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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 13  |  Issue : 3  |  Page : 228-230

Renal transplant in a patient with idiopathic thrombocytopenic purpura refractory to steroid and intravenous immunoglobulin


1 Department of Medicine, Mahatma Gandhi Medical College, Jaipur, Rajasthan, India
2 Department of Nephrology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India

Date of Submission04-May-2019
Date of Decision29-Jul-2019
Date of Acceptance12-Aug-2019
Date of Web Publication17-Sep-2019

Correspondence Address:
Dr. Vinay Rathore
Department of Nephrology, Sawai Man Singh Medical College, Jaipur - 302 004, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijot.ijot_11_19

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  Abstract 


Chronic kidney disease in immune thrombocytopenia (ITP) is uncommon, and renal transplant in this setting is especially rare. We present a case of successful renal transplantation in a patient with chronic ITP refractory to steroid and intravenous immunoglobulin (IVIg). A 27-year-old female suffering from ITP was referred to our center for renal transplant with her mother as a donor. Her platelets count failed to improve despite treatment with prednisolone (1 mg/kg/day for 4 weeks) and IVIg (1 g/kg/day for 2 consecutive days). She was then treated with eltrombopag (50 mg/day), a thrombopoietin receptor agonist, following which her platelet counts improved and allowed kidney transplantation to be performed safely. At 1 year of follow-up, her graft was functioning normally. The case report highlights that renal transplantation is feasible in patients with ITP, even if the disease is refractory to first-line agents. Eltrombopag may be used to increase the platelet count and decrease the risk of bleeding during the peritransplant period.

Keywords: Eltrombopag, immune thrombocytopenia, renal transplant, thrombopoietin receptor agonist


How to cite this article:
Agarwal S, Beniwal P, Agarwal D, Rathore V. Renal transplant in a patient with idiopathic thrombocytopenic purpura refractory to steroid and intravenous immunoglobulin. Indian J Transplant 2019;13:228-30

How to cite this URL:
Agarwal S, Beniwal P, Agarwal D, Rathore V. Renal transplant in a patient with idiopathic thrombocytopenic purpura refractory to steroid and intravenous immunoglobulin. Indian J Transplant [serial online] 2019 [cited 2019 Dec 11];13:228-30. Available from: http://www.ijtonline.in/text.asp?2019/13/3/228/266940




  Introduction Top


Immune thrombocytopenia (ITP) is an acquired autoimmune disorder characterized by low platelet count (<100,000/ml) in the absence of any obvious cause of thrombocytopenia. The occurrence of chronic kidney disease (CKD) in the setting of ITP is uncommon, and renal transplantation in this setting is especially rare with only a handful of reported cases in the literature.[1],[2],[3],[4]

Kidney transplantation in a patient with ITP is especially challenging as poor platelet function secondary to uremia adds to the risk of bleeding, particularly in those patients who fail to respond to first-line agents used for the treatment of ITP. Eltrombopag, a recently introduced thrombopoietin receptor agonist, has been found to be effective for the treatment of ITP refractory to first-line agents.[5],[6]

We report successful kidney transplantation in a patient with ITP refractory to first-line agents treated with eltrombopag.


  Case Report Top


A 27-year-old female was referred to our center for the prospect of renal transplant. She was diagnosed with chronic glomerulonephritis at the age of 22 years and was on conservative management for the same. Her hypertension was controlled on the nifedipine and metoprolol. She also had hypothyroidism which was well controlled on thyroxine (50 mcg/day).

She was diagnosed to have ITP at the age of 18 years during routine evaluation. Her platelet count varied between 30,000 and 50,000/ml. She did not give a history of significant bleeding episode.

Her physical examination was unremarkable, except for pallor and hypertension. The abdominal examination did not reveal splenomegaly. Her hemoglobin was 7.7 g/dl, total leukocyte count was 5790/mm3 with differential counts of neutrophils 70%, lymphocytes 23%, and monocytes 5%, and platelet count was 49,000/mm3. Peripheral blood film was unremarkable except for decreased platelets. Serum creatinine was 6.8 mg/dl, serum protein was 6.7 mg/dl, and serum albumin was 3.9 mg/dl. Urine routine examination revealed 3 + proteinuria, and microscopy revealed 3–5 red blood cells/high-power field. Twenty-hour urinary protein excretion was 450 mg/day. Abdomen ultrasound revealed that the right kidney was 6.1 cm × 2.2 cm and the left kidney was 5.1 cm × 2.3 cm with lost corticomedullary differentiation, and the spleen was normal in size. Serologies for hepatitis B, hepatitis C, HIV, antinuclear antibody, anti-dsDNA, antineutrophil cytoplasmic antibody, and antiphospholipid antibodies were all negative. C3 and C4 complement levels were normal. Vitamin B12 level was 604 pg/ml (normal range: 211–911 pg/ml), and serum folate level was 3.5 ng/ml (normal range: 3–17 ng/ml). Bone marrow examination was consistent with the diagnosis of ITP.

She was started on thrice-weekly hemodialysis and was counseled regarding kidney transplantation. Her mother, aged 52 years, came forward as a prospective donor. She was ABO compatible with the patient.

Her platelet counts did not improve despite treatment with prednisolone (1 mg/kg/day for 4 weeks) and intravenous immunoglobulin (IVIg) (1 g/kg/day for 2 consecutive days). She was then treated with eltrombopag (50 mg/day). Her platelet count started improving after 8 days of therapy and reached 125,000/mm3 after 10 days. At the time of transplantation, her platelet count was 130,000/mm3.

She underwent renal transplant with tacrolimus, mycophenolate mofetil, and prednisolone as an initial immunosuppressive agent. Peri/posttransplant period was uneventful. Platelet transfusion was not required. She had good graft function. At discharge, serum creatinine was 1.4 mg/dl, and platelet count was 97,000/mm3.

At 1 year of follow-up, her platelet count was 47,000/mm3 but was asymptomatic. Her serum creatinine was 1.3 mg/dl.


  Discussion Top


The present case highlights successful renal transplantation in a patient with chronic refractory ITP treated with eltrombopag. The occurrence of ITP with CKD is an uncommon event. Only a handful of cases have been reported in the literature.[1],[2],[3],[4] Index case was diagnosed as ITP 4 years prior to the diagnosis of CKD.

Symptomatology of ITP is highly variable ranging from asymptomatic, mild bruising, mucosal bleeding to life-threatening to bleed including intracranial bleed.[5] Index case did not have any significant bleeding episode in the past despite having platelet count between 30,000 and 50,000/ml. Treatment for ITP may not be indicated in such asymptomatic patients.[5] However, development of a CKD and associated hemostatic defect in uremia increases the risk of bleeding complications and therefore warrants treatment in the index case.[5] In general, steroids and IVIg are considered to be the first-line treatment for ITP.[5] However, up to 20% patient may not respond to this first-line agents.[5] Treatment option for such patients includes immunosuppressive drugs (azathioprine, cyclophosphamide, mycophenolate mofetil, cyclosporine, and rituximab) and splenectomy.[5] While immunosuppressive agent has variable efficacy and toxic effect on the immune system, splenectomy is associated with surgical morbidity and mortality and a lifelong risk of serious infections.

Recently, eltrombopag, an oral thrombopoietin receptor agonist, has been found to increase platelet counts and reduces bleeding episodes in a patient with ITP and is being increasingly used as the second-line therapy because of their safety and efficacy.[7] Eltrombopag binds to the transmembrane and juxtamembrane domain of MPL (thrombopoietin receptor) resulting in the activation of downstream Janus kinase–signal transducer and activator of transcription and mitogen-activated protein kinase pathways stimulating megakaryocytopoiesis.[6] It has excellent oral bioavailability and has a dose-dependent response in patients with ITP.[6] Eltrombopag is usually initiated at a dose of 50 mg/day for most adults. Dose reductions are needed for patients with hepatic derangement and patients of East Asian ancestry.[6] It is generally well tolerated with common side effects being headache, nasopharyngitis, upper respiratory tract infection, fatigue, diarrhea, and elevated liver enzymes.[6]

In the index case, platelet count improved after starting eltrombopag and allowed renal transplant surgery to proceed uneventfully. To our knowledge, this is the first case report of renal transplantation in a patient with ITP treated with eltrombopag. Hwang et al. have reported renal transplantation in a patient with ITP treated with IVIg and simultaneous splenectomy.[2] However; platelet count did not improve in the peritransplant period with IVIg, and the patient had intra-abdominal bleed and required repeat surgery. On the other hand, Kanodia et al. and Takahara et al. have reported a successful transplant in ITP patients treated with IVIg.[3],[4]

The index case had an uneventful course at 1 year of follow-up with no episode of graft dysfunction or infection. Although her platelet count has decreased, she is asymptomatic.

To conclude, renal transplantation in patients with ITP is feasible, even if the disease is refractory to first-line agents. Eltrombopag may be used to increase the platelet count and decrease the risk of bleeding during the peritransplant period.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Einollahi B. Renal transplantation and idiopathic thrombocytopenic purpura: Two case reports. Transplant Proc 2009;41:2923.  Back to cited text no. 1
    
2.
Hwang EM, Woo HY, Choi BS, Yang CW, Kim YS, Moon IS, et al. Renal transplantation in a patient with idiopathic thrombocytopenic purpura. Korean J Intern Med 2005;20:92-5.  Back to cited text no. 2
    
3.
Kanodia KV, Vanikar AV, Shah PR, Trivedi HL. Renal transplantation in idiopathic thrombocytopenic purpura. Saudi J Kidney Dis Transpl 2013;24:793-4.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Takahara S, Ichikawa Y, Ishibashi M, Takaha M, Sonoda T. Renal transplantation and idiopathic thrombocytopenic purpura. Clin Transpl 1986. p.133.  Back to cited text no. 4
    
5.
Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010;115:168-86.  Back to cited text no. 5
    
6.
Gill H, Wong RS, Kwong YL. From chronic immune thrombocytopenia to severe aplastic anemia: Recent insights into the evolution of eltrombopag. Ther Adv Hematol 2017;8:159-74.  Back to cited text no. 6
    
7.
Nomura S. Advances in diagnosis and treatments for immune thrombocytopenia. Clin Med Insights Blood Disord 2016;9:15-22.  Back to cited text no. 7
    




 

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