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[Thrombotic thrombocytopenic purpura (TTP) or Moschowitz syndrome. Our experience]
G Iannandrea1, R Flocco, M Flocco
1Servisio di Anestesia e Rianimazione, Azienda Sanitaria Locale n. 3, Presidio Ospedaliero Cardarelli, Campobasso.
Aim:
The aim of this study was to report that Moschcowitz's syndrome was an inexorable cause of death in our Centre in the cases we observed, all of which failed to respond to the treatment used. This severe prognosis was attributed to the irreversible nature of the pathology when the patients were admitted to intensive care.
Methods:
Three cases are described which were brought to our attention with this pathology. The following association was present in all cases: low platelet count, anemia with schistocytosis and indirect hyperbilirubinemia. Plasmapheresis was the main treatment used in all cases.
Setting:
the intensive care unit of our Hospital is the regional reference centre where plasmapheresis is performed.
Patients:
Three patients were studied: two females aged 62 and 42 respectively, and one male aged 63. All three patients came from this region and no special features were found in their long-term medical history.
Interventions:
all patients underwent blood transfusion, plasmapheresis and received targeted antibiotic and corticosteroid treatment, and parenteral nutrition.
Results:
All three cases died.
Conclusions:
The analysis of these cases highlights the need for an early diagnosis, even if this is not easy, in order to commence successful therapy.
Insights
Moschcowitz's syndrome, a severe condition, proved fatal in all observed cases despite intensive care and treatments like plasmapheresis. Early diagnosis is crucial for potential successful therapy in this rare disorder.
Area of Science:
- Hematology
- Intensive Care Medicine
- Critical Care
Background:
- Moschcowitz's syndrome, a rare and severe thrombotic microangiopathy, presents a significant diagnostic and therapeutic challenge.
- This study examines the grim prognosis of Moschcowitz's syndrome within a regional intensive care setting.
Observation:
- Three critically ill patients with Moschcowitz's syndrome presented with a consistent triad: thrombocytopenia, anemia with schistocytosis, and indirect hyperbilirubinemia.
- Despite comprehensive treatment including plasmapheresis, blood transfusions, antibiotics, corticosteroids, and parenteral nutrition, all patients succumbed to the condition.
Findings:
- Moschcowitz's syndrome was an inexorable cause of death in the observed cases, with treatment failure attributed to the advanced, irreversible nature of the pathology upon ICU admission.
- The study underscores the uniformly fatal outcome in this cohort, irrespective of intensive care interventions.
Implications:
- The findings emphasize the critical need for early and accurate diagnosis of Moschcowitz's syndrome to enable timely and potentially life-saving interventions.
- This case series highlights the limitations of current therapeutic strategies when the disease is advanced, stressing the importance of prompt recognition and management.