Hemophagocytic Syndrome, an Uncommon Complication of Microscopic Polyangitis: A Case Report From Senegal.

INTRODUCTION

We reported a case of hemophagocytic syndrome complicating microscopic polyangitis presented by crescentic glomerulonephritis.

CASE PRESENTATION

A 22-year-old female patient originated from Dakar, Senegal presented with nephrotic syndrome and rapidly progressive glomerulonephritis. On physical examination, we noticed hyperchromic diffuse punctilious purpura skin lesions predominant on the trunk, the neck and the upper thigh. Immunology investigations revealed strongly positive anti SSA/Ro and anti-SSB. Anti-neutrophil cytoplasmic antibodies had positive results with a peri-nuclear type fluorescence, specific to myeloperoxidase. In optic microscopy, renal biopsy showed a crescentic glomerulonephritis with circumferential cellular and fibrous proliferation affecting 85% of glomeruli. The diagnosis of microscopic polyangitis with renal and skin involvement was retained. The patient received methylprednisolone and cyclophosphamide 700 mg/m(2) every 15 days for the first 3 pulses and every 21 days thereafter. After the 5(th) month, she developed obnubilation, fever and central pancytopenia. Bone marrow aspiration was performed, which showed medullary invasion by macrophages with signs of hemophagocytosis. Diagnosis of hemophagocytic syndrome complicating a microscopic polyangitis was retained and methylprednisolone pulses started. The patient was under hemodialysis after follow-up of about 9 months with stable clinical state.

CONCLUSIONS

The occurrence of SAM in pauci-autoimmune vasculitis is rarely described, particularly in Africa. Our case is an illustration of the reality of this association.