RESUMO
Congenital cyclic neutropenia syndrome is a constitutional genetic disorder which is characterized by very low number of neutrophils (neutropenia). Patients suffering from this disorder clinically present with neutropenia at early age, history of recurrent fever, ulcerations in the oral cavity, gingivitis, and other recurrent infections. This paper describes a case report of a child with recurrent mouth ulcers, fever, and later diagnosed with severe congenital cyclic neutropenia. This also emphasizes the importance of identification of rare causes of immunosuppressive conditions in children presenting with recurrent oral ulcers and poor dental hygiene, to prevent long-term complications of oral cavity and also morbidity and mortality secondary to neutropenic sepsis.
Assuntos
Hemofilia A/complicações , Hemorragia/etiologia , Enfisema Pulmonar/complicações , Idoso , Vesícula/complicações , Vesícula/diagnóstico por imagem , Hemoptise/etiologia , Hemorragia/diagnóstico por imagem , Humanos , Masculino , Enfisema Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
An elderly woman presented with extensive bruising and a haemorrhagic stroke. Initial investigations revealed an abnormal clotting screen with a prolonged activated partial thromboplastin time. Further investigations revealed this to be due to antibodies that the patient had developed against clotting factor VIII also known as acquired haemophilia A.