Plasma exchange for treatment of thrombotic thrombocytopenic purpura in critically ill patients.
OBJECTIVE
Description of diagnostic procedures, treatment modalities and intensive care management of patients with thrombotic thrombocytopenic purpura (TTP).


METHODS
Descriptive study.


METHODS
Internal medicine Intensive Care Unit (University Hospital of Vienna).


METHODS
Six patients (two after allogeneic bone marrow transplantation), treated for 12 episodes of TTP.


METHODS
Treatment with plasma exchange (fresh frozen plasma, 50-80 ml/kg per day), prednisone (0.75 mg/kg b.i.d.) and, in some cases, vincristine. Supportive therapy as needed.


RESULTS
Patients were admitted to the ICU because of neurological symptoms with acute onset (42% mild, 58% severe), hemolysis and thrombocytopenia. Additional symptoms were fever (50%), bleeding tendency (50%), acute renal failure (42%) and metabolic derangement (8%). Initial laboratory values showed thrombocytopenia (median 17 G/l), hemolysis (median hemoglobin 10.0 g/dl, lactate dehydrogenase 635 U/l, reticulocyte count 175 G/l) with red cell fragmentation. Coagulation tests were normal. Respiratory assist was needed in six episodes (severe seizures, cardiopulmonary resuscitation). In patients without preexisting hematological abnormality the platelet counts exceeded 100 G/l after 3-8 cycles of plasma exchange. In patients after bone marrow transplantation, the platelet counts never exceeded 40 G/l, but the lactate dehydrogenase levels dropped significantly. The neurological symptoms disappeared in all patients and renal function normalized. One patient died before the initiation of therapy. Three patients relapsed 1-3 times between 2 weeks and 5 months after the last episode. The relapses were associated with symptoms similar to the first episode and responded promptly to plasma therapy.


CONCLUSIONS
TTP is a rare, but life-threatening disorder. It needs immediate diagnosis and has a good prognosis after adequate treatment with plasma exchange.
Knöbl
P
P
Department of Medicine I, University of Vienna, Austria. Pknoebl@pan.at
Rintelen
C
C
Kornek
G
G
Wiltschke
C
C
Kalhs
P
P
Schwarzinger
I
I
Globits
S
S
Staudinger
T
T
Laczika
K
K
Kyrle
P
P
Frass
M
M
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D016638:Critical Illness; D005260:Female; D006801:Humans; D007362:Intensive Care Units; D008297:Male; D010951:Plasma Exchange; D011241:Prednisone / Q000627:therapeutic use; D011247:Pregnancy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D012074:Remission Induction; D012307:Risk Factors; D014750:Vincristine / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D014750:Vincristine; D011241:Prednisone