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

BACKGROUND: Extracellular trap formation (ETosis) by various blood cells has been reported. This trap contains DNA, histones and granular proteins which can elicit an innate immune response by entrapping microorganisms. The trap thus formed has been reported to have an involvement in various pathogenic conditions as well. This review focusses on the trap formation by different blood cells, the immune response associated with trap formation and also its role in various clinical conditions.METHOD: An extensive literature survey on ETosis by blood cells from 2003 to 2019 has been done. After going through the literature throughly, in this review we focuses on the trap formation by different blood cell types such as neutrophils, macrophages, eosinophils, basophils, mast cells, plasmacytoid dentritic cells, and monocytes. The mechanism with which it releases trap, the immune response it elicits and ultimately its involvement in various pathogenic conditions are described here. This article extensively covered all the above aspects and finally comprehends in nutshell the various stimuli that are currently known in trigerring the ETosis, its effect and ultimately its role in disease process. RESULTS: A clarity about the extracellular trap formation by various blood cells, mechanism of ETosis, role of Etosis in microbial invasion and in various pathogenic situations by various blood cells have been described here. CONCLUSION The current understanding about the process of ETosis and its effects has been extensively described here. Along with lot of favourable outcomes, the process of ETosis will lead to lot of pathogenic situations including thrombosis, tumour metastasis and sepsis. Current understanding about ETosis is limited. Indepth understanding of ETosis may have great therapeutic potential in the diagnosis, guiding of therapy and prognostication in various pathogenic situations including infectious conditions, autoimmune disorders and tumors.