|Year : 2020 | Volume
| Issue : 4 | Page : 333-334
Renal transplantation in polycythemia vera:- A rare case report from India
Prashant Bharat Malviya, Sanjay Maitra
Department of Nephrology, Apollo Hospital, Hyderabad, Telangana, India
|Date of Submission||06-Feb-2020|
|Date of Acceptance||07-Aug-2020|
|Date of Web Publication||30-Dec-2020|
Dr. Prashant Bharat Malviya
Department of Nephrology, Apollo Hospital, Jubillee Hills, Hyderabad - 500 096, Telangana
Source of Support: None, Conflict of Interest: None
Polycythemia vera (PV) is a rare myeloproliferative neoplasm usually associated with JAK2 mutation. Manifestations include blood circulation disorder, hypertension and cerebral infarction. Renal disease is quite uncommon in PV and only few cases have been reported in the literature. In majority of these cases it was considered that PVinduced glomerular hyperperfusion and hyperfiltration as a cause of CKD. PV with ESRD and undergoing successful renal transplantation is much rarer. We here report one such patient of PV who had Budd Chiari syndrome, underwent hepatic vein stenting. He developed CKD which progressed to ESRD and underwent renal transplantation. To the best of our knowledge it is first case report from India.
Keywords: Budd Chiari syndrome, CKD ESRD, Polycythemia vera, portocaval shunt, renal transplantation
|How to cite this article:|
Malviya PB, Maitra S. Renal transplantation in polycythemia vera:- A rare case report from India. Indian J Transplant 2020;14:333-4
| Introduction|| |
Polycythemia vera (PV) constitutes < 5% of hematological malignancies and renal involvement is uncommon. We describe a case of transplantation in patient with PV.
| Case Report|| |
A 43-year-old male who was previously alright was diagnosed as polycythemia vera 16 years back on the basis of history (recurrent headache, painful finger tips, rash over abdominal wall), and lab evaluation (raised hemoglobin, hematocrit of 55, bone marrow suggestive of myeloproliferative disorder – polycythemia vera and JAK2 mutation positive). He was prescribed hydroxyurea and underwent intermittent venesection to keep red cell mass below 45. In 2015 he developed Budd Chiari syndrome with thrombosis in hepatic vein for which he underwent portocaval shunt placement. At that time urine examination and renal function test was normal.
In 2017 he presented with renal dysfunction with serum creatinine 4.1 mg/dl and subnephrotic proteinuria. Kidney function was not evaluated between 2015 to 2017. He denied any history of any urinary complaint in past or consumption of any indeginious medications. Urine routine has shown 2 + albumin, nil RBC cast. Ultrasound has shown right side 8.2 cm left side 8.4 cm kidney with grade 2 renal parenchymal changes. After counseling he underwent kidney biopsy which showed:-
- Out of 10 glomeruli 5 glomeruli has shown global glomerulosclerosis
- Other has shown mild mesangial proliferation
- Interstitial fibrosis and tubular atrophy (IFTA) was seen in 60-70% of biopsied sample
- IF showed patchy mesangial deposits of IgG and C3c, IgA was negative
Above biopsy finding of significant glomerulosclerosis and IFTA can be attributed to hyperfiltration injury due to underlying polycythemia vera. His renal function gradually worsened from stage 3 to stage 5 over next 1 and half year and he was counseled for renal replacement therapy. His urine ouput was maintained to around 1 lit per day. In view of stable hepatic function and after taking clearance from hepatologist, he underwent live renal transplantation donor being wife in 2019. Received induction with ATG 1.5 mg/kg for 3 days and methylprednisolone 2 gm divided over 4 days, later initiated on triple drug immunosuppression (Tacrolimus + MMF + prednisolone). His kidney function is good till date with serum creatinine maintained 1.1 mg/dl and has good urine output. He is being continued on hydroxyurea for polycythemia vera and hemoglobin is stable in the range of 12-14 gm/dl without any need for venesection. There was no resurgence of Budd chiari syndrome post transplant.
| Discussion|| |
Polycythemia vera constitutes 4.7% of all myeloproliferative disorder from India. PV associated with renal disease is rare and generally considered to be associated with high viscosity induced renal hyperperfusion and hyperfiltration. Thus it causes passive expansion of the capillaries and intimal injury, which results in vascular microthrombi, glomerular capillary occlusion and a reduction in the GFR, thereby leading to tissue ischemia. If the ischemia persists without relief, it is likely to result in chronic renal damage. PV is often associated with hypertension and hyperuricemia, which affect renal microcirculation. In addition, thrombocytosis and the abnormal activation of megakaryocytes might be critical factors for glomerular sclerosis. Cytokines and growth factors may also play important role in progression of kidney disease. Studies done by Au et al. has observed overproduction of plateletderived growth factor (PDGF) and transforming growth factor (TGF)β in patients with PV. The degranulation of the plateletinduced sustained release of PDGF and other cytokines induces a rapid progression to crescentic glomerulonephritis and glomerulosclerosis. The histological results of the cases varied and predominantly included IgAN, focal segmental glomerulosclerosis and membranoproliferative glomerulonephritis.
Budd-Chiari syndrome (BCS) represents a rare hepatic condition characterized by vascular obstruction of the efferent hepatic flow. Polycythemia vera is considered to be the most frequent condition causing the Budd-Chiari syndrome (10-40% of cases). But combined Budd Chiari syndrome and renal failure has not been described in polycythemia vera patient. Polycythemia with Budd-Chiari syndrome is described in association with renal transplantation of ADPKD patients, and it has been described in patient of behcets syndrome where patient had terminal event with renal biopsy showing glomerulonephritis.
From previous case reports average age of renal involvement was around 53 years with male predominance (1.6:1). The clinical manifestations mainly include nephrotic syndrome (66.7%), and subnephrotic proteinuria (28.6%), hematuria can be seen in few cases. According to previous study nearly a quarter of patients of PV with renal involvement progress to ESRD while others persists to have proteinuria with mild renal dysfunction. Our case presented with subnephrotic proteinuria with severe renal failure and progressed to ESRD over 2 years duration.
The histological lesions in PV associated with renal diseases usually showed FSGS (47.6%) as most common lesion followed by IgAN (38.1%) and MPGN pattern (9.5%). Our patients at the time of diagnosis already had significant global sclerosis in 50% of glomeruli and IFTA (70%) which can be result of underlying pathology of polycythemia vera.
Very few case reports are there of PV with ESRD post renal transplant and none from India. Roghayeh Akbari et al. reported a rare case of PV who underwent renal transplantation with good control of PV during 26 months after transplantation without specific treatment.
| Conclusion|| |
We report rare case of polycythemia vera developing Budd Chiari syndrome who underwent portocaval shunt placement. He had progressive renal failure with biopsy showing global glomerulosclerosis (5/10) and severe IFTA. He underwent live renal transplantation have stable graft function on triple immunosuppression till date (1 year post transplant). It is very rare case report from India. It will be interesting to follow progression of this patient after transplantation.
Declaration of patient consent
The patient consent has been taken for participation in the study and for publication of clinical details and images. Patients understand that the names, initials would not be published, and all standard protocols will be followed to conceal their identity.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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