Suspect Moschcowitz syndrome: how, when, why
1. Department of Medical, Surgical, Neurological, Metabolic and Geriatric Sciences, University of Campania “Luigi Vanvitelli”, Naples, Italy
2. Internal Medicine, Clinical Hospital of Marcianise, ASL Caserta, Caserta, Italy
Thrombotic Thrombocytopenic Purpura, microangiopathic haemolytic anaemia, thrombotic events, multiorgan damage, plasmapheresis.
Thrombotic Thrombocytopenic Purpura (TTP) is an acute syndrome characterized by the presence of microangiopathic haemolytic anaemia and thrombocytopenia.
The first case of TTP was described by Moschowitz in 1924 . Recently, between 1982 and 2001, several authors [2-5] identified in the deficiency of the metalloproteinase ADAMTS13 the primary pathogenic cause. TTP is a rare disease [6-8]. it occurs between 30 and 50 years of age, more frequently in the female sex. In the absence of treatment, mortality exceeds 90%, while it is reduced to 10-20% after adequate plasma plasmapheresis or infusion therapy. However, half the deaths are attributable to complications associated with plasmapheresis and hospitalization (sepsis, haemorrhages, thrombosis, etc.) 
A 36-year-old italian woman came to our observation in the emergency department for abdominal pain, menometrorrhagia and vomiting. The patient had no significant past medical history. She had no significant clinical changes. She was hemodynamically stable.
Our case report demonstrates the importance of the rapidity in setting the diagnosis and the treatment of PTT.
Therapy greatly reduces patient mortality. So far, the treatment performed by more physicians is not valid and nonconforming to the guidelines. Platelet transfusions are still administered by many increasing the risk of precipitating further thrombotic events. Their use is contra-indicated in TTP, unless there is life-threatening haemorrhage.
The symptomatology is often nonspecific and the diagnosis is posed with the presence of both microangiopathic hemolytic anaemia and thrombocytopenia. Multi-organ involvement, with the onset of acute pancreatitis, may be present.
However, symptoms demonstrating a multi-organ pathological involvement may be an expression of iatrogenic damage.
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