Thrombotic thrombocytopenic purpura

It is a rarer disease compared to ITP, but has a serious prognosis.
 * Fevers,
 * Hemolytic anemia (microangiopathic, schistocytes in blood smear),
 * Thrombocytopenia with bleeding manifestations,
 * Neurological symptomatology,
 * Renal impairment.

Idiopathic form

 * In the plasma, von Willebrand factor of high molecular weight (as a result of the deficiency of a specific plasma metalloproteinase) with the ability to induce platelet microthrombotization.

Hereditary form

 * Mutation of the metalloproteinase gene from the ADAMTS group on chromosome 9,
 * In the acquired forms, antibodies against metalloproteinases have been demonstrated,
 * In order to cause a TTP attack, the participation of other agents (infection, etc.) is necessary.
 * Histologically: multiple hyaline microthrombi in arterioles (central CNS + kidneys).

Secondary thrombotic thrombocytopenic purpura

 * In patients after allogeneic transplantation, in systemic diseases, generalized malignancies, after administration of certain drugs (quinine, ticlopidine), complications of intestinal infections.

Clinical course

 * severe, violent,
 * Patient at risk of ischemia + CNS hemorrhage + renal failure,
 * There may be extensive bleeding into the skin ,
 * Acute forms with relapses of thrombocytopenia / chronic forms.

Therapy

 * Recurrent forms – immunosuppressants (prednisone, cyclophosphamide), rituximab,
 * Idiopathic forms – based on administration of fresh frozen plasma + plasmapheresis ,
 * Secondary forms – therapy of the underlying disease, discontinuation of suspected drugs,
 * Hemolytic uremic syndrome (HUS) = organ limited form of TTP,
 * Epidemic form – childhood disease, preceded by enterocolitis ( E. coli ); hemolytic anemia, thrombocytopenia, renal impairment; th.: anti-infective + symptomatic th., or temporary hemodialysis,
 * Sporadická forma – intestinal symptomatology is absent, symptoms are more varied; th.: plasmapheresis.

Related articles

 * Thrombocytopenia
 * Thrombocytopenia of the new born
 * Hematological examination
 * Pre-transfusion examination
 * Examination of blood coagulation
 * Purpura