: 60  According to Courtois & Ford, for DTD to be diagnosed it requires a

history of exposure to early life developmentally adverse interpersonal trauma such as sexual abuse, physical abuse, violence, traumatic losses or other significant disruption or betrayal of the childs relationships with primary caregivers, which has been postulated as an etiological basis for complex traumatic stress disorders. Diagnosis, treatment planning and outcome are always relational. Since C-PTSD or DTD in children is often caused by chronic maltreatment, neglect or abuse in a care-giving relationship the first element of the biopsychosocial system to address is that relationship. This invariably involves some sort of child protection agency. This both widens the range of support that can be given to the child but also the complexity of the situation, since the agencys statutory legal obligations may then need to be enforced. A number of practical, therapeutic and ethical principles for assessment and intervention have been developed and explored in the field:: 67 
Identifying and addressing threats to the childs or familys safety and stability are the first priority. A relational bridge must be developed to engage, retain and maximize the benefit for the child and caregiver. Diagnosis, treatment planning and outcome monitoring are always relational (and) strengths based. All phases of treatment should aim to enhance self-regulation competencies. Determining with whom, when and how to address traumatic memories. Preventing and managing relational discontinuities and psychosocial crises.
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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