Clinical characteristics and treatment outcomes of primary autoimmune hemolytic anemia: a single center study from South India

Raghuveer Prabhu; Renjitha Bhaskaran; Veena Shenoy; Rema G; Neeraj Sidharthan

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자료유형: 학술저널

저자정보: Raghuveer Prabhu (Amrita Vishwa Vidyapeetham University) Renjitha Bhaskaran (Amrita Vishwa Vidyapeetham University) Veena Shenoy (Amrita Vishwa Vidyapeetham University) Rema G (Amrita Vishwa Vidyapeetham University) Neeraj Sidharthan (Amrita Vishwa Vidyapeetham University)

저널정보: 대한혈액학회 Blood Research Blood Research Vol.51 No.2

발행연도: 2016.1

수록면: 88 - 94 (7page)

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BackgroundAutoimmune hemolytic anemia (AIHA) is a less recognized, potentially fatal condition. There is a scarcity of data on clinicoserological characteristics and response to therapy concerning this disease from South India.MethodsData for 33 patients with primary AIHA recorded from July 2009 to June 2015 were retro-spectively analyzed for clinical presentation, response to frontline therapy, durability of response, time to next treatment (TTNT), and response to second-line agents.ResultsThe median follow-up period was 50 months. Among 33 patients, 48% of the cases were warm autoimmune hemolytic anemia (WAIHA), 46% were cold agglutinin disease (CAD), and 6% were atypical. Three-fourth of patients had severe anemia (<8 g/dL hemoglobin [Hb]) at onset; younger patients (age <40 yr) had more severe anemia. All of the patients who required treatment received oral prednisolone at 1.5 mg/kg/d as a frontline therapy, and the response rate was 90% (62% complete response [CR] and 28% partial response [PR]). The overall response to corticosteroids in WAIHA and CAD was 87% and 92%, respectively. The median corticosteroid duration was 14 months, and 50% of the patients required second-line agents. Fourteen patients received azathioprine as a second-line agent, and 11 of these patients responded well, with half of them not requiring a third agent. Four patients developed severe infections (pneumonia, sepsis, and soft tissue ab-scess) and two had life-threatening venous thrombosis. One case of death was recorded.ConclusionAIHA is a heterogeneous disease that requires care by physicians experienced in treating these patients.

참고문헌 (22)

참고문헌 신청

Barcellini W / 2014 / Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients / Blood 124:2930~2936

Petz LD / 2004 / Immune hemolytic anemias / Churchill Livingstone

Gehrs BC / 2002 / Autoimmune hemolytic anemia / Am J Hematol 69:258~271

Barcellini W / 2010 / Comparison of traditional methods and mitogen-stimulated direct antiglobulin test for detection of anti-red blood cell autoimmunity / Int J Hematol 91:762~769

Zanella A / 2014 / Barcellini W. Treatment of autoimmune hemolytic anemias / Haematologica 99:1547~1554

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