582 3 GVHD TMA Anemia thrombocytopenia and proteinuria after blood stem cell transplantation ZHANG Jin FU Jia CHENG Zhen LIU Zhihong National Clinical Research Center of Kidney Diseases Jinling Hospital Nanjing University School of Medicine Nanjing 210016 China ABSTRACT A young female patient with severe aplastic anemia underwent allogeneic peripheral blood stem cell transplantation from a fully HLA-matched sibling. Three months later she suffered from hemolytic anemia thrombocytopenia pericardial effusion edema and proteinuria. Twice renal biopsies disclosed graft-versus-host disease GVHD associated thrombotic microangiopathy. Key words blood stem cell transplantation graft-versus-host disease thrombotic microangiopathy 20 30 mg /d + 2 mg /d 3 3 2012 6 2. 42 g /d 3 2009 1 MMF WBC 1. 3 10 9 /L Hb 50 g /L PLT 6 10 9 /L A CsA mmhg SCr 2 CsA CsA 258. 62 ~ 430. 9 ng /ml 4 WBC 3. 59 10 9 /L Hb 92 g /L PLT 52 10 9 /L 210016 g /L PLT 65 10 9 /L GVHD 60 ~ 80 mg /d + MMF 0. 5 g /d 50 mg /d 1 5 mg 2009-08-11 200 /120 Hb 76 g /L WBC 3. 2 10 9 /L PLT 76 10 9 / L 32% 1. 06 g /d 33. 3 g /L SCr 55. 7 μmol /L LDH 391 U /L 37. 1 μmol /L 31. 7 μmol /L 30 mg /d + 2009 5 CT TW 60 mg /d 50 μmol /L 2010 2 0. 55 g /24h AST 50 U /L WBC 2. 45 10 9 /L Hb 86 38. 5 g /L SCr 94. 6 μmol /L 4 SCr 135. 2 μnol /L TW 5 Hb 71 g /L SCr 183. 9 μmol /L
23 6 2014 12 583 GVHD TW 60 mg /d SCr 90. 2 μmol /L 0. 4 ~ 0. 7 g /d2011 1 TW SCr 12 TW2012 3 1. 55 g /d SCr 112. 3 μmol /L 2012 3 1 / 138 /93 mmhg CsA CsA 430. 9 ng /ml SCr 1. 55 g /d GVHD RB NAG 522 SCr 94% SCr mosm /kg H 2 O 1 Hb 85 g /L WBC 12. 1 10 9 /L PLT 227 10 9 /L 17% 34. 9 g / L 19. 7 g /L 17. 6 mg /dl SCr 80. 4 μmol /L 275 μmol /L C 1. 69 mg / L 6. 1 μmol /L 4. 5 μmol /L LDH 5. 2 mmol /L 1. 09 mmol /L 4. 68 mmol /L > 10 ANAds-DNA IgA 0. 418 g /L IgGIgMIgE O C3 0. 654 g /L C4 0. 221 g /L 33 /ml < 20 /ml vwf 238. 46% < 200% VCAM 13 ADAMTS13 ADAMTS13 H CD4 313 /μl CD8 931 /μl CD3 1 294 /μl CD20 216 /μl Treg 10 /μl B 112 mm 41 mm 56 mm105 mm 35 mm 52 mm 1 SCr GVHD 1 SCr 10 GVHD SCr LDH TMA 2010 5 2 3 GVHD 2A 3 1 ARB Ⅱ ACEI TW P
584 ~ 3 PASM-Masson C3 + + IgGIgAC1q 2D 21 ~ 43 nm 2B + + ~ + + + 2C IgM + 2 /6 2 A. PAS 400 B. Masson 400 C. PAS 400 D. EM 2012 3 28 11 3A + + ~ 3C 3D IgA + + B PASM-Masson IgM + + C3 + + Fibrin 1 C4d 3 A ~ PAS 400 B PAS 400 C PAS 200 D PAS 200 ~ 1 TMA 2 1 1
23 6 2014 12 585 2 3 GVHD TW 60 TMA 6 ~ 12 mg /d 2012 8 0. 55 MMF 0. 75 g /d 2012 12 MMF 2013 10 1. 34 g /d 94 g /L 20 TMA 0. 5% ~ 76% 60% ~ mg /d TW60 mg /d2014 6 0. 63 g /d SCr 99. 9 μmol /L Allo-HSCT 149 41 4 HSCT TMA TMA 12% 2. 4% 2 HSCT TMA 1 IgA TA-TMA 1 2 BMT-CTN 3 > 2 /HP LDH TMA 3 4 Coombs' TMA 6 2 IWG TMA < 50 10 9 /L 50% LDH Hb 4 TMA GVHD TMA LDH 1 TMA TA-TMA TMA TMA 2 GVHD g /d SCr 3 TW HUS / TTP 5 TA-TMA 90% 6 George 7 35 5 423 447 8. 2% TMA TA-TMA 8 > 4% PLT 9 SCr TMA TMA ~ TMA TMA TMA TMA TA-TMA 10 TMA TTP TA-TMA vwf TMA 3 GVHD ADAMTS13 ADAMTS13 3 GVHD TA-TMA TMA
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