Abstract 4527

Background:

Transplantation-associated thrombotic microangiopathy (TA-TMA) is lifethrewten though rare complication of allogeneic hematopoietic stem cell transplantation (HSCT). Diagnosis of TA-TMA is difficult due to its variation of criteria. Recently two new diagnositic criteria for TA-TMA have recently been proposed: the Bone Marrow Transplant Clinical Trials Network (BMTCTN) and the International Working Group (IWG) criteria. Our purpose is to investigate the indence and risk facter of TA-TMA in our institute.

Study design and methods:

All 8 cases of TA-TMA previously diagnosed at our institution between October 2005 and August 2010 were retrospectively evaluated and analysised. Table I showed the transplant details and patients characteristics.

Results:

Six patients performed Matched Unrelated Donor HSCT, two were haploidentical donor HSCT. Four patients complicated with hypohepatia, but only three patients had renal insufficient. The incidence of central nervous symptom abnormalities or dysfunction was very high (sever of eight patients). Four of eight patients were CSA linked TA-TMA, withdrawing CSA resulted in complete response. The other four patients were no CSA linked TA-TMA and developed aGVHD or CMV infection before TA-TMA. They had badly response to common treatment including plasma exchange (PE), steroid treatment and immunosuppression decreased. All of them died of multiple organ failure. Table II showed TA-TMA manifestations and associated circumstance of the patient.

Conclusion:

Our experience suggests that CSA linked TA-TMA is totally different from no CSA linked TA-TMA. The former had good response and prognosis, the later were always associated with GVHD, hypohepatia and virus infectious. The mortality of no CSA linked TA-TMA is high, they had poor prognosis and badly response.

Table I:

Transplant details and patient characteristics

Patients No.SexAgeDiseaseConditioningDonorTA-TMA occurrence (days after SCT)Follow-up and Outcome (days after SCT)
1 M 48 ALL BUCY2 MUD (HLA-B1, A->B) 97 144 died of TA-TMA 
2 F 28 ALL BUCY2 MUD (Matched, O->O) 47 65 died of TA-TMA 
3 M 42 ALL BUCY2+ATG MUD (HLA-B, AB->O) 38 51 died of TA-TMA 
4 M 12 ALL BUCY2+Ara-C+ATG Haploidentical (Mom->Son, O->O) 33 Survival>270 
5 M 17 SAA CTX+ATG MUD (HLA-CW, B->O) 25 Survival >210 
6 F 33 MDS BUCY2 MUD (Matched, AB->O) 71 Survival >1157 
7 F 21 ALL BUCY2+Ara-C+ATG Haploidentical (Dad->daughter, A->O) 31 Survival TA-TMA, but died of infection at 180+ 
8 F 43 ALL BUCY2 MUD (Matched, A->O) 63 66 died of TA-TMA 
Patients No.SexAgeDiseaseConditioningDonorTA-TMA occurrence (days after SCT)Follow-up and Outcome (days after SCT)
1 M 48 ALL BUCY2 MUD (HLA-B1, A->B) 97 144 died of TA-TMA 
2 F 28 ALL BUCY2 MUD (Matched, O->O) 47 65 died of TA-TMA 
3 M 42 ALL BUCY2+ATG MUD (HLA-B, AB->O) 38 51 died of TA-TMA 
4 M 12 ALL BUCY2+Ara-C+ATG Haploidentical (Mom->Son, O->O) 33 Survival>270 
5 M 17 SAA CTX+ATG MUD (HLA-CW, B->O) 25 Survival >210 
6 F 33 MDS BUCY2 MUD (Matched, AB->O) 71 Survival >1157 
7 F 21 ALL BUCY2+Ara-C+ATG Haploidentical (Dad->daughter, A->O) 31 Survival TA-TMA, but died of infection at 180+ 
8 F 43 ALL BUCY2 MUD (Matched, A->O) 63 66 died of TA-TMA 
View Large
Table II:

TA-TMA manifestations and associated circumstance

Patients No.Hb (g/L)PLT (*10E9/L)Schistocyte (%)LDH (U/L)Cr increasedCNS abnormalAntiglibulin testAssociated conditionsTreatment
1 50 <20 11 962 >2 fold increased No Neg CMV, AGVHD, III, PE*9 times, steroid 
2 59 <20 6 1243 >2 fold increased Yes Neg AGVHD, IV PE*4, steroid, Defibrotide Etanercept 
3 86 35 11 481 No Yes Neg AGVHD, II; Hypohepatia PE*2, steroid, Defibrotide 
4 90 79 3 369 No Yes Neg CSA associated PE*2, steroid Anti-CD25, withdraw CSA and recovery 
5 96 40 5 548 No Yes Neg CSA associated withdraw CSA and recovery 
6 89 55 3 340 No Yes Neg CSA associated withdraw CSA and recovery 
7 51 <20 3 398 No Yes Neg AGVHD, II; CMV steroid, withdraw CSA and recovery 
8 61 <20 7 1838 >2 fold increased Yes Neg AGVHD, IV; CMV; Invasive fungal infection CSA associated, steroid 
Patients No.Hb (g/L)PLT (*10E9/L)Schistocyte (%)LDH (U/L)Cr increasedCNS abnormalAntiglibulin testAssociated conditionsTreatment
1 50 <20 11 962 >2 fold increased No Neg CMV, AGVHD, III, PE*9 times, steroid 
2 59 <20 6 1243 >2 fold increased Yes Neg AGVHD, IV PE*4, steroid, Defibrotide Etanercept 
3 86 35 11 481 No Yes Neg AGVHD, II; Hypohepatia PE*2, steroid, Defibrotide 
4 90 79 3 369 No Yes Neg CSA associated PE*2, steroid Anti-CD25, withdraw CSA and recovery 
5 96 40 5 548 No Yes Neg CSA associated withdraw CSA and recovery 
6 89 55 3 340 No Yes Neg CSA associated withdraw CSA and recovery 
7 51 <20 3 398 No Yes Neg AGVHD, II; CMV steroid, withdraw CSA and recovery 
8 61 <20 7 1838 >2 fold increased Yes Neg AGVHD, IV; CMV; Invasive fungal infection CSA associated, steroid 
View Large
Disclosures:

No relevant conflicts of interest to declare.

Topics:
thrombotic microangiopathies, transplantation, tissue microarray, trimethylamine, steroids, hematopoietic stem cell transplantation, human leukocyte antigens, allogeneic hematopoietic stem cell transplant, antigens, cd25, bone marrow transplantation

Author notes

*

Asterisk with author names denotes non-ASH members.

© 2010 by The American Society of Hematology
2010
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