U05-16912

• 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

U05-16912

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

Lambda

Albumin

Electron Microscopy

Diagnosis

• Renal Amyloidosis associated with lambda light chain disease