CLINICAL PATHOLOGICAL CONFERENCE CUTANEOUS LEUKOCYTOCLASTIC VASCULITIS AND MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS WITH MONOCLONAL IGM GAMMOPATHY . Andrea Angioi Divisione di Nefrologia, Dialisi e Trapianto AO Brotzu, Cagliari (CA), IT
Clinical Challenge 50 years old Caucasic Man 2006: Microscopic Hematuria MGUS IgM-K and Leukocytoclastic Cutaneous Purpura Clinical Examination No swollen/enlarged lymph nodes No Epato/Splenomegaly Lower limbs purpura No impairement of the renal function sCr 1.3 mg/dL Creatinine clearance was normal Isolated Microscopic Hematuria No urinary proteins or light chains Cryoprecipitation test and C3/C4 were normal
Clinical Challenge General Parameters: Good objective/subjective feeling Height: 183 cm Weight: 82 Kg – BMI 25.6 (slight overweight) BP: 130/80; 14 B/M No Fever (36,6°C) HR: 75/bfm Job: Phisician No evidence of familiar kidney diseases or hematological malignances. Remote pathologic history: Post-Streptococcical Glomerulonephritis (3 yrs) Tonsillectomy (8 yrs)
Clinical Challenge 2004: Monoclonal IgM K Gammopathy No epatosplenomegaly No swollen/enlarged lymph nodes No haematological-related synthoms No organ injuries No renal abnomalies
Clinical Challenge 2005: First episode of Purpuric Lesions Skin Biopsy was diagnostic for Leukocytoclastic Cutaneus Vasculitis; Immunofluorescence antisera were negative for IgG, IgA, IgM and C3; No clinical/laboratoristic findings for Cryoglobulinemia.
Clinical Challenge 2006: Microscopic Hematuria Instrumental Exams There weren’t pathological findings in both Chest Rx Chest Rx and Abdominal ETG Abdominal ETG . No plasmacells infiltration in the Bone Bone Marrow Biopsy were found. Marrow Biopsy
Clinical Challenge 2007 – 2008: Occasional Microscopic Hematuria Good general conditions No hematological symptoms Recurrent Leukocytoclastic Purpura without signs of Renal impairment or Proteinuria Cryocrit: Negative Hypocryoprecipitation test : Negative 2008: Light chains oligoclonality in urines Serum Light chains k: 9.8 mg/L Serum Light chains λ : 18.6 mg/L k/ λ : 0.52
Clinical Challenge 2009, November: Renal Biopsy Microscopic Hematuria Mild Proteinuria ( 300 mg/24h) Mild increase of sCr ( 1.46 mg/dl) GFR (C&G formula): 71.59 ml/min eGFR (MDRD Formula): 57 ml/min Mild C3 reduction ( 73 ng/dl)
Clinical Challenge - Lab findings pre-Bio (15.11.2009) Haematology Value Unit Viral Markers Value WBC 7.32 x10 3 /uL HCV Ab neg x10 6 /uL HCV-RNA quant. neg RBC 4.22 HBsAg neg Hgb 12.2 g/dL Anti HBs neg Hct 36.6 % fL MCV 86.8 MCH 28.9 pg MCHC 33.2 g/dL x10 3 /uL PLT 239 Formula Value % Neutr 60.1 % % Lymph 27.1 Mono 7.2 % Eosino 3.5 % Baso 0,3 % LUC 1.7 %
Clinical Challenge - Lab findings pre-Bio (15.11.2009) Renal Chemistry Value Unit Immunology Value Unit mg/dL mg/dL sCr 1.45 IgG 569 mg/dL mg/dL BUN 33 IgM 516 Gly 104 mg/dL IgA 86 mg/dL mEq/L ng/dL Na 139 C3 76 mEq/L mg/dL K 4.4 C4 16.5 mEq/L Cl 105 Cryocrit Neg mg/dL mg/dL Ca 9.4 Serum Light Chains K 173 P 4.5 mg/dL Serum Light Chains L 108 mg/dL mOsm/L Osm 285 Rheumatoid Factor Neg
Clinical Challenge - Lab findings pre-Bio (15.11.2009) Urinalysis Value Unit Urine Sediment Val Unit mg/dL Glucose Neg Red cells +++ pH 6.0 White Cells + Chet. Neg Fungi - Proteins Neg mg/dL Bacteria - Bilirubin Neg mg/dL Cells - Hemoglobin Strong Crystals - Urobilin 0.2 UE/dL Casts - Nitrits Neg PS 1.017 Color Yellow Appearance Clear
Clinical Challenge 2009, Dicember: Bone Marrow Aspirate A CD19+ polyclonal lymphocyte infiltrate and a Plasmacell infiltration <1% was described.
Clinical Challenge Renal Biopsy Renal Biopsy Clinical pattern of Clinical pattern of - - Rapidly Progressive Rapidly Progressive Glomerulonephritis (RPGN) Glomerulonephritis (RPGN)
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