Abstract
Abstract 4527
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.
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.
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.
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.
Transplant details and patient characteristics
| Patients No. . | Sex . | Age . | Disease . | Conditioning . | Donor . | TA-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. . | Sex . | Age . | Disease . | Conditioning . | Donor . | TA-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 |
TA-TMA manifestations and associated circumstance
| Patients No. . | Hb (g/L) . | PLT (*10E9/L) . | Schistocyte (%) . | LDH (U/L) . | Cr increased . | CNS abnormal . | Antiglibulin test . | Associated conditions . | Treatment . |
|---|---|---|---|---|---|---|---|---|---|
| 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 increased . | CNS abnormal . | Antiglibulin test . | Associated conditions . | Treatment . |
|---|---|---|---|---|---|---|---|---|---|
| 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 |
No relevant conflicts of interest to declare.
Author notes
Asterisk with author names denotes non-ASH members.
