Chronic lymphocytic leukemia (CLL) is a malignancy characterized by clonal proliferation and accumulation of B lymphocytes. Although leukaemic infiltration of the kidney is well recognized in CLL, acute renal failure (ARF) due to leukaemic infiltration is extremely rare. Here we present a case of ARF as the initial manifestation of CLL. The diagnosis was made by a kidney biopsy. Treatment with cyclophosphamide and prednisolone resulted in a completely improved renal function.
Chronic lymphocytic leukemia (CLL) is a variety of chronic lymphoproliferative disorders. It is characterized by a progressive accumulation of monoclonal incompetent lymphocytes and is similar to peripheral B-cell neoplasm small lymphocytic lymphoma [
A 64-year-old man was admitted to our clinic with a 2-week history of nausea and vomiting. His past medical history included diabetes mellitus type 2 and hypertension controlled on metformin and irbesartan. He had no history of any renal disease. On physical examination the patient’s blood pressure, pulse rate, respiratory rate, and body temperature were 110/70 mm Hg, 96 beats/minute, 14 breaths/minute, and 37°C, respectively. His physical examination also revealed splenomegaly with no evidence of hepatomegaly and lymphadenopathy. The fundus examination was normal. There were no other abnormal findings. Initial laboratory data showed the following values: leukocyte count was 18.6 × 109/L (lymphocytes %65), hemoglobin 78 g/L, thrombocytes 265 × 109/L, blood urea nitrogen 48 mg/dL, serum creatinine 2.2 mg/dL (estimated GFR: 38 mL/min/1.73 m2), sodium 137 mEq/L, potassium 3.2 mEq/L, lactate dehydrogenase 378 U/L, uric acid 4.5 mg/dL, albumin 3.7 g/dL, gamma globulin level 2.5 g/dL, and erythrocyte sedimentation rate 51 mm/h. Serology was negative for antinuclear antibody, antineutrophil cytoplasmic antibody (ANCA), antiglomerular basement membrane antibody, HBsAg, and anti-HCV. Serum complement (C3 and C4) levels were normal and cryoglobulin was negative. Both monoclonal immunoglobulin levels and free light chain analysis for kappa and lambda in serum and urine were normal. Urine analysis showed only mild proteinuria (24 h protein excretion was 100 mg) without dysmorphic cells or casts. Blood smear revealed mild mature lymphocytosis. Sinus rhythm was observed on electrocardiogram. An abdominal ultrasonography (USG) revealed normal kidney size. A bone marrow biopsy was consistent with CLL and these monoclonal B lymphocytes were determined by means of immunological markers: CD5, CD20, CD23, and CD79 (Figures
The laboratory data on admission and during treatment.
Parameter | Admission | 7th day of treatment | 15th day of treatment | 21st day of treatment | At the end of treatment |
---|---|---|---|---|---|
Blood pressure (mmHg) | 110/70 | 115/75 | 120/80 | 115/75 | 120/70 |
Urine volume/24 hours (cc) | 1200 | 1100 | 1400 | 1350 | 1300 |
Body weight (kg) | 60 | 61 | 62 | 61 | 61 |
Peripheral lymphocyte count (109/L) | 18.6 | 17.9 | 16.2 | 14.3 | 12.2 |
Glucose (mg/dL) | 98 | 93 | 95 | 101 | 90 |
Creatinine (mg/dL) | 2.2 | 2.5 | 1.9 | 1.5 | 1.2 |
Sodium (mEq/L) | 137 | 139 | 135 | 137 | 140 |
Potassium (mEq/L) | 3.2 | 3.8 | 3.7 | 3.8 | 4.1 |
Calcium (mg/dL) | 8.7 | 9.5 | 9.2 | 9.3 | 8.9 |
Phosphate (mg/dL) | 3.1 | 3.7 | 3.3 | 3.5 | 2.9 |
Albumin (g/dL) | 3.2 | 3.5 | 3.4 | 3.7 | 3.8 |
Uric acid (mg/dL) | 4.5 | 4.4 | 4.2 | 4.6 | 4.7 |
(a) Bone marrow biopsy revealed nodular paratrabecular and intertrabecular infiltration with small lymphocytes which appeared as mature cells (HE ×200). (b) Monoclonal B lymphocytes were positive for CD5, CD20, CD23, and CD79 in bone marrow (after the specimens were fixed in 10% formalin and embedded paraffin wax the immunohistochemical staining was performed using a Ventana immunostainer (Ventana Benchmark XT, ×200)).
(a) Monotonous small lymphocytic infiltration in renal parenchyma (HE ×200). (b) Lymphocytic infiltration showing immunopositivity for CD5, CD20, and CD79 (after the specimens were fixed in 10% formalin and embedded paraffin wax the immunohistochemical staining was performed using a Ventana immunostainer (Ventana Benchmark XT, ×200)).
Chronic lymphocytic leukemia (CLL) is a neoplastic condition of B-cells, and it usually affects the lymph nodes, liver, spleen, and bone marrow [
Acute renal failure in CLL patients can be associated with acute tubular necrosis, uric acid nephropathy, light chain nephropathy, obstructive nephropathy, amyloidosis, hypercalcemia, glomerulonephritis, and cryoglobulinemia [
The treatment options include steroids, chlorambucil, vincristine, cyclophosphamide, rituximab, fludarabine, and irradiation of the kidneys or a combination therapy consisting of these agents. The response is variable from complete response to a partial or no response [
In general, the preferred treatment modality for CLL is RFC (rituximab + fludarabine + cyclophosphamide) protocol in our clinic. Rituximab could not be used because of reimbursement issues regarding this drug in these patients as an initial treatment in our country. Use of fludarabine was contraindicated in our patient because of Coombs positive hemolytic anemia. Hence, a combination of cyclophosphamide and methylprednisolone was preferred in our patient and the patient responded very well with regression of renal dysfunction and an improvement in the peripheral lymphocyte count, meaning complete remission.
From our case report and the other few cases reported in the literature, we suggest that leukaemic infiltration in the kidneys should be kept in mind when a patient with CLL presents with ARF. Acute renal failure can also occur as the first presentation of CLL like the present case. A kidney biopsy is required for diagnosis. Keeping this possibility in mind is important because it responds very well to chemotherapy, as in our case treated successfully with cyclophosphamide and methylprednisolone.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
The authors declare that there is no conflict of interests regarding the publication of this paper.