Chemotherapy and hematopoietic stem-cell transplantation (HSCT) therapies have shown great success in treating<unk> . The development of the two therapies, alongside more efficient monitoring techniques and supportive care, has reduced the overall mortality of the<unk>  from 75% to 29%. Care differs depending on the phenotype of<unk>  expressed, with treatments varying between those experiencing HLH or lymphoma, and whether or not they have been infected with EBV. Still, a bone marrow transplant that includes CD34+ hematopoietic stem cells is the only known treatment for the disorder as a whole. Patients who cannot find a bone marrow donor have a survival rate of less than 20%. In addition to the typical restrictions imposed on donor-recipient matches,<unk> 1 patients who have been infected with EBV typically receive transplants from EBV-positive donors.
X-linked lymphoproliferative disease
Autoimmune lymphoproliferative syndrome
Adult-onset immunodeficiency syndrome
Primary immunodeficiency
RAS-associated autoimmune leukoproliferative disorder
X-linked severe combined immunodeficiency
Nodal marginal zone B cell lymphoma
Splenic marginal zone lymphoma