PO13 - Complex management in a PWH and Guillain Barre Syndrome - Diagnostic and treatment challenges

Background: Several clinical manifestations are common to both hemophilia and other diseases. Further investigations are needed to identify the etiology in order to take the appropriate hemostatic decision for each particular case.

Aims: To highlight the importance of comprehensive care for patients with hemophilia.

Methods: A 5 years old boy with severe hemophilia B without inhibitors, without prophylactic treatment, came for bruises and subcutaneous hematomas, breathing difficulties, generalized muscles pain and weakness in the lower limbs. Ultimately, the patient needed intense lifesaving multidisciplinary care for a non-hematological pathology.

Results: On admission, the patient had fever, multiple bruises and subcutaneous hematomas on the lower limbs and buttocks, pallor, peripheral cyanosis, inspiratory dyspnea, gasping, inability to walk (onset 48 hours prior to admission). No vesicular breath sounds were audible on the right pulmonary area, with very low intensity ones present on the left side, and there was fast degradation of the heart rate to cardiac arrest (rapidly reversible). Brain CT scan excluded a bleeding. Progressive ascending symmetric weakness of the muscles and other investigations suggested Guillain Barre Syndrome. Kept under hemostatic treatment, he was admitted in the ICU where, for several months, he received multidisciplinary care: tracheal intubation, plasmapheresis, IGIV, tracheostomy by the ENT team, constant monitoring by a pediatrician, neurologist and medical rehabilitation. An intramedullary spinal cord biopsy, performed by a neurosurgeon, confirmed chronic myelitis. No hemorrhagic complications occurred, as adequate plasma levels of FIX were maintained during invasive procedures and for baseline care.

Conclusion(s): The management of hemophilia is not limited to prevention and treatment related directly to the complications of the disease, but includes provision of a proper coagulation status for other pathological situations. Therefore, a high quality of life for hemophilia patients cannot be ensured exclusively by a hematologist and requires lifelong care by a multidisciplinary team.