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

2. CASE 1: 2309744 CASE 2: 20697415

3. CASE 1: 2309744

4. General Data Age: 57-year-old Gender: male Ethnic: Taiwanese Marital status: Married Occupation: 工人 Admission date: 2011/10/25

5. Chief Complaint Accidental finding of proteinuria in health exam

6. Present illness This 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.

7. Present illness Biochemistry disclosed hypoalbuminemia and PEP/IFE turned out to be IgA lambda category. For nephrotic syndrome, he was admitted for kidney biopsy.

8. Past History No admission history No history of major operation Hyperlipidemia known for about 4 years, without medication control

9. Personal History No known allergy to drug or food He denies smoking, alcohol, or betel nut chewing.

10. Physical Examination BT 35.8 ℃ PR: 98/min, RR: 16/min, BP: 107/73 mmHg General appearance: fair Consciousness: alert and oriented HEENT: conjunctiva: pink, anicteric sclera Chest: symmetrical chest expansion, bilateral clear breathing sounds. Heart: regular heart beats, no murmurs. Abdomen: soft and flat normal bowel sounds Extremity: no pitting edema.

11. Laboratory Findings Hemogramunit9/28 WBC/uL5900 RBCmillion/uL4.79 Hemoglobing/dL13.4 Hematocrit%41.2 MCVfL86.0 MCHpg/cell28.0 MCHCg/dL32.5 RDW%14.2 Platelets/uL294k Segment%62.4 Lymphocyte%28.7 Monocyte%5.9 Eosinophil%2.5 Basophil%0.5

12. BiochemistryUnit9/28 Crmg/dL1.09 BUNmg/dL18.4 NamEq/L145 KmEq/L4.6 Camg/dL8.8 Pmg/dL3.6 Albuming/dL2.89 Total proteing/dL4.8 Sugar ACmg/dL100 Triglyceridemg/dL160 Total Cholemg/dL367 HBsAgNegative Anti HBs AbNegative Anti HCV AbNegative Urinalysis9/28 ColorYellow TurbidityClear Sp. Gravity1.003 pH6.5 LeukocyteNegative NitriteNegative Protein2+ GlucoseNegative KetoneNegative Urobilinogen0.1 BilirubinNegative Bloodtrace RBC4 WBC0 Epi.0

13. 10/06 IgGmg/dL364 IgAmg/dL226 IgMmg/dL60.5 IgEIU/mL<16.40 RPRNegative ASLOIU/mL<51.60 C3mg/dL104.0 C4mg/dL25.80 ANANegative 10/13 PEP/IFEParaprotein of IgA lambda category B2 microglobulin3293 ug/L Alb/Cre ratio4052 mg/g MicroALB(U)1785 mg/L Creatinine(U)44.05

14. CXR CXR

15. 2011/10/28 Kidney Echo

16. Left 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.

17. 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.

18. 2011/11/02 Kidney Biopsy DX: AMYLOIDOSIS MICRO 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

19. Diagnosis Nephrotic syndrome, etiology: amyloidosis

20. Discussion

21. CASE 2: 20697415

22. General Data Age: 17-year-old Gender: female Ethnic: Taiwanese Marital status: single Occupation: student Admission date: 2011/09/25

23. Chief Complaint For renal transplantation

24. Present Illness This 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.

25. Past History Hypertension noticed for 2 years, on Valsartan, Carvedilol and Imdur Seizure history No history of major operation

26. Personal History No known allergy to drugs Smoking: denied Alcohol: denied Betel nuts chewing: denied

27. Physical Examination BT 37.1 ℃ PR: 91/min, RR: 18/min, BP: 153/98mmHg BH: 164cm, BW: 45.2kg BMI: 16.8 General appearance: fair Consciousness: alert and oriented HEENT: pale conjunctiva, anicteric sclera Chest: symmetrical chest expansion, bilateral clear breathing sounds Heart: regular heart beats, no murmurs. Abdomen: soft, no tenderness Extremity: no pitting edema.

28. Hemogramunit9/159/28 WBC/uL6500 RBCmillion/uL3.50 Hemoglobing/dL4.99.8 Hematocrit%14.529.4 MCVfL84.0 MCHpg/cell28.0 MCHCg/dL33.3 RDW%15.2 Platelets/uL95 Segment%80.0 Lymphocyte%18.0 Monocyte%2.0 Eosinophil%0 Basophil%0

29. BiochemistryUnit9/1510/01 BUNmg/dL67.155 Crmg/dL5.03 NamEq/L139125 KmEq/L3.22.6 Camg/dL8.16.5 Pmg/dL5.2 Albuming/dL2.71 C3mg/dL59.6 C4mg/dL22.8 ANA1: 640 Anti-DsDNAUnit/ mL 443

30. Urinalysis Color Turbidity Sp. Gravity pH Leukocyte Nitrite Protein Glucose Ketone Urobilinogen Bilirubin Blood Bacteria/Yeast RBC WBC Epi.

31. Serology6/10 C3135 C428.2 ANANegative Anti-dsDNA<40.5 (6/27) Anti-SmithNegative RNPNegative SSA/SSBNegative Anti-cardiolipinNegative CryoglobulinIgG2+, IgA+, IgM+ CryofibrinogenPositive ASLO258 IU/ml Serology6/10 IgG717 IgA289 IgM102 IgE<16.9 ANCANegative Anti-HCVNegative Anti-HIVNegative RPRNegative

32. CXR

33. Operation on 09/30 失血量 :100cc donor's left kidney to recipient right iliac fossa adhesion of retroperitoneal space due to CAPD tube placement external iliac artery caliber good, patency good external iliac vein caliber good but catheter inside small bladder capacity renal artery to external iliac artery, end to side renal vein to external iliac vein, end to side ureter to bladder, extravesical procedure, stent with 6x26 D-J initial perfusion good initial urine good warm ischemic time 25min. cold ischemic time 2hrs

34. Course and treatment 9/25 Admission 9/30 Transplant 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 10/05 Cr 3.8 1370ml 10/06 Cr 4.15 430ml 10/07 Cr 4.77 335ml 10/05 MP pulse therapy 10/07 Rituximab 10/10 Cr 6.08 1180ml 10/12 Kidney Bx 10/07 10/11 Kidney echo 10/15 10/16 10/17 Plasma exchange 10/20 Cr 4.82 860ml 10/23 MBD

35. 2011/10/07 Kidney Echo

37. Pathology of Kidney Biopsy Thrombotic 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.

38. Pathology of Kidney Biopsy TUBULES 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

39. Diagnosis End 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

40. Discussion