Lymphoma

This lecture was conducted during the Nephrology Unit Grand Ground by Medical Student rotated under Nephrology Division under the supervision and administration of Prof. Jamal Al Wakeel, Head of Nephrology Unit, Department of Medicine and Dr. Abdulkareem Al Suwaida. Nephrology Division is not responsible for the content of the presentation for it is intended for learning and /or education purpose only.

Reem al-mutairi425201510

Outlines Introduction of lymphoma Classification of lymphomaHodgkin's lymphoma:i. Introductionii. Symptomsiii.Signsiv.Diagnosisv. Stagingvi.Managementvii.Complication of treatmentviii.Prognosisix.Systemic involvednon-hodgkin’s lymphomaStem cell transplantation

Introduction of lymphomaThe lymphomas are

malignant tumors of lymphoid tissue ,characterized by the abnormal proliferation B or T cells in lymphoid tissue .

Classification of lymphoma

Hodgkin’s lymphomaIntroduction: - Hodgkin lymphoma (formerly called Hodgkin's

disease) is a group of cancers characterized by Reed-Sternberg cells in an appropriate reactive cellular background. An important clinical feature is its tendency to arise within lymph node areas and to spread in an orderly fashion to contiguous areas of lymph nodes . Late in the course of the disease, vascular invasion leads to widespread hematogenous dissemination.

-Hodgkin lymphoma has a bimodal age distribution with one peak in the 20s and 30s, and a second peak over the age of 50.

Pathological classification of hodgkin’s lymphoma

Symptoms of hodgkin’s lymphoma:

General symptoms:I.Fever II.Weight lossIII.Loss of appetiteIV.Night sweatsV.PruritusVI.lethargy

Local symptoms:I.Enlarged, painless, non-tender, superficial

lymph nodesII.Alcohol-induced pain ــــRarely, patients with

Hodgkin’s lymphoma complain of severe pain following alcohol ingestion. The pain typically occurs within a few minutes after the ingestion of even a small amount of alcohol. The mechanism is unknown.

Symptoms related to mass:1.Mediastinal mass: retrosternal chest pain, cough, or shortness

of breath. 2. Retroperitoneal lymphadenopathy discomfort and pain in the paravertebral or

loin regions, particularly in the supine position.

Signs of HL:1.Lymph node enlargement2.Cachexia3.Anemia4.Splenomegaly5.Hepatomegaly6.Jundice. rarely

Diagnosis BLOOD: (FBC, Film, ESR, LFT, LDH, Urate , Ca.)Lymph node excision biopsy , image guided

needle biopsy.Chest X-ray, CT of thorax, abdominal, pelvis and bone marrow biopsyـــــــstaging of HL

stagingI. Confined to single lymph node regionII. Involvement of two or more nodal areas on

the same side of the diaphragmIII. Involvement of nodes on both sides of

diaphragm.IV. Spread beyond the lymph nodes e.g. liver or

bone marrow. each stage is subdivided into A and B

Management I-A and II-A : Radiotherapy & short course of

chemotherapy ( ABVD: Adriamycin, Bleomycin, Vinblastine and Dacarbazine)

II-A with more than 3 areas involved through TO IV-B Radiotherapy & long course of chemotherapy

In relapsed disease : high dose of chemotherapy and peripheral

stem cell transplantation

Complications of treatment Due to radiotherapy: Second malignancies-solid tumors ,IHD,

Hypothyroidism and lung fibrosis Due to chemotherapy: Myelosupperssion, nausea, infection, alopecia

and AMLDue to both of them : NHL & infertility

Prognosis of HLDepends on stage : >95% in I-A of HL 5ــــــــ -year survival <40% with IV-B of HL 5 ــــــــ -year

survival

Systemic involvedSkin lesions — A variety of skin

lesions have been associated with Hodgkin lymphoma. These include ichthyosis, acrokeratosis (Bazex syndrome), urticaria, erythema multiforme, erythema nodosum, necrotizing lesions, hyperpigmentation, and skin infiltration

ichthyosis

acrokeratosis

urticaria

Erythema nodosum

Hyperpigmentation of skin

necrotizing lesion

Nephrotic syndrome — The nephrotic syndrome can occur as a paraneoplastic syndrome in patients with early stage Hodgkin lymphoma, possibly due to secretion of a toxic lymphokine, such as IL-13. The usual pathologic pattern is that of minimal change disease but focal glomerulosclerosis, which represent a more severe manifestation of the same pathologic process, also can occur

Hodgkin’s lymphoma splenic involvement Risk factors for splenic involvement CS III-IV disease B symptoms Mixed cellularity or lymphocytic depletion

histology, Age greater than or equal to 40 years Two or more supradiaphragmatic sites Male sex

Hodgkin’s lymphomaNeurology system : These include paraneoplastic cerebellar

degeneration, chorea, neuromyotonia, limbic encephalitis, subacute sensory neuronopathy, , and subacute lower motor neuronopathy.

Non-hodgkin’s lymphomaThis is lymphoma without of Reed-Sternberg

cell.B lymphocyte more than T lymphocyte.The extranodal involvement is common,more

than HL.Causes of it’s congential and acquired

immunodeficiency e.g drugs , HIV infection, H.pylori infection

Diffuse lymphocytic non hodgkin’s lymphoma

Lymphomas may be grouped by how quickly they are likely to grow:

Indolent (also called low-grade) lymphomas grow slowly. They tend to cause few symptoms.

Aggressive (also called intermediate-grade and high-grade) lymphomas grow and spread more quickly. They tend to cause severe symptoms. Over time, many indolent lymphomas become aggressive lymphomas.

Low grade non Hodgkin's lymphomasSmall cell lymphocyticFollicular (it is the most common type of

lymphoma)Mantle cell Splenic marginal zone lymphoma MALT lymphoma Lymphoplasmacytic NHL

High grade non Hodgkin's lymphomasDiffuse large B cell Diffuse mixed cell lymphoma Burkitt's lymphoma Anaplastic large cell lymphoma Diffuse mixed cell lymphoma

Clinical manifestations : as HL

Diagnosis :BloodLymph node excision biopsy , image guided

needle biopsy.Chest X-ray, CT of thorax, abdominal, pelvis and bone marrow biopsyـــــــstaging of NHL

Stages of NHL : Stage I: The lymphoma cells are in one lymph node group

or in only one part of a tissue or organ (such as the lung). Stage II: The lymphoma cells are in at least two lymph

node groups on the same side of the. Diaphragm Or, the lymphoma cells are in one part of an organ and the lymph nodes near that organ (on the same side of the diaphragm).

Stage III: The lymphoma is in lymph nodes above and below the diaphragm. It also may be found in one part of a tissue or an organ near these lymph node groups.

Stage IV: Lymphoma cells are found in several parts of one or more organs or tissues (in addition to the lymph nodes). Or, it is in the liver, blood, or bone marrow

each stage is subdivided into A and B

Treatment

prognosisIncreased age, advanced stage, concomitant

disease, raised LDH and T cell lymphoma are poor prognostic signs

5-years survival for treated patients > 50% for low grade 30% for high grade

Indications for stem cell transplantationMultiple myelomaLeukemia LymphomaSever combined immunodeficiency or

congenital neutropenia with defective stem cell.

A plastic anemia Sickle cell diseaseEwing’s sarcomaNeuroblastomaMyelodysplastic syndrome

Types of stem cell transplantationAutologous stem cell transplantation: This

type of transplant uses your own stem cells. Your stem cells are removed before high-dose treatment. The cells may be treated to kill lymphoma cells that may be present. The stem cells are frozen and stored. After you receive high-dose treatment, the stored stem cells are thawed and returned to you.

Allogeneic stem cell transplantation: Sometimes healthy stem cells from a donor are available. Your brother, sister, or parent may be the donor. Or the stem cells may come from an unrelated donor. Doctors use blood tests to be sure the donor's cells match your cells.

Syngeneic stem cell transplantation: This type of transplant uses stem cells from a patient's healthy identical twin.

source of stem cell transplantation

Bone marrowPeripheral blood stem cellUmbilical cord blood

Complications of stem cell transplantationInfectionVeno-occlusive diseaseMucositisGraft-versus-host disease (GVHD) GVHD is an inflammatory disease that is

unique to allogeneic transplantation

General prognosis of stem cell transplantationwidely dependent upon disease type, stage,

stem cell source.

Referances National cancer institute, www.cancer.goWWW.UpToDate.COM ,2009