Lymphoproliferative disorders are a class of nonmalignant diseases characterized by uncontrolled growth of lymphoid tissues (spleen, bone marrow, liver, lymph nodes).
Can be congenita...
Typically presents at young age (average 18 months) with massive lymphadenopathy and splenomegaly
Many patients develop secondary autoimmune disease.
Most often, autoimmune destruct...
For ALPS: corticosteroids or IVIG for acute flares
For CD localized disease
Surgical resection or focal radiation. Steroids may be used to shrink lesions prior to surgery.
Dharnidharka VR, Webster AC, Martinez OM, et al. Post-transplant lymphoproliferative disorders. Nat Rev Dis Primers. 2016;2:15088. [View Abstract on OvidInsights]
George LA, Te...
238.79 Other lymphatic and hematopoietic tissues
279.41 Autoimmune lymphoproliferative syndrome
238.77 Post-transplant lymphoproliferative disorder (PTLD)
759.89 Other specified congenital anom...
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