| Lymphoproliferative disorders |
| Classification and external resources |
| Specialty |
Hematology and oncology |
| ICD-10 |
D47.9 |
| ICD-9 |
238.79 |
| eMedicine |
ped/1345 |
| MeSH |
D008232 |
Lymphoproliferative disorders (LPDs) refer to several conditions in which lymphocytes are produced in excessive quantities. They typically occur in people who have a compromised immune system. They are sometimes equated with "immunoproliferative disorders", but technically lymphoproliferative disorders are a subset of immunoproliferative disorders, along with hypergammaglobulinemia and paraproteinemias.
Contents
- 1 Examples of LPDs
- 2 Types
- 2.1 X-linked Lymphoproliferative disorder
- 2.2 Autoimmune lymphoproliferative disorder
- 2.3 Other inherited causes
- 2.4 Acquired causes
- 2.5 Iatrogenic causes
- 3 See also
- 4 References
- 5 External links
Examples of LPDs
- follicular lymphoma
- chronic lymphocytic leukemia
- acute lymphoblastic leukemia
- hairy cell leukemia
- B-cell lymphomas
- T-cell lymphomas
- multiple myeloma
- Waldenstrom's macroglobulinemia
- Wiskott-Aldrich syndrome
- post-transplant lymphoproliferative disorder
- autoimmune lymphoproliferative syndrome (ALPS)
- "Lymphoid interstitial pneumonia"[1]
Types
Lymphoproliferative disorders are a set of disorders characterized by the abnormal proliferation of lymphocytes into a monoclonal lymphocytosis. The two major types of lymphocytes are B cells and T cells, which are derived from pluripotent hematopoetic stem cells in the bone marrow. Individuals who have some sort of dysfunction with their immune system are susceptible to develop a lymphoproliferative disorder because when any of the numerous control points of the immune system become dysfunctional, immunodeficiency or deregulation of lymphocytes is more likely to occur. There are several inherited gene mutations that have been identified to cause lymphoproliferative disorders, however there are also acquired and iatrogenic causes.[2]
X-linked Lymphoproliferative disorder
Main article: X-linked lymphoproliferative disease
A mutation on the X chromosome is associated with a T cell and natural killer cell lymphoproliferative disorder.
Autoimmune lymphoproliferative disorder
Main article: Autoimmune lymphoproliferative syndrome
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.
Children with common variable immune deficiency (CVID) are also at a higher risk of developing a lymphoproliferative disorder.
Some disorders that predispose a person to lymphoproliferative disorders are severe combined immuno deficiency (SCID), Chédiak-Higashi syndrome, Wiskott-Aldrich syndrome (an X-linked recessive disorder), and ataxia telangiectasia.
Interestingly, even though ataxia telangiectasia is an autosomal recessive disorder, people who are heterozygotes for this still have an increased risk of developing a lymphoproliferative disorder.[2]
Acquired causes
Viral infection is a very common cause of lymphoproliferative disorders. In children, the most common is believed to be congenital HIV infection because it is highly associated with acquired immunodeficiency, which often leads to lymphoproliferative disorders.[2]
Iatrogenic causes
There are many lymphoproliferative disorders that are associated with organ transplantation and immunosuppressant therapies. In most reported cases, these cause B cell lymphoproliferative disorders, however some T cell variations have been described.[2] The T cell variations are usually caused by the prolonged use of T cell suppressant drugs, such as sirolimus, tacrolimus, or cyclosporine A.[2]
See also
- Evans syndrome
- Leukaemia
- Lymphoma
- Lymphocytosis
- Myeloma
- Myeloproliferative disease
References
- ^ "Idiopathic Interstitial Pneumonias: Interstitial Lung Diseases: Merck Manual Professional". Retrieved 2008-12-09.
- ^ a b c d e f Winter, S.S. Lymphoproliferative disorders. Emedicine. December 20, 2006. http://www.emedicine.com/ped/topic1345.htm. Accessed March 2007.
- ^ Entrez Gene. FAS Fas (TNF receptor superfamily, member 6). http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=gene&dopt=full_report&list_uids=355. Accessed March 2007.
- ^ Abbas, A.K and Lichtman, A.H. Cellular and Molecular Immunology. Fifth Edition. Elsevier Saunders. Philadelphia. 2005
External links
|
Hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590–9739, 9800–9839)
|
|
B cell
(lymphoma,
leukemia)
(most CD19
|
By development/
marker |
| TdT+ |
- ALL (Precursor B acute lymphoblastic leukemia/lymphoma)
|
|
| CD5+ |
- mantle zone (Mantle cell)
|
|
| CD22+ |
- Prolymphocytic
- CD11c+ (Hairy cell leukemia)
|
|
| CD79a+ |
- germinal center/follicular B cell (Follicular
- Burkitt's
- GCB DLBCL
- Primary cutaneous follicular lymphoma)
- marginal zone/marginal-zone B cell (Splenic marginal zone
- MALT
- Nodal marginal zone
- Primary cutaneous marginal zone lymphoma)
|
|
| RS (CD15+, CD30+) |
- Classic Hodgkin's lymphoma (Nodular sclerosis)
- CD20+ (Nodular lymphocyte predominant Hodgkin's lymphoma)
|
|
PCDs/PP
(CD38+/CD138+) |
- see immunoproliferative immunoglobulin disorders
|
|
|
| By infection |
- KSHV (Primary effusion)
- EBV (Lymphomatoid granulomatosis
- Post-transplant lymphoproliferative disorder)
- HIV (AIDS-related lymphoma)
- Helicobacter pylori (MALT lymphoma)
|
|
| Cutaneous |
- Diffuse large B-cell lymphoma
- Intravascular large B-cell lymphoma
- Primary cutaneous marginal zone lymphoma
- Primary cutaneous immunocytoma
- Plasmacytoma
- Plasmacytosis
- Primary cutaneous follicular lymphoma
|
|
|
| T/NK |
T cell
(lymphoma,
leukemia)
(most CD3
|
By development/
marker |
- TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma)
- prolymphocyte (Prolymphocytic)
- CD30+ (Anaplastic large-cell lymphoma
- Lymphomatoid papulosis type A)
|
|
| Cutaneous |
| MF+variants |
- indolent: Mycosis fungoides
- Pagetoid reticulosis
- Granulomatous slack skin
aggressive: Sézary disease
- Adult T-cell leukemia/lymphoma
|
|
| Non-MF |
- CD30-: Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
- Pleomorphic T-cell lymphoma
- Lymphomatoid papulosis type B
- CD30+: CD30+ cutaneous T-cell lymphoma
- Secondary cutaneous CD30+ large cell lymphoma
- Lymphomatoid papulosis type A
|
|
|
| Other peripheral |
- Hepatosplenic
- Angioimmunoblastic
- Enteropathy-associated T-cell lymphoma
- Peripheral T-cell lymphoma-Not-Otherwise-Specified (Lennert lymphoma)
- Subcutaneous T-cell lymphoma
|
|
| By infection |
- HTLV-1 (Adult T-cell leukemia/lymphoma)
|
|
|
NK cell/
(most CD56) |
- Aggressive NK-cell leukemia
- Blastic NK cell lymphoma
|
|
| T or NK |
- EBV (Extranodal NK-T-cell lymphoma/Angiocentric lymphoma)
- Large granular lymphocytic leukemia
|
|
|
| Lymphoid+myeloid |
- Acute biphenotypic leukaemia
|
|
| Lymphocytosis |
- Lymphoproliferative disorders (X-linked lymphoproliferative disease
- Autoimmune lymphoproliferative syndrome)
- Leukemoid reaction
- Diffuse infiltrative lymphocytosis syndrome
|
|
|
| Cutaneous lymphoid hyperplasia |
- Cutaneous lymphoid hyperplasia
- with bandlike and perivascular patterns
- with nodular pattern
- Jessner lymphocytic infiltrate of the skin
|
|
|
Index of the immune system
|
|
| Description |
- Physiology
- cells
- autoantigens
- autoantibodies
- complement
- surface antigens
- IG receptors
|
|
| Disease |
- Allergies
- Immunodeficiency
- Immunoproliferative immunoglobulin disorders
- Hypersensitivity and autoimmune disorders
- Neoplasms and cancer
|
|
| Treatment |
- Procedures
- Drugs
- antihistamines
- immunostimulants
- immunosuppressants
- monoclonal antibodies
|
|
|