Fatal thrombotic thrombocytopenic purpura as a rare complication following allogeneic stem cell transplantation

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare disease which, together with hemolytic uremic syndrome, is subsumed under thrombotic microangiopathy. After stem cell transplantation (SCT), this syndrome represents a possibly fatal complication with a higher incidence in allogeneic SCT than in autologous SCT. Although plasmapheresis offers an encouraging treatment modality in classic TTP, this seems less effective in bone marrow transplant-associated microangiopathy. This is probably due to a different etiology. We present a case of transplant-associated TTP with a fatal outcome despite multiple courses of plasmapheresis.

Thrombotic microangiopathy in blood and marrow transplant patients receiving tacrolimus or cyclosporine A

“…While this demonstrates the safety and feasibility of prolonged plasmapheresis, the overall poor results suggest low efficacy for this procedure in patients with TMA that is clearly associated with CSA or FK‐506. This is in agreement with the findings in larger studies, such as those by Chemnitz et al, 35 Fuge et al, 19 Iacopino et al, 3 Roy et al, 29 and Teruya et al, 36 although other investigators, mainly in case reports, 37,38 have documented remissions.…”

Transplant-associated microangiopathy (TAM) in recipients of allogeneic hematopoietic stem cell transplants

“…It is known that immune-mediated hemolysis may sometimes result in RBC fragmentation. Total body irradiation (TBI) has been previously associated with TAM 13,32,[34][35][36] This as well as other studies 3,5,8 could not confirm an impact of TBI.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Thrombotic microangiopathy is characterized by systemic or intrarenal platelet aggregation and a microangiopathic hemolytic anemia, with red blood cell (RBC) fragmentation and a negative direct antiglobulin test. Increased platelet consumption leads to thrombocytopenia.…”

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

“…Complete postmortem examinations were reported for 35 patients, 24 from the articles reporting 5 or more patients that were included in our systematic review 10,21 , 27‐34 and 11 from articles reporting fewer than 5 patients 35‐44 . Most autopsy descriptions were very brief, only one or two sentences per patient for 18 of the 35 patients.…”