|Year : 2018 | Volume
| Issue : 2 | Page : 94-95
Successful treatment with bortezomib and dexamethasone combination in a patient with monoclonal gammopathy of renal significance
Department of Hematology, School of Medicine, Baskent University, Istanbul, Turkey
|Date of Submission||11-Dec-2017|
|Date of Acceptance||08-Feb-2018|
|Date of Web Publication||7-Aug-2018|
Oymaci sk. No. 7 Altunizade, Uskudar, 34000
Source of Support: None, Conflict of Interest: None
A 40-year-old woman was reported to the nephrology outpatient clinic because of sudden-onset hypertensive attack, massive proteinuria, and high levels of creatinine. She had no previous medical history. Membranoproliferative glomerulonephritis and monoclonal κ light chain staining was seen on renal biopsy. She was evaluated for plasma cell diseases in hematology clinic. She was diagnosed as having monoclonal gammopathy of renal significance. Six courses of bortezomib plus dexamethasone were given to her. After this treatment schedule, her renal dysfunction and hypertension were resolved. Monoclonal gammopathy of renal significance is a disease caused by monoclonal immunoglobulins secreted by clonal B cells. Monoclonal gammopathy of renal significance should be considered with any unexplained renal impairment or proteinuria. Hematology department must be consulted as well. If treatment is delayed, permanent kidney damage can occur.
Keywords: glomerulonephritis, hypertension, immunoglobulin light chains, membranoproliferative, paraproteinemias, renal
|How to cite this article:|
Atalay F. Successful treatment with bortezomib and dexamethasone combination in a patient with monoclonal gammopathy of renal significance. Egypt J Haematol 2018;43:94-5
|How to cite this URL:|
Atalay F. Successful treatment with bortezomib and dexamethasone combination in a patient with monoclonal gammopathy of renal significance. Egypt J Haematol [serial online] 2018 [cited 2020 Mar 31];43:94-5. Available from: http://www.ehj.eg.net/text.asp?2018/43/2/94/238769
| Introduction|| |
Monoclonal gammopathy of renal significance (MGRS) is a disease caused by monoclonal immunoglobulins secreted by clonal B cells. It can lead to higher morbidity because of renal failure . This clinical situation can cause uncontrolled hypertension, proteinuria, and renal function abnormalities . If these abnormalities are observed, the patient must be rapidly evaluated and may be started on therapy to prevent permanent kidney damage .
Renal biopsy with immunocytochemistry, immunofluorescence, and electron microscopic evaluations must be applied . Hematological evaluation is critical to discover plasma cell disorders. Bone marrow biopsy, aspiration, and cytogenetics; serum and urine immunoelectrophoresis; serum free light chain (FLC) κ and λ levels; and κ/λ ratio should be examined . In this case report, we present a patient who was diagnosed with MGRS and successfully treated.
| Case|| |
In December 2014, a 40-year-old white female who had no previous disease history and took no medications was reported to the nephrology outpatient clinic because of sudden-onset hypertensive attack, proteinuria, and high levels of creatinine. She had no abnormality in physical examination except high blood pressure. Her blood pressure was 160/110 mmHg. Laboratory values revealed creatinine level 1.72 mg/dl (0.5–1.3), uric acid 7.5 mg/dl (2.5–6), albumin 4.3 g/dl (3.5–5.5) calcium 9.8 mg/dl (8.5–10.5), 24-h urine protein 1568 mg/day (0–300), hemoglobin 10.79 g/dl (12–16), and hematocrit 32.1% (35–46%). Kidney biopsy was performed and was compatible with membranoproliferative glomerulonephritis and monoclonal κ light chain staining. The patient returned to the hematology outpatient clinic after biopsy.
The patient was evaluated for plasma cell dyscrasias. No monoclonal gammopathy was revealed in serum and urine immunoelectrophoresis. Serum FLC κ values, serum FLC λ values, and κ/λ ratio were 1091.2, 690.506, and 1.58 mg/dl, respectively (0.33–1.94, 0.57–2.63, and 0.26–1.65). Bone marrow biopsy was performed twice. In both biopsies, 4% monoclonal κ-stained plasma cells were seen. No evidence of bone involvement and plasmacytoma was found on whole-body bone MRI and PET-computed tomography.
The patient was diagnosed with MGRS because of her mild renal failure and proteinuria, proliferative glomerulonephritis with κ monoclonal deposits, elevated levels of κ FLC in serum, and 4% monoclonal κ plasma cell infiltration of bone marrow. Six courses of bortezomib plus dexamethasone (1.3 mg/m2/day and 40 mg/day, days 1, 4, 8, and 11/21 days) were given. Following this treatment schedule, she was normotensive and had no complaints. Her laboratory values revealed creatinine level 1 mg/dl (0.5–1.3), uric acid 6.5 mg/dl (2.5–6), calcium 8.7 mg/dl (8.5–10.5), 24-h urine protein 263.5 mg/day (0–300), albumin 4.2 g/dl (3.5–5.5), hemoglobin 12.1 g/dl (12–16), hematocrit 37.2% (35? 46%), erythrocyte sedimentation rate 10 mmHg (<20). Serum FLC κ, serum FLC λ values, and κ/λ ratio were 4.4, 1.4, and 3.14 mg/dl, respectively. Her laboratory values revealed creatinine level 1 mg/dl (0.5–1.3), uric acid 6.5 mg/dl (2.5–6), calcium 8.7 mg/dl (8.5–10.5), 24-h urine protein 263.5 mg/day (0–300), albumin 4.2 g/dl (3.5–5.5), hemoglobin 12.1 g/dl (12–16), hematocrit 37.2% (35‑46%), erythrocyte sedimentation rate 10 mmHg (<20), and serum FLC κ, serum FLC λ values, and κ/λ ratio were 4.4, 1.4, and 3.14 mg/dl, respectively. She was considered in remission, and it was recommended that she should undergo autologous bone marrow transplantation. She declined the recommended treatment and followed up in the hematology outpatient clinic for 3 years. In the last control, her serum FLC κ, serum FLC λ values, and κ/λ ratio were 32.5, 3.22, and 10.99 mg/dl, respectively. She would like to wait and be seen again next month still.
| Discussion|| |
Monoclonal gammopathy of renal significance can be seen on various clinical tables. Renal biopsy can reveal immunoglobulin light and heavy chain amyloidosis, fibrillary glomerulonephritis, immunotactoid glomerulopathy, monoclonal immunoglobulin deposition disease [nonorganized substance accumulation (Randall type)], Fanconi syndrome, proliferative glomerulonephritis with monoclonal IgG deposits, C3 glomerulopathy with monoclonal gammopathy, proximal tubulopathy caused by light chain deposition, or cryoglobulinemic glomerulonephritis ,,. In this case, we found, during renal biopsy, that proximal tubulopathy-related light chain deposition and monoclonal plasma cell infiltration were observed in less than 10% of the bone marrow. According to the International Myeloma Working Group’s guideline, this patient could not be diagnosed with multiple myeloma . We diagnosed the patient with MGRS because she had monoclonal κ light chain deposition in glomeruli, high levels of creatinine, and less than 10% monoclonal plasma cell infiltration in her bone marrow. There are not yet any standard treatment protocolrecommendations for MGRS. Treatment goals must involve both renal disease and monoclonal B cell disease. The treatment decision should take into consideration the patient’s renal function . Bortezomib-based protocols can be used effectively in patients with renal insufficiency . Nambirajan et al.  reported a patient who was diagnosed with MGRS after renal transplantation and successfully treated with bortezomib. A bortezomib plus dexamethasone combination was used in this patient, and after six courses of chemotherapy, renal function and FLC levels were in normal range. High-dose chemotherapy with peripheral stem cell rescue remains controversial in young patients . Autologous bone marrow transplantation was recommended in this case, but the patient declined the procedure.
| Conclusion|| |
MGRS should be considered with any unexplained renal impairment or proteinuria. Hematology department must be consulted as well. A hematologist who suspects MGRS should perform the diagnostic workup recommended in the guidelines . If treatment is delayed, permanent kidney damage can occur .
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Conflicts of interest
There are no conflicts of interest.
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