CUTANEOUS LEUKOCYTOCLASTIC VASCULITIS AND MEMBRANOPROLIFERATIVE - - PowerPoint PPT Presentation

Andrea Angioi

Divisione di Nefrologia, Dialisi e Trapianto AO Brotzu, Cagliari (CA), IT

CLINICAL PATHOLOGICAL CONFERENCE

CUTANEOUS LEUKOCYTOCLASTIC VASCULITIS AND MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS WITH MONOCLONAL IGM GAMMOPATHY.

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 Marrow Biopsy were found.

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)

Haematology Value Unit WBC 7.32

x103/uL

RBC 4.22

x106/uL

Hgb 12.2

g/dL

Hct 36.6

%

MCV 86.8

fL

MCH 28.9

pg

MCHC 33.2

g/dL

PLT 239

x103/uL

Formula Value % Neutr 60.1

%

Lymph 27.1

%

Mono 7.2

%

Eosino 3.5

%

Baso 0,3

%

LUC 1.7

%

Viral Markers Value HCV Ab neg HCV-RNA quant. neg HBsAg neg Anti HBs neg

Clinical Challenge - Lab findings pre-Bio

(15.11.2009)

Clinical Challenge - Lab findings pre-Bio

(15.11.2009)

Renal Chemistry Value Unit sCr 1.45

mg/dL

BUN 33

mg/dL

Gly 104

mg/dL

Na 139

mEq/L

K 4.4

mEq/L

Cl 105

mEq/L

Ca 9.4

mg/dL

P 4.5

mg/dL

Osm 285

mOsm/L

Immunology Value Unit IgG 569

mg/dL

IgM 516

mg/dL

IgA 86

mg/dL

C3 76

ng/dL

C4 16.5

mg/dL

Cryocrit Neg Serum Light Chains K 173

mg/dL

Serum Light Chains L 108

mg/dL

Rheumatoid Factor Neg

Urinalysis Value Unit Glucose Neg

mg/dL

pH 6.0 Chet. Neg Proteins Neg

mg/dL

Bilirubin Neg

mg/dL

Hemoglobin Strong Urobilin 0.2

UE/dL

Nitrits Neg PS 1.017 Color Yellow Appearance Clear

Clinical Challenge - Lab findings pre-Bio

(15.11.2009)

Urine Sediment Val Unit Red cells +++ White Cells + Fungi

• Bacteria
• Cells
• Crystals
• Casts

Clinical Challenge

2009, Dicember: Bone Marrow Aspirate

A CD19+ polyclonal lymphocyte

infiltrate and a Plasmacell infiltration <1% was described.

Renal Biopsy Renal Biopsy

Clinical pattern of Clinical pattern of

• Rapidly Progressive

Rapidly Progressive Glomerulonephritis (RPGN) Glomerulonephritis (RPGN) Clinical Challenge