Limits...
Autoimmune haemolytic anaemia associated with Epstein Barr virus infection as a severe late complication after kidney transplantation and successful treatment with rituximab: case report.

Hamilton AJ, Webb LH, Williams JK, D'Souza RJ, Ngu LS, Moore J - BMC Nephrol (2015)

Bottom Line: We describe a 44 year old woman who presented 5 years after kidney transplantation with profound transfusion dependent warm AIHA.Investigations confirmed an IgG autoantibody against RBCs and high titre Epstein-Barr virus (EBV) viraemia.Twenty-six units of blood were required during the course of treatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology, Royal Devon and Exeter NHS Foundation Trust, Exeter, UK. alexander.hamilton@nhs.net.

ABSTRACT

Background: Autoimmune haemolytic anaemia (AIHA) is a rare complication following kidney transplantation and usually occurs early in its course. It is characterised by autoantibodies or alloantibodies directed against red blood cells (RBCs).

Case presentation: We describe a 44 year old woman who presented 5 years after kidney transplantation with profound transfusion dependent warm AIHA. Investigations confirmed an IgG autoantibody against RBCs and high titre Epstein-Barr virus (EBV) viraemia. The patient was at higher risk for EBV disease being seronegative at the time of transplantation but had detectable EBV capsid IgG antibody at the time of presentation. The haemolysis was refractory to high dose steroid and intravenous immunoglobulin. There was a rapid and complete resolution of both the anaemia and the viraemia following rituximab therapy, with no adverse events. Twenty-six units of blood were required during the course of treatment.

Conclusions: To our knowledge this is the first reported case of EBV associated AIHA in a renal transplant recipient. It highlights a rare pathology associated with post-transplant EBV infection, of broad interest to transplant physicians, haematologists, and microbiologists, and the effective novel use of monoclonal anti-CD20 therapy.

No MeSH data available.


Related in: MedlinePlus

Serial haemoglobin, LDH and EBV viral load and response to Rituximab therapy
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
getmorefigures.php?uid=PMC4506635&req=5

Fig1: Serial haemoglobin, LDH and EBV viral load and response to Rituximab therapy

Mentions: On examination she was pale, jaundiced and tachycardic, with dark urine. There was no lymphadenopathy. All investigations are shown in Additional file 1: Table S1. They demonstrated a severe anaemia (haemoglobin 57 g/L) with parameters consistent with haemolysis. DAT was positive confirming a warm agglutinin AIHA. She required blood transfusion support and was initially treated with high dose prednisolone (1 mg/kg) with little effect. A 5 day course of intravenous immunoglobulin (0.4 g/kg/day) was started on day 9. At this point a high serum titre of EBV (538355 copies/ml) was detected with serology suggesting this was reactivation from previous exposure to donor virus and not a primary infection (see Additional file 1: Table S1). She went on to have a total body CT scan which detected no abnormal lymphadenopathy and a bone marrow aspirate and trephine was consistent with haemolysis and no evidence of a lymphoproliferative disorder. Tacrolimus was converted to sirolimus on day 28. After an initial fall in serum EBV load there was a deterioration in clinical state with increasing transfusion requirements followed by an increase in EBV load. The patient was given rituximab 375 mg/m2 on day 30, followed by a further three weekly doses. At the beginning of the treatment course she had received 16 units of blood and the EBV viral load was 268649 copies/ml. After the fourth dose of rituximab there was a significant decline in transfusion requirement and the EBV viral load had fallen to undetectable levels. Seven transfusions were required during the rituximab course and three following the course. Her serial haemoglobin, LDH, EBV viral load, rituximab doses and transfusions are shown in Fig. 1. She had achieved complete remission 2 weeks after starting the rituximab. There was no deleterious effect on graft function and no adverse drug events were noted. In total she required 26 units of blood with significant iron overload, the serum ferritin rose to 2850 μg/L which had fallen to 950 μg/L 12 months after remission. Despite the acute illness graft function was unchanged and she remains well on prednisolone and sirolimus immunosuppression with no recurrence of EBV viraemia or anaemia.Fig. 1


Autoimmune haemolytic anaemia associated with Epstein Barr virus infection as a severe late complication after kidney transplantation and successful treatment with rituximab: case report.

Hamilton AJ, Webb LH, Williams JK, D'Souza RJ, Ngu LS, Moore J - BMC Nephrol (2015)

Serial haemoglobin, LDH and EBV viral load and response to Rituximab therapy
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4506635&req=5

Fig1: Serial haemoglobin, LDH and EBV viral load and response to Rituximab therapy
Mentions: On examination she was pale, jaundiced and tachycardic, with dark urine. There was no lymphadenopathy. All investigations are shown in Additional file 1: Table S1. They demonstrated a severe anaemia (haemoglobin 57 g/L) with parameters consistent with haemolysis. DAT was positive confirming a warm agglutinin AIHA. She required blood transfusion support and was initially treated with high dose prednisolone (1 mg/kg) with little effect. A 5 day course of intravenous immunoglobulin (0.4 g/kg/day) was started on day 9. At this point a high serum titre of EBV (538355 copies/ml) was detected with serology suggesting this was reactivation from previous exposure to donor virus and not a primary infection (see Additional file 1: Table S1). She went on to have a total body CT scan which detected no abnormal lymphadenopathy and a bone marrow aspirate and trephine was consistent with haemolysis and no evidence of a lymphoproliferative disorder. Tacrolimus was converted to sirolimus on day 28. After an initial fall in serum EBV load there was a deterioration in clinical state with increasing transfusion requirements followed by an increase in EBV load. The patient was given rituximab 375 mg/m2 on day 30, followed by a further three weekly doses. At the beginning of the treatment course she had received 16 units of blood and the EBV viral load was 268649 copies/ml. After the fourth dose of rituximab there was a significant decline in transfusion requirement and the EBV viral load had fallen to undetectable levels. Seven transfusions were required during the rituximab course and three following the course. Her serial haemoglobin, LDH, EBV viral load, rituximab doses and transfusions are shown in Fig. 1. She had achieved complete remission 2 weeks after starting the rituximab. There was no deleterious effect on graft function and no adverse drug events were noted. In total she required 26 units of blood with significant iron overload, the serum ferritin rose to 2850 μg/L which had fallen to 950 μg/L 12 months after remission. Despite the acute illness graft function was unchanged and she remains well on prednisolone and sirolimus immunosuppression with no recurrence of EBV viraemia or anaemia.Fig. 1

Bottom Line: We describe a 44 year old woman who presented 5 years after kidney transplantation with profound transfusion dependent warm AIHA.Investigations confirmed an IgG autoantibody against RBCs and high titre Epstein-Barr virus (EBV) viraemia.Twenty-six units of blood were required during the course of treatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology, Royal Devon and Exeter NHS Foundation Trust, Exeter, UK. alexander.hamilton@nhs.net.

ABSTRACT

Background: Autoimmune haemolytic anaemia (AIHA) is a rare complication following kidney transplantation and usually occurs early in its course. It is characterised by autoantibodies or alloantibodies directed against red blood cells (RBCs).

Case presentation: We describe a 44 year old woman who presented 5 years after kidney transplantation with profound transfusion dependent warm AIHA. Investigations confirmed an IgG autoantibody against RBCs and high titre Epstein-Barr virus (EBV) viraemia. The patient was at higher risk for EBV disease being seronegative at the time of transplantation but had detectable EBV capsid IgG antibody at the time of presentation. The haemolysis was refractory to high dose steroid and intravenous immunoglobulin. There was a rapid and complete resolution of both the anaemia and the viraemia following rituximab therapy, with no adverse events. Twenty-six units of blood were required during the course of treatment.

Conclusions: To our knowledge this is the first reported case of EBV associated AIHA in a renal transplant recipient. It highlights a rare pathology associated with post-transplant EBV infection, of broad interest to transplant physicians, haematologists, and microbiologists, and the effective novel use of monoclonal anti-CD20 therapy.

No MeSH data available.


Related in: MedlinePlus