Blood Res 2016; 51(2):
Published online June 23, 2016
https://doi.org/10.5045/br.2016.51.2.88
© The Korean Society of Hematology
Department of Medical Oncology and Hematology, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham University, Kochi, India.
Correspondence to : Correspondence to Raghuveer Prabhu, M.D. Department of Medical Oncology and Hematology, Amrita Institute of Medical Sciences, Amrita Lane, Ponekkara P.O., Edappally, Kochi 682041, Kerala, India. sraghuveerprabhu@hotmail.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Autoimmune 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.
Data for 33 patients with primary AIHA recorded from July 2009 to June 2015 were retrospectively analyzed for clinical presentation, response to frontline therapy, durability of response, time to next treatment (TTNT), and response to second-line agents.
The 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 abscess) and two had life-threatening venous thrombosis. One case of death was recorded.
AIHA is a heterogeneous disease that requires care by physicians experienced in treating these patients.
Keywords Autoimmune hemolytic anemia, Warm autoimmune hemolytic anemia, Cold agglutinin disease, Corticosteroids, Azathioprine
Blood Res 2016; 51(2): 88-94
Published online June 23, 2016 https://doi.org/10.5045/br.2016.51.2.88
Copyright © The Korean Society of Hematology.
Raghuveer Prabhu*, Renjitha Bhaskaran, Veena Shenoy, Rema G, and Neeraj Sidharthan
Department of Medical Oncology and Hematology, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham University, Kochi, India.
Correspondence to: Correspondence to Raghuveer Prabhu, M.D. Department of Medical Oncology and Hematology, Amrita Institute of Medical Sciences, Amrita Lane, Ponekkara P.O., Edappally, Kochi 682041, Kerala, India. sraghuveerprabhu@hotmail.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Autoimmune 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.
Data for 33 patients with primary AIHA recorded from July 2009 to June 2015 were retrospectively analyzed for clinical presentation, response to frontline therapy, durability of response, time to next treatment (TTNT), and response to second-line agents.
The 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 abscess) and two had life-threatening venous thrombosis. One case of death was recorded.
AIHA is a heterogeneous disease that requires care by physicians experienced in treating these patients.
Keywords: Autoimmune hemolytic anemia, Warm autoimmune hemolytic anemia, Cold agglutinin disease, Corticosteroids, Azathioprine
Sequence of the therapies and clinical outcomes in the primary autoimmune hemolytic anemia.
Abbreviations: AIHA, autoimmune hemolytic anemia; WAIHA, warm AIHA; CAD, cold agglutinin disease; CR, complete remission; PR, partial remission; NR, no response; AZA, azathioprine; Ritux, rituximab; CsA, cyclosporin; Tf, transfusion; MMF, mycophenolate mofetil; IVIG, intravenous immunoglobulin.
Abbreviations: WAIHA, warm autoimmune hemolytic anemia; CAD, cold agglutinin disease..
a)Mild anemia=Hb 10.1–12, moderate anemia=Hb 8.1–10, severe anemia=Hb 6.1–8, very severe anemia=Hb<6..
Abbreviations: WAIHA, warm autoimmune hemolytic anemia; CAD, cold agglutinin disease; Hb, hemoglobin; LDH, lactate dehydrogenase..
a)CR≥12.0 g/dL Hb, PR=10.1–12.0 g/dL Hb..
Abbreviations: WAIHA, warm autoimmune hemolytic anemia; CAD, cold agglutinin disease; CR, complete response; PR, partial response..
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Sequence of the therapies and clinical outcomes in the primary autoimmune hemolytic anemia.
Abbreviations: AIHA, autoimmune hemolytic anemia; WAIHA, warm AIHA; CAD, cold agglutinin disease; CR, complete remission; PR, partial remission; NR, no response; AZA, azathioprine; Ritux, rituximab; CsA, cyclosporin; Tf, transfusion; MMF, mycophenolate mofetil; IVIG, intravenous immunoglobulin.