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CASE REPORT
A CASE REPORT OF EVANS SYNDROME
R. V. DOSI, A. P. AMBALIYA, R. D. PATELL, R. S. PATIL, P. J. SHAH
ABSTRACT
Evans Syndrome(ES) is the rare simultaneous or subsequent development of immune
thrombocytopenia purpura(ITP) and autoimmune hemolytic anemia(AIHA). It portends
a poorer prognosis and a more aggressive line of management than either condition
presenting alone. Here we report a case of a young female who presented with both
bleeding and acute decompensated anemia. Although she was successfully treated,
mystery still shrouds the etiology, pathophysiology, as well as line of management of
this rare and enigmatic disease.
Key words: Autoimmune hemolytic anemia, Evans syndrome, immune thrombocytopenic
purpura
INTRODUCTION
CASE REPORT
Indian Journal of Medical Sciences, Vol. 66, No. 3 and 4, March and April 2012
Website:
www.indianjmedsci.org
DOI:
10.4103/0019-5359.110920
PMID:
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EVANS SYNDROME
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DISCUSSION
Since its first recognition in the 1950s, Evans
Syndrome has been identified as an extremely
rare occurrence, seen in less than 0.8-3.7% of
all patients with ITP or AIHA at onset. ES has
long been considered as a rather incidental and
anecdotal combination of simultaneous and
sequential ITP and AIHA and/or autoimmune
neutropenia in the absence of any underlying
cause.[1] More recently, the spectrum of the
disease has broadened, especially in children,
and there is increasing evidence to suggest
that ES may be associated with or show other
diseases or conditions such as systemic lupus
erythematous(SLE), [2] lymphoproliferative
disorders.[3,4]
Indian Journal of Medical Sciences, Vol. 66, No. 3 and 4, March and April 2012
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EVANS SYNDROME
REFERENCES
1. EvansRS, TakahashiK, DuaneRT, PayneR, LiuC.
Primary thrombocytopenic purpura and acquired
haemolytic anemia: Evidence for a common
etiology. AMA Arch Intern Med 1951;87:4865.
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Indian Journal of Medical Sciences, Vol. 66, No. 3 and 4, March and April 2012