Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can be associated with a variety of haematological manifestations. Haemolytic anaemia may be the first isolated clinical presentation of SLE.Aim: To study haemolytic anaemia in relation to various clinical and serological manifestations in SLE patients.Methods: The medical files of 107 lupus patients admitted to the Rheumatology Department at Kasr El-Aini Hospital, during the period from 1-1-2009 to 31-12-2010 were retrospectively reviewed. Twenty one SLE patients with haemolytic anaemia (Group A) were identified and compared to other SLE patients without haemolytic anaemia (86 patients) (Group B) in relation to various clinical and serological perspectives which were available in the database. Both groups were matched for age, sex, disease duration and age of onset.Results: The studied lupus patients with haemolytic anaemia had significantly higher frequency of nephritis, CNS manifestations (specifically lupus headache), arthritis, C3 consumption and lymphopenia. Moreover, they had higher degrees of systemic damage (measured by SLICC/ SDI score) and higher activity class (measured by SLEDAI score at the time of the haemolytic episode) compared to lupus patients without haemolytic anaemia. Conclusion: Haemolytic anaemia in SLE may be considered a risk factor for a severe disease with higher frequency of renal, CNS and articular involvement.