Use of the flexible fiberoptic bronchoscope and similar devices has become among the preferred techniques in the management of such cases. However, these devices require a different skill set than that employed for conventional laryngoscopy and are expensive to purchase, maintain and repair.When taking the patients medical history, the subject is questioned about any significant signs or symptoms, such as difficulty in speaking or difficulty in breathing. These may suggest obstructing lesions in various locations within the upper airway, larynx, or tracheobronchial tree. A history of previous surgery (e.g., previous cervical fusion), injury, radiation therapy, or tumors involving the head, neck and upper chest can also provide clues to a potentially difficult intubation. Previous experiences with tracheal intubation, especially difficult intubation, intubation for prolonged duration (e.g., intensive care unit) or prior tracheotomy are also noted.A detailed physical examination of the airway is important, particularly:
the range of motion of the cervical spine: the subject should be able to tilt the head back and then forward so that the chin touches the chest. the range of motion of the jaw (the temporomandibular joint): three of the subjects fingers should be able to fit between the upper and lower incisors. the size and shape of the upper jaw and lower jaw, looking especially for problems such as maxillary hypoplasia (an underdeveloped upper jaw), micrognathia (an abnormally small jaw), or retrognathia (misalignment of the upper and lower jaw). the thyromental distance: three of the subjects fingers should be able to fit between the Adams apple and the chin.
Although the majority of cases initially respond well to treatment, relapses are not uncommon and immunosuppressive drugs (e.g. ciclosporin, mycophenolate mofetil, vincristine and danazol) are subsequently used, or combinations of these.The off-label use of rituximab (trade name Rituxan) has produced some good results in acute and refractory cases, although further relapse may occur within a year. Splenectomy is effective in some cases, but relapses are not uncommon.The only prospect for a permanent cure is the high-risk option of an allogeneic hematopoietic stem cell transplantation (SCT). Prognosis
In a nationwide study of Evans syndrome the median survival was 7.2 years (primary Evans syndrome: 10.9 years; secondary Evans syndrome: 1.7 years). Secondary Evans syndrome was associated with higher mortality rate than primary Evans syndrome, with a 5-year survival of 38%. Among patients with Evans syndrome, the prevailing causes of death were bleeding, infections, and hematological cancer.It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia, in one cohort 58% of children with Evans syndrome had CD4-/CD8- T cells which is a strong predictor for having autoimmune lymphoproliferative syndrome. Epidemiology
Evans syndrome is considered a very rare autoimmune disease. Only one study has estimated incidence and prevalence adults. In Denmark in 2016 the annual incidence was 1.8 per 1,000,000 person years, and the prevalence was 21.3 per 1,000,000 living persons. In pre-pubertal children the incidence has been estimated to be between  0.7 and 1.2 per 1,000,000 person-years. See also
Hematology

References


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