A Middle-Aged Woman Presented with Catastrophic Anti-phospholipid Syndrome in Nephrology Department of BSMMU

A life threatening rare condition called catastrophic antiphospholipid syndrome leading to multiple organ failure is characterized by vascular thrombosis in the presence of anti-phospholipid antibody which often appear as a medical emergency. Antiphospholipid antibody syndrome whether primary or secondary cause thromboembolic manifestation resulting recurrent fetal loss, but catastrophic antiphospholipid antibody syndrome may not present in such a way, rather multi-system involvement occurs within a short period of time. We would like to present a case of 50 years old female who is hypertensive, non-diabetic, a known case of hypothyroidism for two years, who was admitted to our hospital after developing fever for 7 days and black discoloration of lateral three fingers of left hand for short period of time. The patient had no medical problems and had been in her usual state of health until 7 days before admission. Patient is anaemic and found to have severe renal failure. She was found high titer antiphospholipid antibody both IgM and IgG positive and anti-cardiolipin antibody positive. Her routine investigations revealed very high neutrophilic leukocytosis, high acute phase reactant, urinary findings revealed no active sediment; we thought that our patient might have sepsis that may trigger catastrophic antiphospholipid syndrome. Multi-disciplinary consultation gave us valuable opinion. Considering her septicemia, she was given broad spectrum antibiotic. Anticoagulation was given with unfractionated heparin followed by warfarin and as an immunosuppressive protocol methylprednisolone followed by prednisolone along with pulse cyclophosphamide was given. Treatment option with plasma pheresis and monoclonal antibody was not attempted, but she was given several session of hemodialysis, within a few days her biochemical parameters improved. Severe renal failure in this patient may be explained by septicemia or thrombotic micro-angiopathy that was reversed with anti-coagulation or proper antibiotic. Amputation of three digit of left hand was done by orthopedic surgeon but unfortunately two weeks after admission the patient expired due to sudden stroke.