|Year : 2017 | Volume
| Issue : 2 | Page : 82-83
Renal transplant in a lupus nephritis patient with β-thalassemia trait
Uma Shankar Gaur, Dhananjai Agarwal, Pankaj Beniwal, Vinay Malhotra
Department of Nephrology, SMS Medical College, Jaipur, Rajasthan, India
|Date of Web Publication||12-Sep-2017|
Uma Shankar Gaur
House No. 136, Sona Vihar, Delhi Road, Alwar - 301 001, Rajasthan
Source of Support: None, Conflict of Interest: None
Progression of proliferative lupus nephritis to end-stage renal disease is common. Anemia in chronic kidney disease has multifactorial etiology, but it is rarely associated with β- Thalassemia trait. Iron deficiency is common in hemodialysis patients due to increased blood loss. Microcytic hypochromic anemia may be due to iron deficiency, hemoglobinopathies and aluminum toxicity. Because chronic kidney disease is a chronic inflammatory state, it is difficult to exclude iron deficiency with classical biochemical markers. Treatment of anemia in chronic kidney disease is important as iron therapy may cause iron overload, increased susceptibility to infection, atherosclerosis and increased oxidative stress. Multiple blood transfusions may cause iron overload, risk of infection transmission and alloimmunization. Alloimmunization decreases donor pool and increases chances of rejection.
Keywords: Anaemia, end-stage renal disease, lupus nephritis
|How to cite this article:|
Gaur US, Agarwal D, Beniwal P, Malhotra V. Renal transplant in a lupus nephritis patient with β-thalassemia trait. Indian J Transplant 2017;11:82-3
|How to cite this URL:|
Gaur US, Agarwal D, Beniwal P, Malhotra V. Renal transplant in a lupus nephritis patient with β-thalassemia trait. Indian J Transplant [serial online] 2017 [cited 2019 May 21];11:82-3. Available from: http://www.ijtonline.in/text.asp?2017/11/2/82/214380
| Introduction|| |
Lupus nephritis is common in systemic lupus erythematosus (SLE) patients., Approximately 10%–30% of patients with proliferative lupus nephritis progress to end-stage renal disease (ESRD)., Anemia of varying degree is a common finding in ESRD. In lupus ESRD, iron deficiency is common but there are other causes also. Lupus nephritis is rarely associated with β-thalassemia minor. We report a patient with lupus nephritis presenting as ESRD and diagnosed as microcytic hypochromic anemia due to β-thalassemia minor.
| Case Report|| |
A 33-year-old female, a case of SLE with systemic symptoms of alopecia, fatigue, and arthralgia was diagnosed as lupus nephritis with proteinuria of 2.4 g/day and active urine sediment in September 2012. On initial evaluation, hemoglobin and serum creatinine were 7.9 gm/dl and 2.0 mg/dl, respectively. ANA and ds DNA were positive, whereas C3 and C4 were low. On renal biopsy, she had Class IV A/C with 60% tubulointerstitial fibrosis. She was started on mycophenolate mofetil (MMF) and prednisone. Hydroxychloroquine and atorvastatin were given as adjunctive therapy. She had microcytic hypochromic anemia. She was prescribed hematinics including parenteral iron therapy and erythropoietin. After that, she did not have any anemia evaluation. After 6 months of treatment, she had persistently rising creatinine (5.2 mg/dl). Repeat renal biopsy showed chronic glomeruloscelerosis (92%) and tubulointerstitial fibrosis. Immunosuppressant tapered over a period of month. Immunosuppressant tapered over a period of a month. At 9 months, she was taking prednisone 10 mg/day only. In October 2016, she was started on maintenance hemodialysis. She did not have any history of vascular thrombotic episode. She was referred to our center for live renal transplantation. She had clinically (signs and symptoms) and serologically silent lupus, so immunosuppressants were stopped. She has microcytic hypochromic anaemia (mean cell volume = 70 fl, Hb = 7.2 g/dl and red cell distribution width = 14.3%) with low reticulocyte production index (2.0%). She didn't received any blood transfusion. On physical examination, no organomegaly or lymphadenopathy was present. Stool for occult blood was negative. Upper gastrointestinal endoscopy and colonoscopy were normal. Family history of any hematological disorder was negative. Transferrin saturation and serum ferritin were 24% and 678 ng/ml, respectively. Serum folic acid, Vitamin B12, C-reactive protein, and serum lactate dehydrogenase were within normal limits. Coombs test was negative. She was given parenteral iron 200 mg with continuation of erythropoietin. After 4 weeks, hemoglobin increased to 7.5 g/dl. Possibility of aluminum toxicity was rare, because she didn't have a history of aluminum-containing phosphate-binding agents. She has microcytic hypochromic anemia refractory to iron therapy. Hemoglobin electrophoresis was planned to rule out hemoglobinopathies. The patient was diagnosed to have β-thalassemia trait as HbA2 (6%) was elevated. HbF level (1.5%) was normal. Later on, the mother was also found positive for β-thalassemia trait. On pretransplant evaluation, antiphospholipid antibodies were absent. She received a renal allograft from her mother in January 2017. She is taking tacrolimus, MMF and prednisone. During postoperative period, she has calcineurin inhibitor (CNI) toxicity, which settles down on CNI dose reduction. She was discharged at day 10 with nadir serum creatinine of 1.0 mg/dl. Irrespective of all efforts, her hemoglobin is 8.9 g/dl at 3 months of post-transplant.
| Discussion|| |
β-thalassemia trait is rare in patients with SLE, but when they coexist, it can manifest as severe anemia. In a study  from Italy, patients with SLE have a lower prevalence of β-thalassemia than a control group (9.8% vs. 13.1%).
ESRD may have anemia due to various factors such as autoimmune anemia, hemolytic anemia, anemia of chronic disease, and nutritional anemia. Hemolytic anemia may be due to dialysate (containing copper, aluminum, chloramines, overheating, underheating, and hyperosmolality), drugs (e.g., quinine), thrombotic microangiopathy, kinked dialysis blood tubing, single-needle dialysis, small-caliber dialysis needle, reuse of dialyzer (formaldehyde), and insufficient dialysis. Anemia of chronic diseases may occur in chronic inflammatory state.
We did not find any evidence of them. She had iron refractory microcytic anemia; so hemoglobinopathies were suspected.
Chronic kidney disease (CKD) may have low reticulocyte index due to bone marrow fibrosis. She also had no clinical and biochemical evidences of chronic hemolysis. Hence, we did not perform bone marrow biopsy.
Anemia correction causes improvements in quality of life, cognitive function, sleep patterns, nutrition, sexual function, menstrual regularity, immune responsiveness, and platelet function. Iron therapy may cause iron overload, increased susceptibility to infection, atherosclerosis, and increased oxidative stress. Repeated blood transfusion may cause iron overload, risk of infection transmission, and alloimmunization. Alloimmunization decreases donor pool and increases chances of rejection. Because CKD is a chronic inflammatory, immunodeficient, and oxidative state; these therapies have more complications than in general population.
Iron therapy should be given after confirmation of iron deficiency, looking at risk–benefit ratio for therapy. β-thalassemia trait often doesn't have any specific treatment, except genetic counseling.
| Conclusion|| |
Although possibility of β-thalassemia in association with lupus nephritis is low, it should be suspected in difficult to treat microcytic hypochromic anemia. Due to increased risk of complications in CKD, empiric treatment with iron and blood transfusion should be avoided.
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Conflicts of interest
There are no conflicts of interest.
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