Shulman-upshaw syndrome is a hereditary form of Thrombotic thrombocytopenic purpura (TTP), which is a rare blood condition marked by the formation of blood clots known as thrombi within the circulation. This condition results in the consumption of platelets therefore leading to low platelet counts.

There is still no specific diagnostic procedure to confirm the diagnosis of Shulman-upshaw syndrome. Diagnosis is often based on the symptoms and blood tests like blood count and blood film.

Removal, treatment and return of blood plasma components from blood circulation have been an optional treatment for Shulman-upshaw syndrome since the 1990s. This method is known as plasmapheresis.

In the initial stage, symptoms of Shulman-upshaw syndrome start with malaise, headache, fever and sometimes diarrhea. As the condition leads to a more advanced stage, clots begin to form within blood vessels leading to the consumption of the platelets. This will then result to noticeable bruises and in rare cases spontaneous bleeding may occur. The most common site of bleeding is from the gums or nose. Bruises will take the form of purpura; large bruises may also develop known as ecchymoses. Other indicative symptoms of Shulman-upshaw syndrome include: • Strange behavior, altered mental status, headache and even stroke • Renal failure • Fever • Anemia, jaundice and characteristic blood film It should also be noted that symptoms of Shulman-upshaw syndrome may circulate and fluctuate.

Until now, the real cause of Shulman-upshaw syndrome remains indefinable; recently however studies point to the inherited deficiency of ADAMTS13.

shulmanupshawsyndrome