Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome following allogeneic HPC transplantation: a diagnostic dilemma

BACKGROUND: Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) has been described as a specific sequela of allogeneic HPC transplantation (HPCT). Nevertheless, because multiple transplant-related sequela can cause the characteristic clinical features of TTP-HUS, the diagnosis is difficult. STUDY DESIGN AND METHODS: All English-language articles describing patients with TTP-HUS following HPCT were identified. Articles reporting five or more total patients, including at least one patient diagnosed with TTP-HUS following allogeneic HPCT, were reviewed. All articles describing autopsies of patients diagnosed with TTP-HUS following allogeneic HPCT were also reviewed. RESULTS: Thirty-five articles reporting 5 or more total patients described 447 patients diagnosed with TTP-HUS following allogeneic HPCT. The frequency of diagnosis of TTP-HUS following allogeneic HPCT varied by 125-fold (0.5%-63.6%). Twenty-eight different sets of diagnostic criteria were described in the 35 articles; 25 articles included both RBC fragmentation and increased serum LDH. Many risk factors described as correlating with the diagnosis of TTP-HUS also predict greater risk for multiple transplant-related complications. Benefit of plasma exchange treatment could not be documented. Survival information was reported for 379 patients, 232 (61%) died, and reported mortality rates varied from 0 to 100 percent. Autopsies have been reported for 35 patients who were diagnosed with TTP-HUS following allogeneic HPCT; none had systemic thrombotic microangiopathy, the diagnostic abnormality of TTP-HUS; and infection (19 patients) was the most commonly reported cause of death. CONCLUSIONS: The clinical features of TTP-HUS following allogeneic HPCT may be caused by common transplant-related complications; the benefit from plasma exchange treatment is uncertain.