Primary bilateral T-cell renal lymphoma presenting with sudden loss of renal function

Edmond O'Riordan1,, Roy Reeve2, John B. Houghton3, Donal J. O'Donoghue1 and Steven Waldek1

1 Departments of Renal Medicine, 2 Pathology and 3 Haematology, Hope Hospital, Salford, UK

Keywords: acute renal failure; bilateral; chemotherapy; primary T-cell renal lymphoma; renal function loss

Introduction

Secondary involvement of the kidneys occurs in ~50% of patients with lymphoma, as shown by autopsy studies [1]. The existence of primary renal lymphoma has been a subject of debate due to the rarity of the cases and the inability to rule out absolutely the presence of microscopic foci of tumour elsewhere [1,2]. Fourteen percent of post-transplant lymphoproliferative disorders are of T-cell origin; however, T-cell lymphomas are rarely found in native kidneys. We could find only 10 definite cases of infiltrative T-cell lymphoma presenting with acute renal failure. We report this rare case as it demonstrates the rapid onset of this disease and the poor patient outcome, despite good renal response to intensive chemotherapy.

Case

We report the case of a 52-year-old man who presented to the emergency department in April 1999 with acute renal failure. This patient had an adrenal adenoma excised 6 months previously for hypertension due to Conns syndrome. Consequently the patient was under regular follow up with the nephrology service at our hospital. A serum creatinine estimation 2 months prior to presentation in the outpatient department showed serum creatinine to be 78 µmol/l. The patient was asymptomatic at this time.

Ten days prior to presentation the patient had experienced dull, bilateral loin pain. This resolved after 4 days but the patient then developed progressive ankle swelling, weight gain, and reduced urinary volume. Physical examination was normal apart from mild obesity. Blood pressure was 153/89 mmHg and urinalysis revealed ++ of protein but no haematuria. Admission biochemistry confirmed acute renal failure, with serum creatinine 363 µmol/l and urea 17.7 mmol/l. Haematological indices were within normal parameters with Hb 13.3 g/dl, WCC 4.7, and platelets 184. Lactate dehydrogenase was elevated at 367 U/l. Autoantibody screen including ANCA and anti-GBM antibodies were negative. C-reactive protein was elevated at 12 and erythrocyte sedimentation rate elevated at 35 mm/h. An urgent renal ultrasound was performed which showed bilateral large kidneys 15 cm in length. Renal function continued to deteriorate after admission, peaking 3 days later at a creatinine of 463 µmol/l, urea of 20.1, and a potassium of 5.7.

Creatinine clearance stabilized at 22 ml/min with a urinary protein of 0.4 g/24 h.

Diagnostic renal biopsy was performed. This showed almost complete destruction of the renal architecture by malignant lymphoid cells. These cells were characterized by large nuclei with scanty cytoplasm. Large numbers of mitoses were visible. These subsequently stained positive for the T-cell marker (CD3).

Computerized tomography (CT) of the thorax, abdomen, and pelvis, and bone marrow aspirate and trephine were performed to stage the lymphoma. Apart from diffusely enlarged kidneys, with no focal alteration of attenuation, these investigations were unremarkable.

The patient commenced VAPEC-B chemotherapy and was treated with this regimen for 7 weeks. Initial therapy was complicated by hyperkalaemia due to tumour lysis but regular biochemical checks and prompt medical management obviated the need for dialysis. Two weeks after admission the patient was discharged with a serum creatinine of 324 µmol/l. Three weeks after discharge the creatinine was 158 µmol/l and 6 weeks post-discharge it was 123. The measured creatinine clearance improved in similar fashion to 82 ml/min at 6 weeks and 84 ml/min at 15 weeks. Proteinuria decreased to 0.1 g/24 h during this time. Twelve weeks after initial presentation the patient was biopsied again. This re-biopsy showed complete resolution of tumour. A repeat CT scan performed at 14 week after biopsy showed no evidence of disease recurrence. Consolidation therapy was then given with three doses of adriamycin and cyclophosphamide at 3-weekly intervals.

At review 6 months after discharge from hospital, the patient remained well with a creatinine clearance of 105 ml/min and 24-h urinary protein excretion of 0.1 g/day.

Eight months after presentation the patient presented with a right-sided Bell's palsy, which subsequently became bilateral. He then developed difficulty with swallowing and became hoarse, suggesting palsy of the IX and X cranial nerves. Physical examination suggested bilateral III and V nerve palsies. Laryngoscopy revealed a left vocal-cord paralysis. CT and magnetic resonance (MR) of the brain were completely normal. Lumbar puncture was performed. This showed a small number of atypical lymphocytes. A diagnosis of meningeal involvement was made and intrathecal (IT) methotrexate and oral dexamethasone were commenced. The symptoms initially responded well to treatment. A repeat CT scan of the thorax, abdomen, and pelvis showed no evidence of lymphoma. Eleven months after diagnosis the patient developed further worsening of his dysphonia and episodes of collapse. He failed to respond to an increased dose of dexamethasone. A repeat MR scan of the head showed no evidence of lymphoma, but lumber puncture revealed occasional lymphocytes. IT methotrexate was recommenced and high-dose systemic methotrexate was planned. However, 7 weeks after commencing this course, 13 months after initial presentation, the patient represented with malaise and dyspnoea. A chest X-ray revealed a right lower-lobe pneumonia and his WCC was 56 000x109/l. Blood film and bone marrow examination revealed acute T-cell lymphoblastic leukaemia (CD2, CD4, CD5, CD7, and CD8 positive). Despite intensive treatment, the patient succumbed 5 days later.

Discussion

Renal failure may result from a large variety of causes in patients with lymphoma, with lymphomatous infiltration and destruction of renal tissue a rare presenting finding. In the case report and review published by Truong et al. [3], 53 cases have been reported in the literature to 1987, with only 17 of those reported having no extra-renal involvement.

T-cell lymphomas are even more unusual, with 10 cases described in the literature, and only five of these have been in adult patients [48]. The case described by Miyake et al. [8] appears to be the only recorded instance of adult T-cell lymphoma presenting with isolated renal involvement. Although these tumours are rare, the paucity of reporting is undoubtedly because the technology of classifying these tumours into T or B cell has only recently become widely available.

The absence of lymphoid tissue in the normal renal parenchyma has led to controversy about the existence of primary renal lymphoma as a distinct disease. The rarity of cases reported and the poor prognosis, with dissemination of disease usually resulting in death, has added weight to this argument. However, others have argued that lymphoma may arise from the renal hilum or from foci of inflammation that attract lymphocytes to the area, such as pyelonephritis.

Interestingly, in another case of T-cell lymphoma, a small choroid plexus focus was found at autopsy, despite a normal CT scan [9]. Our case fulfils the criteria outlined by Malbrain et al. [2] for the diagnosis of primary extra-nodal lymphoma of the kidneys.

The response to treatment was extremely gratifying with an excellent recovery of renal function. The patient experienced tumour lysis syndrome after the first dose of chemotherapy. Hydration, in combination with allopurinol and bicarbonate prophylaxis, was given, but despite this the patient developed significant hyperkalaemia. This was managed medically but on a ward with acute haemodialysis facilities, which we feel are essential in the safe management of these patients. Estimation of serum potassium was performed 8 hourly for the first 4 days.

Patient outcome in bilateral primary renal lymphoma remains obscure due to the few reported cases in the literature. The reported outcome in cases treated with chemotherapy that had no extra-renal involvement at presentation is outlined in Table 1Go. The only other case of primary adult T-cell lymphoma presenting with renal failure was treated with MACOP-B and was disease free 17 months post-diagnosis [8].


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Table 1. Outcome in patients with bilateral primary renal lymphoma

 
In conclusion, lymphoma is a rare cause of acute renal failure. Although typically of B-cell origin in the non-immunosuppressed patient, T-cell tumours are now recognized. Renal function can respond very well to chemotherapy. Despite no evidence of disseminated disease at presentation, these patients have a poor prognosis, as demonstrated by our case and the limited available literature.

Notes

Correspondence and offprint requests to: Dr Edmond O'Riordan, Department of Renal Medicine, Hope Hospital, Salford M6 8HD, UK. Back

References

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Received for publication: 27.12.00
Revision received 8. 2.01.