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.