PMC4531942_01
Positive C-ANCA in immunofluorescence testing(x100). Centrally accentuated finely granular cytoplasmic immunofluorescence is present in all neutrophils, with no stanining of neutrophil polymorphonuclear lobes.
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Serology showed ANCA positive (C-ANCA pattern) in a titer of 1:640 (Fig. 1) and nasal septal biopsy showed the necrotizing granulomatous vasculitis.(Fig. 2,3).
Nasal septal biopsy shows granulomatous inflammtion with central neutrophilic infiltration and surrounding zone of histiocytes in cental area. The peripheral area shows dense lymphocytic infiltration(H & E stain, x100)
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Serology showed ANCA positive (C-ANCA pattern) in a titer of 1:640 (Fig. 1) and nasal septal biopsy showed the necrotizing granulomatous vasculitis.(Fig. 2,3).
Chest radiogaph shows diffuse air-space consolidation with air-bronchogram in the whole right lung field.
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Chest X-ray showed homogeneous, ill-defined, patch consolidation on right lung field(Fig. 4).
HRCT of the chest shows nonsegmental ground glass opacity with air-bronchogram in the right lung field.
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Hemoglobin was 5.0g/dL, platelet 157,000/mm3 and HRCT showed diffuse pulmonary hemorrhage(Fig. 5) and bronchoscopic examination showed fresh oozing blood from distal airway.
Peripheral blood smear(Wright-Giemsa stain, x1000) shows frequent irregular shaped erythrocyte fragments.
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The peripheral blood smear showed many RBC fragments(Fig. 6); lactate dehydrogenase was increased to 902 IU/L(normal, <500IU/L) with LD1 flipped pattern; haptoglobin was reduced to below 5.8mg/L(normal, 16 to 20Omg/L); Coombs' test and antiplatelet antibody were negative.