suppression. These factors can have a profound impact onpatient care and prognosis such as in our case because this diseaseentity often responds to therapy with hyperfractionated cyclophos-phamide, vincristine, doxorubicin, dexamethasone, methotrexateand cytarabine combination (hyper-CVAD/MTX-Ara-C) and con-comitant rituximab. A careful pathological examination with ancil-lary studies including immunohistochemistry, flow cytometry andkaryotyping may lead to accurate diagnosis.1 2

Renal biopsy remains the standard for the diagnosis ofHIV-associated nephropathy but renal hyperechogenicity on ultra-sound when compared with histopathology has a sensitivity andspecificity reported as 96% and 51%, respectively with the‘decreased renal sinus fat’ sign seen in up to 49% of patients.3

PHBL as the initial manifestation of HIV–AIDS with hepatitis Bpositivity and renal disease in an adult has been seldom reported.1 2

Figure 2 (A) CT scan axial view of the abdomen showing same withgross hepatomegaly and multiple hypovascular lesions. (B) CT scancoronal view of the abdomen performed on admission showed multiplehypovascular hepatic lesions of 2.3×1.7 cm in size.

Figure 1 Ultrasound scan of the abdomen performed on admissionshowed multiple heteroechoic hepatic lesions with the largestmeasuring 7.8×5.3 cm in diameter.

Figure 3 (A) Tumour photomicrography: H&E staining showing cellularsmears comprising of monomorphic population of lymphoid cellsrevealing high mitotic activity (10× magnification). (B) H&E staining withlarge irregular nuclei, and multiple nucleoli (40× magnification). (C) Onimmunohistochemistry, the tumour cells showed expression of thepan-B-cell marker PAX5 (40× magnification). (D) The tumour markersCD3 was negative (40× magnification). (E) The tumour marker Ki67 waspositive (showing a proliferation index >95) (40× magnification).

2 Reyes AJ, et al. BMJ Case Rep 2016. doi:10.1136/bcr-2016-217620

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Learning points

▸ Primary hepatic Burkitt’s lymphoma (PHBL) is an extremelyrare and aggressive form of extranodal lymphoma but isunusual as an initial manifestation of HIV–AIDS withhepatitis B seropositivity in an adult.

▸ To date, at least 15 cases of PHBL in adults have beenreported in the literature; all of the 11 patients withoutconcurrent HIV infection had the sporadic form of the disease.

▸ Definite diagnostic or specific imaging criteria for diagnosingPHBL have not been established. The clinical manifestationsarise from liver infiltration by B cells and there must be noevidence of lymphomatous involvement in the spleen, lymphnodes, bone marrow or other lymphoid structures withabsence of leukemic blood involvement in the peripheralblood smear.

▸ Early diagnosis of PHBL can have a profound impact onpatient care and improves prognosis.

▸ Renal ultrasound scan can help predict a non-invasivediagnosis of HIV-associated nephropathy as renalhyperechogenicity has a strong correlation withhistopathological confirmation.

Contributors AJR conceived the report and wrote the first draft. The design,acquisition of data, analysis and interpretation were performed by AJR, KR, SA andWG. All authors read and contributed to the final version of the manuscript. KRtakes responsibility for the integrity of the final contents.

Competing interests None declared.

Table 2 Medical treatment

Dosage Period of treatment

Intravenous therapyPacked red blood cells 1 unit 3 times a week 2 weeksFresh frozen plasma 4 units stat 1 occasionPantoprazole 80 mg infusion 3 times daily 2 daysZoledronic acid 4 mg diluted in 100 mL of isotonic solution intravenous infusion over 30 min 1 occasionNormal saline intravenous solution 1 L daily 1 weekCalcium gluconate 10% 10 mL diluted in 10 mL of isotonic solution intravenous infusion over 10 min 3 different occasions (repolarisation)Dextrose 50% 25 mL intravenous push stat 3 different occasions (repolarisation)Regular insulin 10 units intravenous push stat 3 different occasions (repolarisation)

Subcutaneous drugErythropoietin 4000 units 3 times per week 2 months

Oral drugEfavirenz 600 mg daily 2 monthsAbacavir 200 mg 2 times a day 2 monthsLamivudine 150 mg as first dose, then 75 mg daily 2 monthsPantoprazole 40 mg once daily 2 weeksTrimethoprim–sulfamethoxazole 80 mg/400 mg per tablet, 2 tablets 2 times a day 6 weeksNystatin suspension 1 mL 3 times daily 7 daysCalcium carbonate 1 g 2 times a day 2 monthsCalcitriol 0.25 mg once daily 2 monthsFolic acid 5 mg orally once daily 2 monthsFerrous sulphate 200 mg once daily 2 monthsSodium polystyrene 15 g 3 times daily 2 monthsAllopurinol 100 mg 2 times a day 1 monthMetronidazole 500 mg 3 times daily 2 weeks

Lactulose 15 mL 3 times daily 2 weeksMetoclopramide hydrochloride 10 mg 2 times a day 2 weeks

Stat, administered immediately without delay.

Figure 4 Renal tract ultrasound performed on second admissionshowed (A) right kidney and (B) left kidney with positive features ofHIV-associated nephropathy such as increased renal parenchymalechogenicity bilaterally with loss of corticomedullary differentiationgrade III and a decrease in the amount of renal sinus fat.

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Patient consent Obtained.

Provenance and peer review Not commissioned; externally peer reviewed.

REFERENCES1 Singh S, Kundu PR, Tanwar P, et al. Primary hepatic Burkitt’s lymphoma in an

immunocompromised adult. Clin Cancer Investig J 2013;2:253–5.

2 Modi G, Madabhavi I, Patel A, et al. Primary hepatic Burkitt’slymphoma: a bizarre site and triumph tale. J Clin Exp Hepatol 2015;5:159–62.

3 Sekiguchi Y, Yoshikawa H, Shimada A, et al. Primary hepaticcircumscribed Burkitt’s lymphoma that developed after acute hepatitis B:report of a case with a review of the literature. J Clin Exp Hematop2013;53:167–73.

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