Real World Experience of Clinical Profile and Outcomes of Autoimmune Hemolytic Anemia: Single Centre Experience

Background: Autoimmune haemolytic anaemia (AIHA) is a relatively uncommon condition for acute fall in haemoglobin with grave consequences if not diagnosed and treated promptly. The literature on the clinical outcome, response to treatment, and occurrence of acute complications is relatively scarce from the real world.

Aim: We retrospectively analysed 100 cases of autoimmune haemolytic anaemia (AIHA) for clinic-pathological features and response to therapy.

Patients and methods: It is a single centre retrospective analysis of all cases of AIHA managed at our centre from Jan 2010- June 2016. Patients were analysed for the demographic features, presenting complaints, clinical/ pathological findings at diagnosis, types of therapy given and response to therapy. AIHA was diagnosed by features suggestive of haemolysis (anaemia with unconjugated hyperbilirubinemia, raised LDH and reticulocytosis) and/or positive DAT in the absence of any underlying secondary causes for anaemia according to the monospecific-DAT results and the thermal characteristics of the autoantibody, patients were classified as warm AIHA (DAT positive for IgG with or without C3D), cold agglutinin disease (DAT positive for C3D only with cold agglutinins), mixed (DAT positive for IgG and C3d with coexistence of warm autoantibodies and high titre cold agglutinins), or atypical AIHA (either DAT negative). Based on the severity of anaemia, patients were classified into very severe (Hb≤60gm/L), severe (Hb-61 to 80gm/L), moderate (Hb-81 to 100gm/L) and mild (Hb>100gm/L).

Results: A total of 100 consecutive patients were included fulfilling the inclusion criteria. The median follow-up of the study population was 17.7months (mean - 28.2m, range-0.3-197m). The median age of the patients was 33y (mean 36.3y: range 13-80y). There was female predominance (n=64, 64%). Easy fatiguability (85%) followed by dyspnoea on exertion (75%) were commonest presenting complaints. The median duration of complaints was 3 months (mean 8.93: range 0.2- 96). Jaundice (58%) and cola coloured urine (11%) were other important manifestations. Preceding history suggestive of viral infections was present in 22% of the patients and only one patient had significant drug history temporally correlating with AIHA. Splenomegaly was present in 67% with a median palpability of 2cm (mean 2.71: range 0- 20) and hepatomegaly in 49% with a median palpability of 1cm (mean 1.402: range 0 - 8) below the coastal margin. Lymphadenopathy was present in 8% of the patients.

On severity grading 67% had very severe anaemia, 21% had severe, 9% moderate anaemia and 1% had mild anaemia. 24% of the patients had simultaneous low platelet counts less than 1 lakh. 17% patients had atypical AIHA - DAT negative. 21% patients had secondary AIHA of which eight patients had simultaneous low platelets (suggestive of Evans). The commonest cause for the secondary AIHA was rheumatological disorders (SLE 15 patients, MCTD one patient) followed by lymphomatous disorders in rest.

Most common therapeutic modality employed was corticosteroids (methyl prednisolone pulse therapy n-20, prednisolone n-67), followed by IvIg (n-4), chemotherapy (RCHOP-1, RCVP-1, CVP-1) and rituximab (100mg/wk - 1, 375mg/m²/wk - 2). 75% of the patients had some clinical response (CR 47%, PR 22%, SD 6%) to the first line therapy. The median time to response was 1.5 months. 52% of the patients required repeat therapy in the form either steroid-sparing agent, immunosuppressant or repeat course of steroids. Six patients required splenectomy. The response to second-line therapy was seen in 92% of patients (CR 31/52, PR 9/52, SD 8/52). More than two lines/ times of therapy for non-response/relapse was required in 10% of the patients, of whom, 50% of the individuals were steroid dependent. A total of two patients succumbed to the illness (first owing to tuberculosis possible exacerbated by immunosuppressive therapy, and second due to progressive disease).

Limitations of the study: All limitations of a retrospective study are applicable to our study as well. The selection of patients with fairer prognosis can explain a lower mortality in our study.

Conclusion: Primary AIHA is the commonest form of AIHA. Most secondary AIHA has rheumatological etiology. Response to steroids is good leading to considerable remission but which is not long lasting and majority requiring second line/ second time therapy with same or alternative agents.