|Year : 2021 | Volume
| Issue : 4 | Page : 354-356
Managing end-stage renal disease and live kidney transplant in a patient with hemophilia A: A case report and review of literature
Chandani Bhagat1, Godara Suraj1, Gupta Naveen2, Jeswani Jitesh1
1 Department of Nephrology, Mahatma Gandhi Medical College, Rajasthan, India
2 Department of Hematology, Mahatma Gandhi Medical College, Rajasthan, India
|Date of Submission||26-May-2020|
|Date of Decision||04-Oct-2020|
|Date of Acceptance||28-Apr-2021|
|Date of Web Publication||30-Dec-2021|
B4/158 Safdarjung Enclave, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Hemophilia A is caused by a genetic mutation affecting the levels of factor VIII in serum. With improvement in management and widespread availability of factor VIII, the life expectancy of hemophiliac patients has increased. The change in the history of hemophiliacs has led to the increased surge of chronic diseases in such patients. End-stage renal disease is rarely seen among hemophiliacs and institution of renal replacement therapy is controversial not only for the lack of literature but also differing opinions on protocols for factor VIII replacement. We describe here a case of a moderate hemophiliac A patient with end-stage renal disease undergoing renal replacement therapy initially hemodialysis and later on undergoing kidney transplantation. The patient successfully underwent live unrelated kidney transplant without any major complications and is currently healthy with good graft function. Patients with hemophilia A can successfully undergo renal replacement therapy under factor VIII cover.
Keywords: Hemophilia A, kidney transplant, renal replacement therapy
|How to cite this article:|
Bhagat C, Suraj G, Naveen G, Jitesh J. Managing end-stage renal disease and live kidney transplant in a patient with hemophilia A: A case report and review of literature. Indian J Transplant 2021;15:354-6
|How to cite this URL:|
Bhagat C, Suraj G, Naveen G, Jitesh J. Managing end-stage renal disease and live kidney transplant in a patient with hemophilia A: A case report and review of literature. Indian J Transplant [serial online] 2021 [cited 2022 Jan 26];15:354-6. Available from: https://www.ijtonline.in/text.asp?2021/15/4/354/334432
| Introduction|| |
Hemophilia is a hereditary X-linked recessive hemorrhagic disease and occurs in about 1 out of 10,000 and affects an estimated 400,000 people globally. The severity level of hemophilia A is categorized as severe if the activity was <1% of normal, whereas 1%–5% and >5% of normal are defined as moderate and mild disease, respectively. Dialysis and surgical interventions are more worrisome in hemophiliacs because of underlying changes in clotting process associated with renal failure. However, with the development of tertiary care medical facilities, hemodialysis is being performed safely under factor VIII cover. As of date, there have been three case reports, none reported from India on kidney transplantation in hemophiliacs.
| Case Report|| |
Our index patient is a 49-year-old male, residing in a rural district of Western India. He was diagnosed to have hemophilia A at the age of 36 years when he presented to a tertiary care hospital with complaints of abdominal pain and underwent open laparotomy for intestinal perforation. He had a history of joint swelling and pain since childhood, which were managed with oral analgesics, but was not worked up for clotting factor deficiencies. He suffered renal hematoma following minor trauma at the age of 40 years, for which he underwent right-sided open nephrectomy under factor VIII cover and was normal till 1 year when he was found to have serum creatinine in the range of 4–5 mg/dl. The patient and his family were explained about the need for renal replacement therapy and the options of lifelong dialysis versus renal transplantation. The family opted for live renal transplantation. The patient was being maintained on heparin-free hemodialysis twice a week without factor VIII cover till the time he was planned for renal transplant.
Operative and perioperative details
The patient underwent live unrelated renal transplant 6 months back. The baseline factor VIII levels were 2.2% and there were no inhibitors in serum to factor VIII. Preoperative dialysis was given to the patient. The recipient surgery lasted 200 min and the warm ischemia time was 35 min. On postoperative day 2, urine output was 4 l, serum creatinine was 0.8 mg/dl, hemoglobin was 8 g/dl, INR was 1.2, and aPTT was 35 sec. Foley catheter was removed on postoperative day 4, abdominal drain on day 6, and DJ stent on day 12.
Protocol for factor VIII administration
The renal transplant surgery was done under cover of factor VIII starting from preoperative day 1 to postoperative day 12. Factor VIII and Factor VIII inhibitor levels were measured intermittently and dose (based on body weight of 58 kg) of factor VIII was changed subsequently. The factor VIII levels were measured either 10 min after infusion of factor VIII or pre infusion trough levels. The complete dosing schedule (in accordance with world federation of hemophilia guidelines) is explained in [Table 1].
At 6 months of follow-up, the patient is doing well with adequate urine output and serum creatinine of 0.6 mg/dl. No surgical wound complications or graft dysfunction have been noted.
| Discussion|| |
Renal replacement therapy is almost unknown in literature for hemophiliacs with chronic renal failure. However with increased survival of haemophiliacs inflicted with HIV/HCV virus, there has been increased prevalence of chronic liver and kidney dysfunction in them.,
In our patient, different conditions namely previous nephrectomy, use of nephrotoxic drugs (analgesics), and factor VIII therapy coexisted, making him susceptible to the development of chronic kidney disease.
Because of the reduced risk of bleeding and not requirement of coagulation factor infusions after any treatment, peritoneal dialysis was initially the preferred dialytic modality for hemophiliac patients; however, considering the previous history of major abdominal surgery in our patient (which is one of the most important contraindications to PD), we opted for heparin-free hemodialysis.
Since kidney transplant like other abdominal surgeries predisposes the patient to potential perioperative hemorrhage, strict measures were taken to maintain hemostasis. The estimated blood loss was approximately 300 ml, which is well within the normal expected range. Abdominal drain was kept 1 day extra to detect any late postoperative hemorrhage.
There are few reports,, about kidney transplantation in hemophiliac patients. Renal transplant surgery in hemophiliac patients is a medical dilemma to nephrologists in two aspects. First, the protocols for factor VIII therapy in liver transplant surgery cannot be applied to kidney transplant as factor VIII is metabolized by liver. Second, the clotting abnormality associated with nephropathy itself leads to requirement of higher dosage of factor VIII as compared to other routine surgical intervention in hemophiliac patients. The required level of factor VIII in our patient was adjusted to that of a normal patient, i.e. 100% during the initial perioperative period, and supplemental doses of factor VIII were added for minor procedures like drain and stent removal. Fortunately, we did not observe any perioperative hemorrhage and ultrasound Doppler at 2 weeks showed no perirenal hematoma with normal resistive index in renal artery.
In conclusion, management of renal diseases and RRT in hemophilia is complex and requires a full expertise, including hemophilia centers, surgeons, and nephrologists.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Soucie JM, Evatt B, Jackson D. Occurrence of hemophilia in the United States. The Hemophilia Surveillance System Project Investigators. Am J Hematol 1998;59:288-94.
Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Ilinas A, Ludlam C A, et al.
Treatment guidelines working group on behalf of the world federation of hemophilia. Haemophilia 2013;19:e1-e47.
Small S, Rose PE, McMillan N, Belch JJ, Rolfe EB, Forbes CD, et al.
Haemophilia and the kidney: Assessment after 11-year follow-up. Br Med J (Clin Res Ed) 1982;285:1609-11.
Konkle BA, Kessler C, Aledort L, Andersen J, Fogarty P, Kouides P, et al.
Emerging clinical concerns in the ageing haemophilia patient. Haemophilia 2009;15:1197-209.
Konkle BA. The aging patient with hemophilia. Am J Hematol 2012;87 Suppl 1:S27-32.
Roy-Chaudhury P, Manfro RC. Renal replacement therapy for hemophiliacs. Int J Artif Organs 1997;20:241-3.
ElBakkouri J, Mamdouh A, Faez S, Medkouri G, Ramdani B, Benchemsi N. Kidney transplantation in a patient with haemophilia A. Transfus Clin Biol 2009;16:471-3.
Gomperts ED, Malekzadeh MH, Fine RN. Dialysis and renal transplant in a hemophiliac. Thromb Haemost 1981;46:626-8.
Koene RA, Gerlag PG, Jansen JL, Moulijn AC, Skotnicki SH, Debruyne FJ, et al
. Successful hemodialysis and renal transplantation in a patient with hemophilia A. Proc Eur Dial Transplant Assoc 1977;14:401-6.