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Case Reports
. 2015 Nov 29;7(6):e30284.
doi: 10.5812/numonthly.30284. eCollection 2015 Nov.

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

Mouhamadou Moustapha Cisse  1 , Daher Abdoul Karim Omar  1 , Jean De Dieu Nzambaza  1 , Sidy Ba  1 , Awa Cheikh Ndao  2 , Abibatou Sall  3 , Cherif Mouhamed Dial  4 , Maria Faye  1 , El Hadji Fary Ka  1 , Moustapha Faye  1 , Ahmed Tall Lemrabott  1 , Abdou Niang  1 , Boucar Diouf  1
Affiliations
Case Reports

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

Mouhamadou Moustapha Cisse et al. Nephrourol Mon. .

Abstract

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.

Keywords: Crescentic Glomerulonephritis; Hemophagocytic Syndrome; Microscopic Polyangitis.

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Figures

Figure 1.
Figure 1.. Disseminated Vascular Purpura on the Back
Figure 2.
Figure 2.. Kidney Biopsy Showed Crescentic Glomerulonephritis
A, Three glomeruli (arrow) focus of circumferential crescents, interstitial inflammatory fibrosis with tubular atrophy. Trichrome of Masson. Power 150; B, A glomerulus with global crescentic glomerulonephritis. Trichrome of Masson Power 300.
Figure 3.
Figure 3.. Bone Marrow Aspiration Showed Hemophagocytosis
A, Normal macrophage; B, Macrophage with healthy cells; C, Macrophages with cells in digestion; D, Macrophages with cells in digestion.
See this image and copyright information in PMC

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