Pathology Conference

Presented by F1 林立原Commented by Dr.薛綏2011/11/23

CASE 1: 2309744CASE 2: 20697415

CASE 1: 2309744

General DataAge: 57-year-oldGender: maleEthnic: TaiwaneseMarital status: MarriedOccupation: 工人Admission date: 2011/10/25

Chief ComplaintAccidental finding of proteinuria in health exam

Present illnessThis 57 year-old male with

hyperlipidemia for 4 years, which was noticed in an health exam, without medication or life style modification. One month prior to this admission, proteinuria was noticed in health exam. Thus, he visited Nephro OPD for help. Foamy urine with intermittent swollen legs for 1 year was reported.

Present illnessBiochemistry disclosed

hypoalbuminemia and PEP/IFE turned out to be IgA lambda category.

For nephrotic syndrome, he was admitted for kidney biopsy.

Past HistoryNo admission historyNo history of major operationHyperlipidemia known for about

4 years, without medication control

Personal HistoryNo known allergy to drug or foodHe denies smoking, alcohol, or

betel nut chewing.

Physical ExaminationBT 35.8℃ PR: 98/min, RR: 16/min, BP:

107/73 mmHgGeneral appearance: fairConsciousness: alert and orientedHEENT: conjunctiva: pink, anicteric scleraChest: symmetrical chest expansion,

bilateral clear breathing sounds.Heart: regular heart beats, no murmurs.Abdomen: soft and flat normal bowel

soundsExtremity: no pitting edema.

Laboratory FindingsHemogram

unit 9/28

WBC /uL 5900RBC million/

uL4.79

Hemoglobin

g/dL 13.4

Hematocrit % 41.2MCV fL 86.0MCH pg/cell 28.0MCHC g/dL 32.5RDW % 14.2Platelets /uL 294kSegment % 62.4Lymphocyte

% 28.7

Monocyte % 5.9Eosinophil % 2.5Basophil % 0.5

Biochemistry

Unit 9/28

Cr mg/dL 1.09BUN mg/dL 18.4Na mEq/L 145K mEq/L 4.6Ca mg/dL 8.8P mg/dL 3.6Albumin g/dL 2.89Total protein g/dL 4.8Sugar AC mg/dL 100Triglyceride mg/dL 160Total Chole mg/dL 367HBsAg NegativeAnti HBs Ab NegativeAnti HCV Ab Negative

Urinalysis 9/28Color YellowTurbidity ClearSp. Gravity 1.003pH 6.5Leukocyte NegativeNitrite NegativeProtein 2+Glucose NegativeKetone NegativeUrobilinogen 0.1Bilirubin NegativeBlood traceRBC 4WBC 0Epi. 0

10/06IgG mg/dL 364 IgA mg/dL 226IgM mg/dL 60.5IgE IU/mL <16.40RPR NegativeASLO IU/mL <51.60C3 mg/dL 104.0C4 mg/dL 25.80ANA Negative

10/13PEP/IFE Paraprotein of IgA lambda

categoryB2 microglobulin

3293 ug/L

Alb/Cre ratio 4052 mg/gMicroALB(U) 1785 mg/LCreatinine(U) 44.05

CXR

2011/10/28 Kidney Echo

2011/10/28 Kidney EchoLeft Kidney Length: 9.5 cm Right Kidney Length: 9.7 cm Both kidneys are normal in size with

mildly irregular contour. The cortical echogenicity is increased with adequate thickness. The pelvocalyceal systems are not dilated. No obvious evidence of renal stone, mass or cyst is noted.

Impression: Parenchymal renal disease.

Bone marrow bx on 10/27 DX: BONE MARROW, RIGHT ILIAC, NEEDLE BIOPSY ----NO INCREASE OF PLASMA CELLS ----HYPOCELLULARITY

MICRO D: SECTIONS SHOW 15-25% CELLULARITY. THERE

ARE ONLY RARE PLASMA CELLS, WHICH ACCOUNT FOR LESS THAN 5% OF ALL NUCLEATED CELLS. THE M/E RATIO IS ABOUT 1/1. MEGAKARYOCYTES ARE FOUND ABOUT 0-3/HPF. NO INCREASE OF BLASTS IS NOTED. THERE ARE NO GRANULOMAS, NOR FOREIGN MALIGNANT CELLS. RETICULIN CONTENT IS NORMAL. STAINABLE IRON CONTENT IS NORMAL.

2011/11/02 Kidney BiopsyDX: AMYLOIDOSISMICRO D: H AND E SECTIONS SHOW 13 GLOMERULI.

THREE ARE OBSOLETE. OTHERS HAVE MODERATE TO MARKED AMORPHOUS DEPOSITS IN THE WALL OF GLOMERULAR CAPILLARIES. THE TUBULES HAVE ATROPHY WITH PROTEIN CASTS. THE INTERSTITIUM SHOWS MILD TO MODERATE FIBROSIS AND PROBABLE DEPOSITS. THE ARTERIOLES ALSO HAVE DEPOSITS IN THE WALL. IMMUNOFLUORESCENCE SECTIONS SHOW 9 GLOMERULI WITH IRREGULAR STAINING OF IGM(1+).

THE CONGO RED STAIN IS POSITIVE

DiagnosisNephrotic syndrome, etiology:

amyloidosis

Discussion

CASE 2: 20697415

General DataAge: 17-year-oldGender: femaleEthnic: TaiwaneseMarital status: singleOccupation: studentAdmission date: 2011/09/25

Chief ComplaintFor renal transplantation

Present IllnessThis 17 years female has

systemic lupus erythematosus since 2004/02 and lupus nephritis WHO class IV since 2004/04, and end-stage-renal disease on CAPD since 2009/11. She was admitted for renal transplantation.

Past HistoryHypertension noticed for 2 years,

on Valsartan, Carvedilol and Imdur

Seizure historyNo history of major operation

Personal HistoryNo known allergy to drugsSmoking: deniedAlcohol: deniedBetel nuts chewing: denied

Physical ExaminationBT 37.1℃ PR: 91/min, RR: 18/min, BP:

153/98mmHgBH: 164cm, BW: 45.2kg BMI: 16.8General appearance: fairConsciousness: alert and orientedHEENT: pale conjunctiva, anicteric

scleraChest: symmetrical chest expansion,

bilateral clear breathing soundsHeart: regular heart beats, no murmurs.Abdomen: soft, no tenderness Extremity: no pitting edema.

Hemogram unit 9/15 9/28WBC /uL 6500RBC million/

uL3.50

Hemoglobin g/dL 4.9 9.8Hematocrit % 14.5 29.4MCV fL 84.0MCH pg/cell 28.0MCHC g/dL 33.3RDW % 15.2Platelets /uL 95Segment % 80.0Lymphocyte % 18.0Monocyte % 2.0Eosinophil % 0Basophil % 0

Biochemistry

Unit 9/15 10/01

BUN mg/dL

67.1 55

Cr mg/dL

5.03

Na mEq/L

139 125

K mEq/L

3.2 2.6

Ca mg/dL

8.1 6.5

P mg/dL

5.2

Albumin g/dL 2.71

C3 mg/dL

59.6

C4 mg/dL

22.8

ANA 1: 640Anti-DsDNA Unit/

mL443

CXR

Course and treatment

9/25 Admission

9/30Transplant

9/27 9/29 DFPP

10/02 DFPP

10/01 Cr 5.03

10/02 Cr 3.07 5600ml

10/03 Cr 2.82 4400ml

10/05 10/06 10/07 10/11 10/13 DFPP

10/05Cr 3.8 1370ml

10/06Cr 4.15 430ml

10/07Cr 4.77 335ml

10/05MP pulse therapy10/07

Rituximab

10/10 Cr 6.081180ml

10/12Kidney Bx

10/07 10/11 Kidney echo

10/15 10/16 10/17 Plasma exchange

10/20 Cr 4.82860ml

10/23 MBD

Pathology of Kidney BiopsyThrombotic microangiopathy;---

Calcineurin inhibitor toxicity

H & E SECTIONS HAVE 6 GLOMERULI. 2 HAVE FOCAL THROMBI WITH FIBRIOID NECROSIS. OTHERS HAVE LOOP COLLAPSE AND MILD TO MODERATE SCLEROSIS. THE INTERSTITIUM HAS CONGESTION, HEMORRHAGE AND MILD FIBROSIS. SCATTERED INFILTRATION OF NEUTROPHILS AND LYMPHOCYTES ARE SEEN.

Pathology of Kidney BiopsyTUBULES HAVE PROTEINANEOUS AND

CELLULAR CASTS, TUBULAR CELL SHOW ISOMETRIC VACUOLIZATION, FOCAL NECROSIS AND REGENERATION, WITH MINIMAL TUBULITIS. ARTERIOLES HAVE FOCAL THROMBI.

IMMUNOFLUORESCENCE SECTIONS HAVE 7 GLOMERULI WITH FOCAL NECROSIS AND THROMBI AND STAIN WITH 2-3+ FIBRINOGEN AND 1-2+C1Q.

C4d AND B-K VIRAL STAINS ARE BOTH NEGATIVE

DiagnosisEnd stage renal disease, on

peritoneal dialysis since 2009/11, post living donar kidney transplant on 2011/9/30

Post kideny transplant renal function deterioration due to Calcineurin inhibitor related thrombotic microangiopathy

Systemic lupus erythematosus, with lupus nephritis

Discussion