Blood Res.  2016 Jun;51(2):88-94. 10.5045/br.2016.51.2.88.

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

• Prabhu R
• Bhaskaran
• Shenoy V
• G R
• Sidharthan N

• KMID: 2309209
• DOI: http://doi.org/10.5045/br.2016.51.2.88

Abstract

BACKGROUND
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.
METHODS
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.
RESULTS
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.
CONCLUSION
AIHA is a heterogeneous disease that requires care by physicians experienced in treating these patients.

Keyword

Autoimmune hemolytic anemia; Warm autoimmune hemolytic anemia; Cold agglutinin disease; Corticosteroids; Azathioprine

MeSH Terms

Adrenal Cortex Hormones
Anemia
Anemia, Hemolytic, Autoimmune*
Azathioprine
Follow-Up Studies
Humans
India*
Prednisolone
Retrospective Studies
Sepsis
Venous Thrombosis
Adrenal Cortex Hormones
Azathioprine
Prednisolone

Figure

• 

  Fig. 1 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.

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