| Literature DB >> 31388268 |
Fergün Yılmaz1, Demet Kiper2, Meltem Koç3, Tuğçe Karslı2, Merve Kılınç2, Fusun Gediz4, Tayfur Toptaş1, Bahriye Payzın2.
Abstract
We aimed to analyze 10-year experience of WAIHA patients at a single referral center in Turkey. Clinical data, survival outcome of sixty patients who were diagnosed with WAIHA were retrospectively analyzed. All the patients were direct antiglobulin test (DAT) positive. In 21 (30%) patients, IgG plus C3d DAT positivity was documented. 16 patients were secondary WAIHA and most common underlying causes were lymphoproliferative diseases (5 patients) and connective tissue disease (8 patients). Corticosteroids were first choice as a first line therapy with 54.5% CR and 40.2% PR rates. 43.3% of the patients relapsed after a median 12 months. In relapsed patients, rituximab and splenectomy achieved 85% overall response rates. The median OS was not reached. The median DFS was 40 months (95% CI, 19.6-60.4). OS and DFS at 36 months were 89.6% and 51.1%, respectively. DFS at 36 months was lower in patients with IgG plus C3d positive DAT than patients with only positive Ig G DAT (36 vs. 54%) but this difference could not reach statistical significance (p = 0.23). WAIHA was a rare disease with a good prognosis. Corticosteroids were the first option and splenectomy and rituximab received good responses in relapsed patients. Attention should be paid especially in patients with IgG plus C3d DAT positivity since lower DFS were reported. Characteristics and pathogenesis of patients with IgG plus C3d DAT positivity was still an obscure.Entities:
Keywords: Mixed positive direct antiglobulin test; Treatment; Warm autoimmune hemolytic anemia
Year: 2019 PMID: 31388268 PMCID: PMC6646622 DOI: 10.1007/s12288-019-01103-y
Source DB: PubMed Journal: Indian J Hematol Blood Transfus ISSN: 0971-4502 Impact factor: 0.900