Significance of autoimmune haemolytic anaemia and immune thrombocytopenia (Evans' syndrome) in systemic lupus erythematosus.
Patients with systemic lupus erythematosus (SLE) have an extremely variable prognosis and mortality. The purpose of this report is to highlight the importance of looking for lupus parameters in patients with autoimmune haemolytic anaemia (AIHA) and/or immune thrombocytopenia (ITP) as this represents a subgroup of systemic lupus erythematosus (SLE) patients with a fairly good prognosis. We report a case of an 8-year-old Malay boy who was admitted to hospital with fever and gum bleeding. Physical examination revealed a malar rash, oral ulcers, petechial haemorrhages and bruises over the limbs, generalised lymphadenopathy and hepatosplenomegaly. Laboratory investigations confirmed the diagnosis of SLE. The patient's serum showing the presence of antinuclear factor, antiphospholipid antibodies and a biological false-positive test for syphilis. Immunological and haematological parameters were in keeping with combined AIHA and ITP (Evans' syndrome). No organ involvement was present and the patient responded well to corticosteroid therapy. This case demonstrates the importance of making an early diagnosis of SLE with haematological complications, in order to ensure full benefit of therapy and emphasises the good prognosis expected in this subgroup of SLE patients.
Pasangna
J
J
Division of Haematology, Institute for Medical Research, Kuala Lumpur.
Lim
C B
CB
Duraisamy
G
G
Lajin
I
I
D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D002648:Child; D042241:Early Diagnosis; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D013921:Thrombocytopenia / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D016896:Treatment Outcome
D005938:Glucocorticoids; D011239:Prednisolone