Home About us Current issue Back issues Submission Instructions Advertise Contact Login   

Search Article 
  
Advanced search 
 
Saudi Journal of Kidney Diseases and Transplantation
Users online: 3321 Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size 
 

Table of Contents   
CASE REPORT  
Year : 2016  |  Volume : 27  |  Issue : 1  |  Page : 147-149
Evans syndrome and its link with systemic lupus erythematosus


1 Department of Nephrology, Armed Forces Medical College and Command Hospital, Southern Command, Pune, India
2 Department of Hematology, Armed Forces Medical College and Command Hospital, Southern Command, Pune, India
3 Department of Pathology and Laboratory Sciences, Armed Forces Medical College and Command Hospital, Southern Command, Pune, India
4 Department of Radiology, Armed Forces Medical College and Command Hospital, Southern Command, Pune, India

Click here for correspondence address and email

Date of Web Publication15-Jan-2016
 

   Abstract 

Evans syndrome is a rare syndrome associated with the presence of autoimmune hemolytic anemia and simultaneous or sequential development of thrombocytopenia. It was first described by Evan and Duane in 1951. It is one of the rare presenting features of autoimmune disorders, especially systemic lupus erythematosus (SLE), and sometimes may even precede the onset of disease. Primary Evans syndrome with no cause is very rare and is seen in children. Here, we describe a case of secondary Evans syndrome with severe hemolytic anemia leading to acute kidney injury and recovery thereafter only to develop lupus nephritis a few months later. This is one of the rare presentations of SLE and there are only anecdotal case reports.

How to cite this article:
Mendonca S, Srivastava S, Kapoor R, Gupta D, Gupta P, Sharma M L. Evans syndrome and its link with systemic lupus erythematosus. Saudi J Kidney Dis Transpl 2016;27:147-9

How to cite this URL:
Mendonca S, Srivastava S, Kapoor R, Gupta D, Gupta P, Sharma M L. Evans syndrome and its link with systemic lupus erythematosus. Saudi J Kidney Dis Transpl [serial online] 2016 [cited 2019 Nov 14];27:147-9. Available from: http://www.sjkdt.org/text.asp?2016/27/1/147/174177

   Introduction Top


Evans syndrome is the presence of autoimmune hemolytic anemia (AIHA) associated with simultaneous or sequential development of thrombocytopenia. It was first described by Evan and Duane in 1949 [1] and is seen in 0.8- 3.7% of patients with AIHA or Idiopathic thrombocytopenic purpura (ITP). [2] It is one of the rare presentations of systemic lupus erythematosus (SLE), and often precedes it. We present a case of SLE who first presented with severe anemia and thrombocytopenia associated with acute kidney injury (AKI), and further evaluation revealed the patient as having SLE. The incidence of AKI in Evans syndrome is presently unknown.


   Case Report Top


A 34-year-old lady presented with complaints of breathlessness on exertion and skin rash of two weeks' duration and decreased urine output of two days. Further history revealed that she had an abortion five months ago. There was also a history of weight loss, hair loss, oral ulcers and polyarthralgia. Clinically, she had severe pallor and petechiae on the upper and lower limbs, icterus, pedal edema and mild splenomegaly. She also had a sparse hairline and malar rash with painless oral ulcers. A clinical diagnosis of SLE was made as she met all the American Rheumatology Association criteria for SLE.

Investigations revealed a hemoglobin of 4.1 g/dL and total leukocyte count of 3500/mm 3 , with a normal differential count. Her platelet count was 10,000/mm 3 . Peripheral blood smear revealed fragmented red blood cells with evidence of hemolysis and thrombocytopenia. Her lactate dehydrogenase was 2800 IU/mL (normal range 200-400 IU/mL) and reticulocyte count was 4% with reduced serum haptoglobulin. Prothrombin time/activated partial thromoplastin time were normal. Serum bilirubin was 3.5 mg/dL and indirect bilirubin 3 mg/dL with normal transaminases. She had acute kidney injury with a blood urea of 89 mg/dL and serum creatinine of 3.5 mg/dL.

Urine revealed numerous red blood cells and hemoglobinuria. Direct Coombs test (DAT) was positive. The anti-nuclear antibody (ANA) was positive at 1:40 dilution by indirect immunoflourescence and dsDNA titers were 114.68 IU/mL (normal range 35-55 IU/mL). Anti-phospholipid antibody was positive with positive lupus anticoagulant. Bone marrow aspiration revealed cellular reactive bone marrow with megakaryocytic thrombocytopenia.

A clinical diagnosis of secondary Evans syndrome was made in view of hemolytic anemia, thrombocytopenia and DAT positivity with features of SLE associated with AKI due to hemolysis.

She was given a pulse of methyl prednisolone 15 mg/kg and blood component support in the form of packed red blood cells and platelet transfusion. This was followed by oral prednisolone 1 mg/kg. Gradually, she began to improve and her hemolysis resolved with the urine output gradually improving in three days and renal functions returning to normal in one week. Over a period of one month, the features of SLE improved and there was no hemolysis, but she continued to have thrombocytopenia with a platelet count between 10,000 and 20,000/mm 3 . A decision to give rituximab was taken as cyclophosphamide and mycophenolate mofetil (MMF) was avoided to prevent bone marrow toxicity and hence rituximab was the only option. She was given 1 g/m 2 of rituximab with a plan of weekly doses for four weeks. The platelet count improved over one month and was stabilized between50,000 and 60,000/mm 3 . She gradually improved over the next few months. However, after five months, she developed sub-nephrotic proteinuria of 2.4 g/24 h, associated with dysmorphic red blood cells in the urine. A kidney biopsy was performed that revealed features of class IV lupus nephritis [Figure 1].
Figure 1: Periodic Acid Schiff stain (magnification 400×) of the kidney biopsy showing diffuse proliferative nephritis characterized by irregular mesangial proliferation and segments with endocapillary proliferation.

Click here to view


Because she was only on maintenance doses of oral prednisolone, her immunosuppression was increased with prednisolone 1 mg/kg and MMF 750 mg twice daily was added as her hematological parameters were now stable, with a lower chance of bone marrow suppression. After a period of one month, she went into complete remission and is presently doing fine with maintenance doses of prednisolone and MMF.


   Discussion Top


Evans syndrome reflects a state of profound immune dysregulation as opposed to coincidental immune cytopenia, as seen in autoimmune diseases. Its presentation as SLE is very rare and sometimes precedes the detection of SLE. Evans syndrome associated with AKI is unheard of and is usually due to massive hemolysis, causing acute tubular injury. In the largest European case series of 68 cases by Marie Michel et al, 34 cases had secondary Evans syndrome and, of these, only seven cases had SLE. [3] There are no definite treatment guidelines as most of the recommendations are based on anecdotal reports due to its rarity. [4]

It is very important to distinguish between primary and secondary Evans syndrome as the management and outcome is different and, rarely, splenectomy may be required for primary Evans syndrome with persistently low platelet count, whereas this may not be necessary in autoimmune diseases like SLE. [5]

SLE presenting as Evans syndrome with AKI is unheard of and is one of the first cases to be recorded. The aim of presenting this case is to be aware of this syndrome as a presenting feature of SLE and, if present, to screen for secondary causes, which when detected early can be appropriately treated in time.

 
   References Top

1.
Evans RS, Duane RT. Acquired hemolytic anemia; the relation of erythrocyte antibody production to activity of the disease; the significance of thrombocytopenia and leukopenia. Blood 1949;4:1196-213.  Back to cited text no. 1
[PUBMED]    
2.
Delezé M, Oria CV, Alarcón-Segovia D. Occurrence of both hemolytic anemia and thrombocytopenic purpura (Evans' syndrome) in systemic lupus erythematosus. Relationship to antiphospholipid antibodies. J Rheumatol 1988;15:611-5.  Back to cited text no. 2
    
3.
Michel M, Chanet V, Dechartres A, et al. The spectrum of Evans syndrome in adults: New insight into the disease based on the analysis of 68 cases. Blood 2009;114:3167-72.  Back to cited text no. 3
    
4.
Norton A, Roberts I. Management of Evans syndrome. Br J Haematol 2006;132:125-37.  Back to cited text no. 4
    
5.
Dhingra KK, Jain D, Mandal S, Khurana N, Singh T, Gupta N. Evans syndrome: A study of six cases with review of literature. Hematology 2008;13:356-60.  Back to cited text no. 5
    

Top
Correspondence Address:
Satish Mendonca
Departments of Nephrology, Armed Forces Medical College and Command Hospital, Southern Command, Pune, Maharashtra
India
Login to access the Email id


DOI: 10.4103/1319-2442.174177

PMID: 26787583

Rights and Permissions


    Figures

  [Figure 1]



 

Top
   
 
 
    Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  
 


 
    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures
 

 Article Access Statistics
    Viewed3137    
    Printed22    
    Emailed1    
    PDF Downloaded665    
    Comments [Add]    

Recommend this journal