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Case Report |
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Acquired Angioedema Associated With
Hereditary Angioedema Due to C1 Inhibitor Deficiency |
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M Guilarte,1 O Luengo,1
C Nogueiras,1 M Labrador-Horrillo,1
E Muñoz,1 A López,2 V Cardona1 |
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1Allergy Section, Department of
Internal Medicine, Hospital Universitari Vall d’Hebron,
Universitat Autònoma de Barcelona, Barcelona, Spain
2Department of Haematology, Hospital Universitari Vall
d’Hebron, Universitat Autònoma de Barcelona, Barcelona,
Spain |
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J Investig Allergol Clin Immunol
2008; Vol. 18(2): 126-130 |
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Abstract |
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Angioedema caused by
C1 inhibitor
deficiency is a rare
disorder that may be
either hereditary or
acquired, the latter
being mainly
associated with
lymphoproliferative
disorders. A 51-year-old
woman who had
suffered from
episodes of acute
peripheral edema
since she was 12 was
diagnosed with
hereditary
angioedema at the
age of 40 and
remained stable with
stanozolol. Due to a
worsening of her
symptoms she was
reassessed and low
levels of C1q and an
abnormal lymphocyte
count were detected.
Immunophenotyping of
peripheral blood
revealed 9 %
monoclonal lambda B
cells with a
follicular center
phenotype. The
histopathology was
consistent with a
grade II follicular
lymphoma stage IV-A.
With chemotherapy,
the hematologic
disease was
controlled and C1q
levels returned to
normal values. This
represents a rare
case of a patient
with hereditary
angioedema who
developed acquired
angioedema due to a
lymphoma that was
associated with a
reduction in the
levels of C1q as her
symptoms worsened.
Key words:
Acquired angioedema.
Hereditary
angioedema. C1q. C1
inhibitor deficiency. |
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