Extended report
Prevalence and clinical features of cryoglobulinaemia in
multitransfused
-thalassaemia patients
R Perniolaa, C De Rinaldisa, E Accoglib, G Lobregliob
a Paediatric Unit,
Vito Fazzi Regional Hospital, Lecce, Italy, b Laboratory Medicine Unit,
Vito Fazzi Regional Hospital, Lecce, Italy
Correspondence to: Dr R Perniola, Via Cerrate Casale 6, I-73100 Lecce, Italy.
Accepted for publication 16 June 1999
OBJECTIVE
The aim of
the study was to determine the prevalence of cryoglobulinaemia and its
clinical features among
-thalassaemia patients.
METHODS
Eighty eight
multitransfused
-thalassaemia patients were studied. They were
physically examined and asked about the presence of cryoglobulinaemia
related symptoms. Hepatitis C virus (HCV) serology, HCV-RNA, HCV
subtypes, viraemia, serum ferritin, liver and kidney function tests,
rheumatoid factor (RF), circulating immune complexes (CIC), complement
levels and autoantibodies were all evaluated. The patients were divided
into four groups: HCV-RNA positive patients with and without
cryoglobulinaemia (groups A and B), HCV-Ab positive/HCV-RNA
negative patients (group C), HCV-Ab negative patients (group D).
RESULTS
Cryoglobulinaemia
was present in 35 of 53 (66.0%) patients with chronic HCV infection.
They had higher viraemia than non-cryoglobulinaemic viral carriers, but
no statistical difference relating to sex or HCV subtypes was found. In
comparison with the other groups, group A patients were older, had
undergone transfusion therapy for a longer period, had received a
higher number of transfusions, and had increased levels of RF and CIC,
as well as consumption of C4; in addition, they had a higher prevalence
of cirrhosis. Cutaneous lesions (purpura, Raynaud's phenomenon,
nodules and leg rash), peripheral neuropathy and sicca syndrome
symptoms were present only in group A. Musculoskeletal symptoms (bone
pain, arthralgia and myalgia), weakness, splenomegaly, lymphadenopathy, skin ulcers and proteinuria were also commoner in group A, but the
difference did not reach statistical significance, possibly because of
partial overlap between cryoglobulinaemia and
-thalassaemia syndromes.
CONCLUSION
Because of
its high prevalence in multitransfused
-thalassaemia patients,
cryoglobulinaemia needs to be systematically studied and considered in
the differential diagnosis of various
-thalassaemia manifestations.
© 1999 by Annals of the Rheumatic Diseases
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