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Immunoproliferative disorders[1]
Immunoproliferative disorders are conditions characterized by the abnormal proliferation of immune cells. There are three main classes: lymphoproliferative disorders involving excessive lymphocytes, hypergammaglobulinemia with high gamma globulin levels, and paraproteinemia with monoclonal proteins. Common symptoms include diarrhea, abdominal pain, weight loss and fatigue. Lymphoproliferative disorders occur in immunocompromised individuals and can be caused by genetic mutations or acquired/iatrogenic factors. Hypergammaglobulinemia and paraproteinemia involve excessive antibodies and can indicate underlying disorders. Diagnosis involves scans and blood tests, while treatment options include chemotherapy, radiation and surgery.
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Immunoproliferative disorders[1]
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- 3. INTRODUCTION • Immunoproliferative disorders,alsoknown as immunoproliferative diseases or immunoproliferative neoplasms, are disorders of the immune system that are characterized by the abnormal proliferation of the primary cells of the immune system, which includes B cells, T cells and Natural killer (NK) cells, or by the excessive production of immunoglobulins.
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- 5. SIGNS & SYMPTOMS 1.Thesymptoms of these conditions can vary from patient to patient and condition to condition, but the most common symptoms include: 2.Diarrhea 3.Abdominal pain 4.Unexplained weight loss 5.Anorexia 6.Mass in the abdomen
- 6. LYMPHOPROLIFERATIVE DISORDERS • 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.
- 7. TYPES OF LPDS 1.A mutation on the X chromosome is associated with a T cell and natural killer cell lymphoproliferative disorder 2. 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 There are two main types of LPDs X-linked Lymphoprolif erative disorder Autoimmune lymphoprolife rative disorder
- 8. CAUSES • Acquired causes •Iatrogenic causes • Other inherited causes
- 9. HYPERGAMMAGLOBULINEMIA • Hypergammaglobulinemia isa medical condition with elevated levels of gamma globulin.
- 10. TYPES Hypergammaglobulinemia Type 1 X-linked immunodeficiency with hyper– immunoglobulinM, which is also called type 1 hyper IgM TYPE 2 Immunodeficiency with hyper IgM type 2 is caused by a mutation in the Activation-Induced Cytidine Deaminase (AICDA) gene Type3 Immunodeficiency with hyper IgM type 3 is caused by a mutation in the gene that codes for CD40 Type 4 Immunodeficiency with hyper IgM type 4 is poorly characterized. Type 5 Immunodeficiency with hyper IgM type 5 is caused by a mutation in the Uracil- DNA glycosylase (UNG) gene
- 11. PARAPROTEINEMIA • Paraproteinemia, ormonoclonal gammopathy, is the presence of excessive amounts of paraprotein or single monoclonal gammaglobulin in the blood. It is usually due to an underlying immunoproliferative disorder. • It is sometimes considered equivalent to plasma cell dyscrasia
- 12. TYPES Paraproteinemia Light chains Heavychains Whole immunoglobuli ns The three types of paraproteins may occur alone or in combination in a given individual. Note that while most heavy chains or whole immunoglobulins remain within blood vessels, light chains frequently escape and are excreted by the kidneys into urine, where they take the name of Bence Jones protein.
- 13. DIAGNOSIS & TREATMENT Immunoproliferativedisease can be tested with a number of scans • MRI, • X-ray, • CT/CAT scan • Blood scans Treatment can include • Chemotherapy • radiation, • surgery or • any combination of the three. • Patients with later-stage leukemia must often undergo a bone marrow transplant in order to replace the cancer-stricken cells.
- 14. REFERENCES 1. Health CommunicationNetwork. Immunoproliferative disorders - Topic Tree. 2. http://www.use.hcn.com.au/subject.%60Immunoproliferative%20Disorde rs%60/home.html. Accessed March 2007 3. "Idiopathic Interstitial Pneumonias: Interstitial Lung Diseases: Merck Manual Professional". Retrieved 2008-12-09 4. http://www.emedicine.com/ped/topic1345.htm. Accessed March 2007 5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=gene& dopt=full_report&list_uids=355.