NON-HODGKINS LYMPHOMAOliveros francis!!!!!!!!!!!!!!!!!

A heterogeneous group of B- and T-cell malignancies that are diverse in cellular origin, morphology, cytogenetic abnormalities, response to treatment, and prognosis

Any of a large group of cancers of lymphocytes (white blood cells).

NON-HODGKINS LYMPHOMA (NHL):

chronic myeloid leukaemia (CML)HAEMATOPOIETIC MALIGNANCIESPolycythemia vera (PV)Idiopathic myelofibrosis (MF)Essential thrombocythemia (ET)Acute myeloid leukaemia (AML)Chronic myeloid leukaemia (CML)Acute lymphatic leukaemia (ALL)Chronic lymphatic leukaemia (CLL)hairy cell leukaemia (HCL)Hodgkins lymphomaBurkitt's lymphomacutaneous T-cell lymphoma (CTCL)Non-hodgkins lymphoma (NHL)Myeloproliferative diseasesLeukaemiasMalignant lymphomas

HAEMATOPOIETIC MALIGNANCIESFamily of chronic neoplastic diseasesDue to a clonal disorder arising at the level of the pluripotent stem cellCharacterised by abnormal proliferation of 1 or more blood cell linesNeoplastic disease of a haematopoietic precursor cellCharacterised by replacement of normal bone marrowOften infiltration into other organsMalignant clones suppress normal cell formationNeoplastic disease of lymphatic tissueOriginates in lymph node or spleenHodgkins (15%)non-Hodgkins (85%)Myeloproliferative diseasesMalignant lymphomasLeukaemia

THE LYMPHATIC SYSTEM

Lymphatic TissueLymph nodes, spleen, liver, skin and the respiratory, GI and GUT tractLymphocytes undergo further proliferation and differentiation in lymphoid tissueB-lymphocytestend to reside in lymph nodes & spleenT-lymphocytestend to circulate throughout the lymphatic system

LYMPH NODE - NORMAL HISTOLOGYafferent lymphatic vesselcapsulefollicle (mainly B-cells) - germinal centre - mantle zoneCcortexmedullaparacortexefferent lymphatic vesselarteryvein

Incidence of 13.3/100,000 per year Predominates in the 40-70 years age groupmost common neoplasm in the 20-40 age groupIncidence is rising150% growth over the past 30 yearsincreasing by 4% annually since 1970sMortality rate is also rising2% rise per yearthird highest rise, exceeded only by lung cancer in women and malignant melanomaNHL INCIDENCE

STAGING OF NHL

The Non-Hodgkins Lymphoma Pathologic Classification Project. Cancer. 1982;49:2112.MODIFIED ANN ARBOR STAGING OF NHLStage I Involvement of a single lymph node region Stage II Involvement of 2 lymph node regions on the same side of the diaphragm Stage IIIInvolvement of lymph node regions on both sides of the diaphragm Stage IVMultifocal involvement of 1 extralymphatic sites associated lymph nodes or isolated extralymphatic organ involvement with distant nodal involvement

CATEGORIES OF NON-HODGKINS LYMPHOMA

FOLLICULAR NON-HODGKINS LYMPHOMA

AGGRESSIVE NON-HODGKINS LYMPHOMA

INDOLENT NON-HODGKINS LYMPHOMA

B-CELL LYMPHOMAS

Diffuse Large B-Cell Lymphoma (DLBLC). DLBCL is the most common type of non-Hodgkins lymphoma, accounting for about 30% of all NHL cases. It is an aggressive, fast-growing lymphoma that usually affects adults but can also occur in children. DLBCL can occur in lymph nodes or in organs outside of the lymphatic system. DLBCL includes several subtypes such as mediastinal large B-cell lymphoma, intravascular large B-cell lymphoma, and primary effusion lymphoma.

Follicular Lymphoma (FLs). Follicular lymphoma is the second most common type lymphoma, accounting for about 20% of all NHL cases. It is usually indolent (slow growing) but about half of follicular lymphomas transform over time into the aggressive diffuse large B-cell lymphoma.

Mantle Cell Lymphoma. Mantle cell lymphoma is an aggressive type of lymphoma that represent about 7% of NHL cases. It is a difficult type of lymphoma to treat and often does not respond to chemotherapy. It is found in lymph nodes, the spleen, bone marrow, and gastrointestinal system. Mantle cell lymphoma usually develops in men over age 60.

Small Lymphocytic Lymphoma (SLL). SLL is an indolent type of lymphoma that is closely related to B-cell chronic lymphocytic leukemia (CLL). It accounts for about 5% of NHL cases.

Marginal Zone Lymphomas (MZL). MZLs are categorized depending on where the lymphoma is located. Mucosa-associated lymphoid tissue lymphomas (MALT) usually involve the gastrointestinal tract, thyroid, lungs, saliva glands, or skin. MALT is often associated with a history of an autoimmune disorder (such as Sjogren syndrome in the salivary glands or Hashimoto's thyroiditis in the thyroid gland).

Lymphoplasmacytic Lymphoma. Lymphoplasmacytic lymphoma, also called Waldenstrom's macroglobulinemia or immunocytoma, is a rare type of lymphoma accounting for about 1% of NHL cases. It usually affects older adults and most often involves bone marrow, lymph nodes, and spleen.

Primary Central Nervous System Lymphoma. This lymphoma involves the brain and spinal cord. Although it is generally rare, it is common in people who have AIDS.

Burkitt's Lymphoma. This is one of the most common types of childhood NHL, accounting for about 40% of NHL pediatric cases in the United States. It usually starts in the abdomen and spreads to other organs, including the brain. In African children, it often involves facial bones and is associated with Epstein-Barr infection.

Lymphoblastic Lymphoma. This lymphoma is also common in children, accounting for about 25% of NHL pediatric cases, most often boys. It is associated with a large mediastinal mass (occurring in chest cavity between the lungs) and carries a high risk for spreading to bone marrow, the brain, and other lymph nodes.

PATHOGENESIS

Malignant transformation of either the T or B cells Differentiation in the peripheral lymphoid tissues

PredisposingGenderRaceFamily HistoryInfectionsImmune System Deficiency DisordersAutoimmune DisordersChemical ExposureRadiation ExposureLifestyle Factors

PrecipitatingUnknown (idiopathic)

T lumphocytes proliferate on antigenic stimulation and migrate into follicles, where they intact in B lymphocytesThese activated follicles becme germinal centers, containing macrophages, follicular dendrite cells and maturing T and B cellsDevelops in any lymphoid tissues (lymph nodes

Spreads to various lymphoid tissues throughout the body, especially the liver, spleen and bone marrowNon-hodgkins lymphomaGroup of tumors will develop

Most common: painless enlargement of one or more lymph node, usually in the neck, armpits, or groin. (painless, superficial lymphadenopathy)Usually asymptomatic

Systemic B Sx:Drenching night sweatsUnexplained weight lossFeverSevere itching

PREVENTION and Management

Resting your stomach and being alert for signs of dehydrationGentle exercise along with adequate intake of fluids and a diet that is high in fruits, vegetables, and fiber. Get extra rest while you are receiving chemotherapy or radiation therapy. Change your diet, rinsing your mouth with liquid medicines, and putting a baking soda paste or nonprescription medicine on mouth sores.If you find you have trouble sleeping, having a regular bedtime, getting some exercise during the day, avoiding naps, and using other tips to relieve sleep problems may help you sleep more easily.

You may be able to reduce your stress by expressing your feelings to others. Learning relaxation techniques may also help you reduce your stress.Adapting to your body image changes may involve talking openly about your concerns with your partner and discussing your feelings with your doctor. Your doctor may also be able to refer you to organizations that can offer additional support and information.To prevent weight loss and conserve your strength, it is important to eat well during treatment for cancer.If pain occurs, many treatments are available to relieve it. If your doctor has given you instructions or medicines to treat pain, be sure to follow them. You may use home treatment for pain to improve your physical and mental well-being. Be sure to discuss any home treatment you use for pain with your doctor.

TREATMENT

Radiation therapy -uses high doses of X-rays, gamma rays, or other types of ionizing (damaging) radiation to kill cancer cells. It may be applied to the whole body or to a specific zone.

Chemotherapy is the use of cytotoxic (cell damaging) medicines to target and kill tumors. The drugs work by interrupting the DNA of fast-growing cells, preventing them from growing or reproducing.

Immunotherapy uses the bodys own immune system to attack and remove cancer cells. Doctors inject a patient with a special type of antibody, or cell marker, that binds to antigens on a cells surface.

Bone marrow transplantationFor patients with very advanced disease, extremely high does of chemotherapy may be needed. This type of chemotherapy wipes out the bodys entire immune system, including the bone marrow that produces blood cells. So, patients need a bone marrow transplant in order to recover.

History and OverviewStatisticsWhat it effects (biology)Classification and StagingSymptomsCauses and Risk FactorsDiagnosis and Treatment

Lymphomas are a cancer of the lymphatic systemLymphatic vesselsLymph nodes (underarms, groin, neck, spleen, tonsils and bone marrow)

The Lymphatic system is our bodies main fight against infectionLymphocytes (B-cell and T-cell)Carried through our lymphatic system and help our bodies fight infectionLymphocytes are carried through the lymph vessels as well as the blood stream, so cancer can start in nodes and spread anywhere throughout the body.

American Medical Association

54,370 new cases 19,410 deathsIn California Alone there are est. 5,550 new cases and a Death rate of 1,730 (California has the highest incidence and Death rate for Non-Hodgkins LymphomaNon-Hodgkins Lymphoma is the 6th and 7th most deadly cancers for men and women Recent studies have shown that overweight patients are more likely to die then those patients who are in better shape.

Usually classified by how the cells look under a microscope and how quickly they grow and spreadAggressive lymphomas (high-grade lymphomas)

Indolent Lymphomas (low-grade lymphomas)

Stage is the term used to describe the extent of tumor that has spread through the body( I and II are localized where as III and IV are advanced.Each stage is then divided into categories A, B, and EA: No systemic symptomsB: Systemic Symptoms such as fever, night sweats and weight lossE: Spreading of disease from lymph node to another organ

Two main types of Non-Hodgkins Lymphoma:

B-Cell and T-Cell LymphomasB-Cell lymphomas (80%)T-Cell lymphomas (15%)

B-Cells help make antibodies, which are proteins that attach to and help destroy antigensLymphomas are caused when a mutation arises during the B-cell life cycleVarious different lymphomas can occur during several different stages of the cycleFollicular lymphoma, which is a type of B-cell lymphoma is caused by a gene translocation which results in an over expressed gene called BCL-2, which blocks apoptosis.

The T-cells are born from stem cells, similar to that of B-cells, but mature in the thymus.They help the immune system work in a coordinated fashion.These types of lymphomas are categorized by how the cell is affectedAnaplastic Large cell Lymphoma, t-cell lymphoma caused by a gene translocation in chromosome 5

Painful Swelling of lymph nodes located in the neck, underarm and groin.Unexplained FeverNight SweatsConstant FatigueUnexplained Weight lossItchy Skin

The Exact causes are still unknownHigher risk for individuals who:Exposed to chemicals such as pesticides or solventsInfected w/ Epstein-Barr VirusFamily history of NHL (although no hereditary pattern has been established)Infected w/ Human Immunodeficiency Virus (HIV)

X-RaysCT scansMagnetic Resonance Imaging (MRI)BiopsyLymphangiogramPictures of the lymphatic system taken w/ x-ray after a special dye is injected to illuminate lymph nodes and vessels

Non-Hodgkins Lymphoma is usually treated by a team of physicians including hematologists, medical oncologists and a radiation oncologist.

In some cases such as for Indolent lymphomas, the Doctor may wait to start treatment until the patient starts showing symptoms, known as watchful waiting

ChemotherapyRadiationBone Marrow TransplantationSurgeryBortezomib (Velcade)ImmunotherapyUsing the bodies own immune system combined with material made in a lab.

Survival Rates vary widely by cell type and staging.

1 Year Survival Rate: 77%

5 Year Survival Rate: 56%

10 Year Survival Rate: 42%

********Although it was originally designed for staging Hodgkins disease, the modified Ann Arbor staging system is also commonly used to define the extent of disease in NHL.83 However, this system does not address certain prognostic or therapeutic issues known to be important in NHL, such as bulky disease (lesion >10 cm in diameter). Each of the stages is further subdivided A patients without B symptomsB patients with B symptoms (unexplained weight loss, sweats, high fever, or pruritis) E extranodal lymphoid malignancies; a symbol for the specific site may also be used: nodes (N), spleen (S), liver (H), pleura (P), lung (L), bone (O), bone marrow (M), skin (D) ****