Thrombotic thrombocytopenic purpura (TTP) is a relatively rare multisystem disorder. It is a true medical emergency but poorly described in children. Diagnosis may be delayed or missed as children with TTP usually present with symptoms that are common to many other disease states. Although recent improvements in the understanding of the fundamental lesson in TTP have led to dramatic improvements in its diagnosis and treatment, mortality remains high even when appropriately managed.

Methods:

We presented 4 children with TTP that required Intensive Care over the last 12 months. Of these, one had congenital TTP, two idiopathic TTP and one had TTP following allogenic bone marrow transplantation.

Patients were admitted to the ICU because of fever, acute onset neurological symptoms pallor and thrombocytopenia. Additional features were acute renal failure and metabolic derangement with laboratory features of elevated lactate dehydrogenase and shistocytosis.

Mechanical ventilation was required in three cases. Specific treatment depended on the type of TTP with repeated transfusions of fresh frozen plasma in congenital TTP, fresh plasma and plasma exchange in idiopathic TTP and immunosuppressive therapy in post BMT patient.
The neurological symptoms disappeared in all patients and renal function normalized rapidly. However, 2 patients died, one within 24 hours of initiation of therapy. One patient relapsed thrice and required multiple sittings of plasmapheresis.

Conclusion: TTP is a relatively rare, but life-threatening disorder that may be encountered by ICU practitioners. Awareness, prompt diagnosis and aggressive treatment may help control the high mortality associated with this challenging disorder.

NCPCC 2005 - Conference Abstracts.Pediatric Oncall [serial online] 2006 [cited 15 May 2006(Supplement 5)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/
TTP_plasmapheresis.asp