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IMMUNOPROLIFERATIVE DISORDER
Saad Anwar Microbiologist
AIOU,ISLAMABAD PAKISTAN
INTRODUCTION
• Immunoproliferative disorders,also known 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.
CLASSES
Immunoproliferative disorder
lymphoproliferative disorders
(cellular)
Hypergammaglobulinemia
(Humoral)Paraproteinemia
(Humoral)
SIGNS & SYMPTOMS
1.The symptoms of these conditions can vary from patient to patient and condition to condition, but the most common symptoms include:
2.Diarrhea3.Abdominal pain4.Unexplained weight loss5.Anorexia6.Mass in the abdomen
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.
TYPES OF LPDS
1. A mutation on the X chromosome is associated with a T cell and natural killer cell lymphoproliferative disorder2. 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 Lymphoprol
iferative disorder
Autoimmune
lymphoproliferative disorder
CAUSES
• Acquired causes• Iatrogenic causes• Other inherited causes
HYPERGAMMAGLOBULINEMIA
• Hypergammaglobulinemia is a medical condition with elevated levels of gamma globulin.
TYPESHypergammaglobulinemi
aType 1X-linked
immunodeficiency with hyper–
immunoglobulin M, which is also called type 1 hyper IgM
TYPE 2Immunodeficiency
with hyper IgM type 2 is caused by a mutation in the
Activation-Induced Cytidine
Deaminase (AICDA) gene
Type3Immunodeficiency
with hyper IgM type 3 is caused by a mutation in
the gene that codes for CD40
Type 4Immunodeficiency
with hyper IgM type 4 is poorly
characterized.
Type 5Immunodeficiency
with hyper IgM type 5 is caused by a mutation in the
Uracil-DNA glycosylase (UNG)
gene
PARAPROTEINEMIA• Paraproteinemia, or monoclonal
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
TYPES
Paraproteinemia
Light chains Heavy chains
Whole immunoglob
ulins
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.
DIAGNOSIS & TREATMENTImmunoproliferative disease 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.
REFERENCES
1.Health Communication Network. Immunoproliferative disorders - Topic Tree.
2.http://www.use.hcn.com.au/subject.%60Immunoproliferative%20Disorders%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.
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