U

52 y old female with PMH of mild RA, increased LFT, asthma, atypical chest pain, depression Presented late 2004 with chronic abdo pain, had laparoscopic fibroid removal and R salpingoophorectomy Feb 2005 Jan 2005: Serum albumin 38, creatinine 83 Prior dipsticks with intermittent blood and protein Few weeks after d/c felt very SOB and attributed it to her asthma Presented with SOB and volume overload in June 2005, found to have bilateral PE, started on coumadin, no work up done July 2005: U dipstick 3+ protein, creatinine 91, albumin 21, 24h Urine 8g prot Repeated abdo US showed normal kidneys, abnormal spleen ? Infiltration vs. infarct 24h urine form ER: Bence Jones +, light chains serologies negative, SPEP negative, hct 0.41 August renal clinic: SBP in 90s, mild volume o/l, no rales, no heave, no murmurs, JVP not high, minimal joint deformities in feet Arranged heme consult and MRV MRV negative, spleen enlarged U

Biopsy

Immunofluorescence IgG – Negative IgA – Negative IgM – Mild to moderate mesangial staining C3 – Negative C1q – Negative Kappa – Negative Lambda – Moderate mesangial staining. Moderate vascular staining Fibrinogen – Negative Albumin – Moderate hyaline droplet change in tubular cytoplasm

IgM

Lambda

Albumin

Electron Microscopy

Diagnosis Renal Amyloidosis associated with lambda light chain disease