Lymphoproliferative disorders

Lymphoproliferative disorders (LPDs) refer to a specific class of diagnoses, comprising a group of several conditions, in which

Lymphoproliferative disorders (examples)

• Follicular lymphoma
• Chronic lymphocytic leukemia
• Acute lymphoblastic leukemia
• Hairy cell leukemia
• Hemophagocytic lymphohistiocytosis (HLH)
• B-cell lymphomas
• T-cell lymphomas
• Multiple myeloma
• Waldenström's macroglobulinemia
• Wiskott–Aldrich syndrome
• Langerhans cell histiocytosis (LCH)
• Lymphocyte-variant hypereosinophilia
• Pityriasis lichenoides (PL, PLC, PLVA)
• Post-transplant lymphoproliferative disorder
• Autoimmune lymphoproliferative syndrome (ALPS)
• Lymphoid interstitial pneumonia^[1]
• Epstein–Barr virus-associated lymphoproliferative diseases
• Castleman disease
• X-linked lymphoproliferative disease

Types

Lymphoproliferative disorders are a set of disorders characterized by the abnormal proliferation of

A mutation on the X chromosome is associated with a T cell and natural killer cell lymphoproliferative disorder.^{[citation needed]}

Autoimmune lymphoproliferative disorder

Some children with autoimmune lymphoproliferative disorders are heterozygous for a mutation in the gene that codes for the Fas receptor, which is located on the long arm of chromosome 10 at position 24.1, denoted 10q24.1.^[3] This gene is member 6 of the TNF-receptor superfamily (TNFRSF6). The Fas receptor contains a death domain and has been shown to play a central role in the physiological regulation of programmed cell death. Normally, stimulation of recently activated T cells by antigen leads to coexpression of Fas and Fas receptor on the T cell surface. The engagement of Fas by Fas receptor results in apoptosis of the cell and is important for eliminating T cells that are repeatedly stimulated by antigens.^[4] As a result of the mutation in the Fas receptor gene, there is no recognition of Fas by Fas receptor, leading to a primitive population of T cells that proliferates in an uncontrolled manner.^[2]

Other inherited causes

Boys with X-linked immunodeficiency syndrome are at a higher risk of mortality associated with Epstein–Barr virus infections, and are predisposed to develop a lymphoproliferative disorder or lymphoma.^{[citation needed]}

Children with common variable immunodeficiency (CVID) are also at a higher risk of developing a lymphoproliferative disorder.^{[citation needed]}

Some disorders that predispose a person to lymphoproliferative disorders are severe combined immunodeficiency (SCID), Chédiak–Higashi syndrome, Wiskott–Aldrich syndrome (an X-linked recessive disorder), and ataxia–telangiectasia.^{[citation needed]}

Even though ataxia telangiectasia is an autosomal recessive disorder, people who are

• Evans syndrome
• Leukaemia
• Lymphoma
• Lymphocytosis
• Myeloma
• Myeloproliferative disease

References