Primary antiphospholipid antibody syndrome and autoimmune haemolytic anaemia--a rare combination.
Primary Antiphospholipid Antibody Syndrome (PAPS) and Autoimmune haemolytic anemia (AIHA) is a very rare combination. Antiphospholipid Antibody Syndrome (APS) with underlying SLE, however, has a well documented association with Coomb's positive Auoimmune Haemolytic Anaemia. We describe a young girl with PAPS presenting with deep venous thrombosis, livedo reticularis and features of AIHA. The patient was refractory to treatment for 5 years however, her condition improved dramatically with anticoagulants, corticosteroid therapy and the addition of hydroxychloroquine and azathioprin. We have also discussed hydroxychloroquine therapy in PAPS which is not yet fully established and the probability of this patient developing other autoimmune disorders in future.
Suhail
Saera
S
Rheumatology Department, Baqai Medical University, Nazimabad, Pakistan.
Baig
Muhammad Shakil
MS
Humail
Syed Mujahid
SM
Riaz
Amir
A
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D018501:Antirheumatic Agents / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D015897:Comorbidity; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D014859:Warfarin / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D000925:Anticoagulants; D018501:Antirheumatic Agents; D005938:Glucocorticoids; D006886:Hydroxychloroquine; D014859:Warfarin; D011239:Prednisolone; D001379:Azathioprine