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Pre-Chiasmatic, Single Injection of Autologous Blood to Induce Experimental Subarachnoid Hemorrhage in a Rat Model
Published on: June 18, 2021
Central nervous system bleeding in patients with rare bleeding disorders
S M Siboni1, E Zanon, G Sottilotta
1Centro Emofilia e Trombosi A Bianchi Bonomi, Dipartimento di Medicina Interna e Specialità Mediche, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico di Milano, Università degli Studi di Milano, Milano, Italy. simona.siboni@unimi.it
Summary
Central nervous system (CNS) bleeding is a serious complication in rare bleeding disorders (RBDs). Recurrence is frequent, highlighting the need for early prophylactic treatment in some patients.
Area of Science:
- Hematology
- Neurology
- Rare Diseases
Background:
- Central nervous system (CNS) bleeding is a severe complication in patients with rare bleeding disorders (RBDs).
- Understanding the epidemiology and outcomes of CNS bleeding in RBDs is crucial for patient management.
Purpose of the Study:
- To retrospectively analyze the incidence, recurrence, mortality, and neurological sequelae of CNS bleeding in patients with RBDs.
- To evaluate the efficacy of treatments, including replacement therapy and surgery, for CNS bleeding in RBDs.
- To identify risk factors and optimal management strategies for CNS bleeding in RBDs.
Main Methods:
- Retrospective data collection on patients with RBDs experiencing CNS bleeding.
- Analysis of demographic data, bleeding characteristics, treatment interventions, and patient outcomes.
- Categorization of bleeding location, type, and onset, alongside assessment of recurrence and neurological sequelae.
Main Results:
- 36 CNS bleeding episodes were analyzed in 24 patients with severe deficiencies in Factors V, X, VII, or XIII.
- Recurrence of CNS bleeding occurred in 25% of patients, with 29% experiencing early onset before age two.
- Intracerebral bleeding was the most common type (53%), with no recorded deaths; 71% of patients received secondary prophylaxis.
Conclusions:
- Recurrence of CNS bleeding is relatively common in severe Factor V, X, VII, and XIII deficiencies.
- Replacement therapy is the primary treatment, with surgery reserved for severe cases; early prophylaxis may be necessary for certain RBDs.
- Further research is needed to optimize prophylactic treatment strategies, including dosage and frequency, for CNS bleeding in RBDs.
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