Background: January 1994 and December 2010

Background
There has been no report on the clinical features or natural history

of autoimmune hemolytic anemia (AIHA) in the Erbil adult
population. This study retrospectively analyzed the clinical
characteristics and presentation of AIHA in this locality.
Subjects and Methods
Patients newly diagnosed with AIHA between January 1994 and
December 2010 at Nanakaly Hospital for Blood Disorders were
enrolled in this work. Patient characteristics at diagnosis, response
to treatment, and the natural course of the disease were
documented.
Results
Thirty-two patients (31 females and 1 male) with a median age of
48 years (range, 17-86) were enrolled. Of these, 21.9% were
initially diagnosed with secondary AIHA. Thirteen patients
(40.6%) were initially diagnosed with Evans' syndrome. Of the 29
patients who were placed on therapy, 27 (93.1%) showed a partial
response or better. Nevertheless, 1 year after initiating treatment,
80% of the patients were still treatment-dependent.
During follow-up (median length 14 months; range, 0.5-238), 14 of
25 patients (56.0%) who were initially diagnosed with primary
warm antibody AIHA were found to have systemic lupus
erythematosus (SLE). Median time to conversion to SLE was 8.0
months (95% CI, 4.3-11.7), and the probabilities of conversion at
12 and 24 months were 63% and 91%, respectively. Younger age
(＜60 years) and a positive fluorescent anti-nuclear antibody test
were associated with a higher probability of SLE conversion
(P=0.01 and P＜0.001, respectively).
Conclusion
Primary AIHA is rare. Regular, vigilant testing for SLE is required
in patients initially diagnosed with AIHA.
Key Words Autoimmune hemolytic anemia, Evans' syndrome,
Systemic lupus erythematosus, Thrombosis
Introduction
Autoimmune hemolytic anemia (AIHA) is defined as the increased
destruction of red blood cells (RBCs) in the presence of anti-RBC
autoantibodies [1]. AIHA is a relatively uncommon cause of
anemia. Recent population-based studies have calculated the
incidence of AIHA to be 0.8/100,000/year [2], and its prevalence to
be 17/100,000 [3]. AIHA may be primary (idiopathic) or secondary
to various diseases, including systemic autoimmune disorders [4-
6], malignancies [7], and infections [8, 9]. AIHA can also be
induced by certain drugs [10, 11]. This disorder is heterogeneous
with respect to the type (warm or cold) of antibodies involved. In
spite of a long history of this disorder, management of AIHA is still
mainly based on empirical data and on the results of small,
retrospective, uncontrolled studies. Therapies for AIHA have been
reviewed by several experts [12-15], but treatment guidelines have
not yet been established. The current recommendations for the
diagnosis and management of this disorder originate from Western
Europe and North America, where the epidemiology of
hematologic disorders may be different from that in the Orient.
Although a few studies have described the clinical characteristics
of AIHA in the Asian populations [11, 16-20], information from
Asian regions is still limited. Furthermore, there has been little
report on the clinical features or natural history of AIHA in the
Korean adults. In the present study, we retrospectively analyzed
clinical characteristics and outcomes of patients with AIHA in our
institute.
Subjects and Methods

1. Patients
Patients who were consecutively diagnosed with AIHA based on
positive results to either Coombs' test or cold agglutinin assay, at
Chungnam National University Hospital between January 1994
and December 2010, were enrolled.
All patients were Koreans. Patients with drug-induced hemolytic
anemia were excluded. All patients underwent the following
laboratory investigations: CBC with reticulocyte counts, peripheral
blood smear, chemistry (including lactate dehydrogenase [LDH]
and direct and indirect bilirubins), urine analysis, serum
haptoglobin, plasma hemoglobin, direct and indirect Coombs' tests,
and cold agglutinin assay. Screening tests for SLE, including
fluorescent anti-nuclear antibody (FANA), complement-3 (C3),
and -4 (C4) tests, were also performed. Patients who were positive
for FANA underwent additional studies for autoantibodies, such as
anti-double strand (ds) DNA antibody and anti-Smith antibody.
Lupus anticoagulants (LA) and anti-cardiolipin antibodies (aCL)
were examined. Bone marrow studies were performed to rule out
lymphoproliferative disorders. SLE was diagnosed according to the
American College of Rheumatology revised classification criteria
for SLE [21]. Patients fulfilling only 3 of the revised classification
criteria for SLE from the American College of Rheumatology were
defined as having “incomplete” SLE [22]. Evans' syndrome was
diagnosed, if the patient tested positive for hemolytic anemia by
the Coomb's test, and for idiopathic thrombocytopenic purpura, in
the absence of any known underlying etiology.
2. Treatment and response evaluation

Patients with AIHA who did not present with Evans' syndrome
(hereafter described as “AIHA only”) received oral prednisolone
(Pd) alone (1 mg/kg/d) or intravenous (IV) methylprednisolone
(methyl-Pd; 10 mg/kg/d for 3 d) followed by Pd, whereas patients
with Evans' syndrome received corticosteroids (oral Pd alone or IV
methyl-Pd followed by Pd) or IV immune globulin (IVIg) as first-
line therapy. Response criteria [22] were classified as follows:
(a) complete response (CR), defined as a stable hemoglobin level
of ＞12 g/dL, no requirement for transfusion, and absence of
clinical and laboratory signs of hemolysis (no jaundice,
normalization of serum LDH, haptoglobin and indirect bilirubin
levels, and normal reticulocyte count), irrespective of Coombs' test
result; (b) partial response (PR), defined as a rise in hemoglobin
levels of ＞2 g/dL, no or reduced transfusion requirement, and
improvement of clinical and laboratory signs of hemolysis; and (c)
no response
3. Follow-up
Patients were followed up every 1-2 weeks until responses were
achieved, and then every 2-3 months. Investigations for SLE were
performed annually in most patients. Conversion to SLE and
complications such as renal insufficiency and thrombosis were
evaluated.
4. Statistical analyses
Continuous variables were analyzed using a Student's t test, and
binary variables were analyzed using a Chi-square test. The
probability of conversion to SLE in patients with primary AIHA
was plotted as Kaplan-Meier curves, and probabilities with respect
to risk factors were compared using the log-rank test and a Cox
proportional hazard model. All analyses were performed using
SPSS version 17.0 (SPSS Inc., Chicago, IL). P -value of ＜0.05
was considered statistically significant.

Background: January 1994 and December 2010

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Background: January 1994 and December 2010

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Background

There has been no report on the clinical features or natural history

Subjects and Methods

2. Treatment and response evaluation

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