ABSTRACT
The reported incidence of thrombocytosis among cases of refractory anaemia with ring sideroblasts (RARS) may be as high as 15%. We report four additional cases of this association, which appear to be hematologically heterogeneous. One patient clearly represents a case of RARS with reactive thrombocytosis. Two cases have features suggestive of the coincidental occurrence of essential thrombocythemia and RARS. The fourth case could be best classified as a subtype of myelodysplastic/myeloproliferative disease unclassifiable (MDS/MPD U). Only new biological or molecular markers will allow better differentiation between these disorders.
Subject(s)
Anemia, Sideroblastic/diagnosis , Myelodysplastic Syndromes/diagnosis , Myeloproliferative Disorders/diagnosis , Thrombocytosis/diagnosis , Aged , Aged, 80 and over , Anemia, Sideroblastic/complications , Blood Sedimentation , Bone Marrow/pathology , Diabetes Mellitus, Type 2/complications , Diagnosis, Differential , Erythroblasts/pathology , Heart Failure/complications , Humans , Karyotyping , Male , Myelodysplastic Syndromes/classification , Myeloproliferative Disorders/classification , Pneumonia/complications , Thrombocytosis/complicationsABSTRACT
A cohort of 148 consecutive patients with hepatitis C virus infection were studied at the rheumatology out-patient clinic of a tertiary care teaching hospital. The diagnosis of hepatitis C virus infection was supported by detection of HCV RNA in the serum. Cryoglobulin screening was done in all patients and the presence of a monoclonal component was investigated when the cryocrit was higher than 1%. Patients with lymphoproliferative disorders were further investigated. Four patients had a B lymphoproliferative disorder, which represents a prevalence of 2.7% in this cohort of patients with hepatitis C virus infection. Mixed cryoglobulinemia (MC), with cryocrit higher than 1%, was found in 16 of 148 patients (11%). It was type III MC in 13 patients and type II MC in 3. All patients who developed a B lymphoproliferative disorder had mixed cryoglobulinemia, with a monoclonal component (type II MC) in two patients and without a monoclonal component (type III MC) in the other two. The incidence of B-lymphoproliferative disorders among this cohort of patients with hepatitis C virus infection seems to be significantly increased. However, the high frequency of asymptomatic, undiagnosed HCV infection among the apparently healthy general population may decrease the true significance of this association. Systematic screening of cryoglobulin production in patients with hepatitis C virus infection might clarify whether the risk of B lymphoproliferative disorders increases when type II or type III mixed cryoglobulinemia is present.
