LYMPHOMAS

Reported by J.P. Calibuso, S.M.Macapugay, M.G.Malihan, J.C.Rueda

It is the primary tumors originating the lymphatic system and the most common tumor of the lymphoid system (Black). They are the abnormal overgrowth of one type of leukocyte, the lymphocyte (Ignativicius)

LYMPHOMAS

Thomas Hodgkins first described the disorder 1832; in the 20th century, realization that the disease is a lymphoid malignancy led to it being renamed Hodgkins Lymphoma A potential curable lymphoma with distinct histology , biologic behavior, and clinical characteristics.

Hodgkins Lymphoma

Sternberg and Reed Described as giant cells which are Reed Sternberg cells of Hodgkins Lymphoma

(It is known as the cancer of the lymph)

Reed- Sternberg Cells

Exact cause is unknown Epstein-Barr Virus (EBV) is believe to be a causative agent EBV-associated lymphomas are well documented in clients who have received organ transplant or immune deficiency disease HIV infected patients have a higher prevalence of the disease

Risk Factors

More prevalent in males Age peaking at 15 to 3 years old or 55 years old and above Occurs more frequently with in Jews and along first degree relatives 1% Genetic predisposition contributes to the pathogenesis if HL Siblings of an affected individual have 3 to 7 fold increase risk for developing HL

Risk Factors

Exists in 5 types (WHO) Classic Nodular Sclerosis Mixed cellularity Lymphocyte depleted Lymphocyte rich Nodular lymphocyte Predominant HD

Types

Nodular Sclerosis 62 to 80% of all cases of HL. Usually in adolescents and young adults. Involves mediastinum and other supra diaphragmatic sites Mixed cellularity 15 to 30% of all cases of HL. Abdominal lymph nodes and spleen. Most commonly observed with patients with HIV.

Types

Lymphocyte depleted 1% of all cases of HL. Usually older age and usually seen with HIV positive status. Lymphocyte rich 5% of cases of HL. Similar with mixed cellularity.

Types

Nodular Lymphocyte
Predominant HD 5% of all cases of HL. Reed sternberg cell are either infrequent or absent

Types

Cancerous transformation from a particular sire in LN Continuing growth in the entire node and becomes replaced With zones of necrosis observing the normal nodular pattern Mechanisms of growth remains unknown

Pathophysiology

Some suggested that the disease progress by extension to adjacent structures May be disseminated by lymphatic cicrculation Hematologic spread may occur Possibility by means of direct infiltration of tissues

Pathophysiology

Asymptomatic Painless lymphadenopathy Enlarged lymph nodes commonly found in supraclavicular and cervical Severe pruritus (early signs) Irrelgular fever (elevated for few days then drops to normal to subnormal for several day) Continuous fever may indicate impeding death

Clinical Manifestations

Jaundice Hepatosplenomegaly Renal failure Progressive anemia accompanied by fatigue, malaise and anorexia Edema and cyanosis of the face and neck Pulmonary Sx: Non-productive cough, stridor, dyspnea, chest pain, cyanosis, pleural effussion

Alcohol induced pain in the bone Bone pain, vertebral compression Paraplegia Nerve pain

Stage I - Single lymph node group Stage 2 - Multiple lymph node groups on same site on same site of diaphragm

Costwold Staging
Modified Ann Arbor Staging

Stage 3 - Multiple lymph node gropu on both sides of diaphragm Stage 4 - Multiple extranodal sites on lymph nodes and extranodal disease

Costwold Staging
Modified Ann Arbor Staging

Imaging: anteroposterior and lateral chest radiography, CT scan of chest, abdomen and pelvis, positron emission tomography (PET). Biopsy: bone marrow biopsy, sampling of pleural effusion, CNS evaluation of lumbar puncture. Blood studies: CBC, ESR, lactate dehydrogenase, serum creatinine and alkaline phosphatase, HIV test

Diagnostic procedure

Thyroid dysfunction Thyroid cancer Sexual dysfunction Male impotence Male and female infertility Female dyspareunia Infection (Herpes Zoster or Varicella) Pulmonary dysfunction Pneumonitis

Complications

Radiation Therapy Involves three locations: the mantle, the para-aortic region and the pelvis. In clinical stage I and II disease, combined chemotherapy and radiation therapy

Medical Management

Chemotherapy - MOPP (mechlorethamine, Vincristine(Oncovin) Procarbazine, Prednisone - ABVD (Doxorubicin (adriamycin), Bleomycin, Vinblastine, Dacarbazine)

Medical Management

Salvage Chemotherapy
-

ICE (Ifosfamide, Carboplatin, Etoposide) DHAP (Cisplatin, Cytarabine, Predinose) ESHAP (Etoposide, Methyprednisolone, Cytarabine, Cisplatin

Medical Management

Salvage Chemotherapy
-

ICE (Ifosfamide, Carboplatin, Etoposide) DHAP (Cisplatin, Cytarabine, Predinose) ESHAP (Etoposide, Methyprednisolone, Cytarabine, Cisplatin

Hematopoetic Stem Cell Transplant

Medical Management

Comprises a group of malignacies with a common origin in the lymphoid cells. All lymphoid cancer the do not have the Reed-Sternberg Cell

Non-Hodgkins Disease

Result from chromosomal translocation, infections(EBV, HTLV-1, herpes virus, helicobacter pylori, caposis sarcoma), environmental factors, immunodeficiency states and chronic inflammation. Most common in males Age 50s to 60s White people are high risk

Etiology and Risk Factors

Abnormal proliferation of neoplastic lymphocytes occurs Cell remains fixed at one phase of development and continue proliferate Clinical manifestations are due to mechanical obstruction of the enlarged lymph nodes Lymphocytic infiltration of the abdomen or oropharynx also can occur

Pathophysiology

Enlarge lymph node (cervical, axillary, inguinal, and femoral) Swelling is generally painless Extranodal sites of involvement are the nasopharynx, GI tract, bone, thyroid, testes, and soft tissue. Some clients have retroperitoneal and abdominal masses with abdominal fullness, back pain, ascites and leg swelling.

Clinical Manifestation

Clients may also experience systemic B symptoms, including night sweats, fever, and weight loss. Other Conditions Tuberculosis, syphilis, systemic lupus erythematous, lung cancer, bone cancer.

Contd..

Low grade - diffuse, lymphocystic, well differentiated. - Nodular, lymphocytic, poorly differentiated - Nodular, mixed, lymphocytic and histocytic
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Intermediate grade Nodular, histocytic Diffuse, lymphocytic Diffuse, mixed, lymphocytic, and histocytic

Rappaport Staging

High grade Diffuse, hidtocytic Diffuse, lymphoblastic Diffuse, uundifferentiated

Contd

CBC, ESR, and peripheral blood smear Blood cultures Lymph node biopsy CT scan MRI Bilateral bone marrow biopsies

Diagnostic procedures

Eradication of Tumor Cells/Surgery Radiation and Chemotherapy Monoclonal antibodies (Rituximab (Rituxan), Ibritumomab Tiuxetan (Zevalin), Alemtuzumab (Campath) Corticosteroids

Medical Management