M. Faye

Light Chain Deposition Disease in Black African, Report of Three Cases from Senegal

Background: Although there are numerous publications on multiple myeloma in black people, it seems sub-Saharan literature particularly Senegalese’ one is silent about Light chain deposition disease (LCDD) which is linked to immunoglobulin light chain deposition in glomerulus. The authors report the first three observations of LCDD collected in Nephrology department of Aristide Le Dantec hospital in Dakar, Senegal. Cases: we report three cases a man and two women of 61, 69 and 47 years old respectively, admitted to the Nephrology department of Aristide Le Dantec hospital for rapidly progressive renal failure in one case and a nephrotic syndrome in 2 other cases. The renal biopsy showed a nodular glomerulosclerosis and immunofluorescence microscopy, revealed deposition of IgG light chains suggesting LCDD. On the other hand, the diagnosis of multiple myeloma of IgG kappa type in 2 cases and IgG lambda type in the other one was done. A combination of chemotherapy (Mephalan Prednisone) and hemodialysis was instituted for all 3 cases. The evolution was marked by the appearance of an end stage renal disease in 2 cases and the third one was expired due to an infected bed sore secondary to a pathological fracture of the neck of the femur. Conclusion: Although the LCDD is rare, the prognosis of this syndrome seems to be poor as more than half of the patients die or progress to ESRD within 2 years.

Scleroderma renal crisis in tropical region: two senegalese cases

Scleroderma renal crisis (SRC) is defined as the new onset of accelerated arterial hypertension and /or rapidly progressive oliguric renal failure during the course of systemic sclerosis. It is a rare but life-threatening complication. This formerly serious complication has got a considerable brighter outlook since the introduction of angiotensin converting enzyme inhibitors (ACE) however the mortality is still remaining high. We report two cases of SRC which to our knowledge are the firsts described in Dakar. They were two women aged 45 and 32 years, one of them was previously following for systemic sclerosis. Both of them had malignant hypertension associated with rapidly progressive renal failure, the other was put under corticosteroid therapy four months before SRC occurrence. The histological and laboratory finding showed thrombotic microangiopathy. The height blood pressure returned to normal value after treatment with ACE inhibitors. The final outcome was undesirable with the death of one after two months due to the hemodialysis discontinuation and persistence of renal failure in the other.

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/m2 every 15 days for the first 3 pulses and every 21 days thereafter. After the 5th 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.