LYMPHOMAS By DR : Ramy A. Samy

INTRODUCTION Neoplastic lymphoid proliferation Two types – Hodgkins & Non-Hodgkins. Fever, lymphadenopathy, Immunodeficiency / autoimmune syndrome Viral, genetic, unknown etiology. Lack of programmed cell death - Apoptosis

Lymphomas are a cancer of the lymphatic system Lymphatic vessels Lymph nodes (underarms, groin, neck, spleen, tonsils and bone marrow)

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

Lymphoma Row of enlarged lymph nodes

Lymphoma - Gross

Lymphoma Intestine

Lymphoma Intestine

Mediastinal Lymphnodes-NHL

NON-HODGKIN LYMPHOMAS Essentials of Diagnosis Often presents with painless lymphadenopathy. Pathologic diagnosis of lymphoma is made by pathologic examination of tissue.

General Considerations The non-Hodgkin lymphomas are a heterogeneous group of cancers of lymphocytes. The disorders vary in clinical presentation and course from indolent to rapidly progressive.

Associated with Non-H lymphoma SLE Celiac Disease AIDS Organ transplant patients Rheumatoid Arthritis

Classification of the lymphomas is a controversial area still undergoing evolution Eighty-five percent (85 %)of non-Hodgkin lymphomas are B-cell, 15% are T-cell. These lymphomas are either indolent (low grade) and incurable or aggressive (intermediate and high grade) and potentially curable.

Classification Usually classified by how the cells look under a microscope and how quickly they grow and spread Aggressive lymphomas (high-grade lymphomas) Indolent Lymphomas (low-grade lymphomas)

Classification of Malignant Lymphomas Low Grade: small lymphoid cells, nodular growth Intermediate Grade: large cells, follicular and diffuse patterns High Grade: immunoblastic, lymphoblastic, Burkitt’s disease T-cell lymphomas: peripheral, cutaneous

World Health Organization proposed classification of non-Hodgkin lymphomas. Precursor B    B cell lymphoblastic lymphoma Mature B    Diffuse large B cell lymphoma    Mediastinal large B cell lymphoma   Follicular center cell lymphoma    Small lymphocytic lymphoma    Lymphoplasmacytic lymphoma    Mantle cell lymphoma   Burkitt lymphoma    Marginal zone lymphoma      MALT type      Nodal type      Splenic type

Diffuse - & - Follicular NHL- Histologic types Diffuse - & - Follicular

Small – Intermed. – Large NHL- Histologic types Small – Intermed. – Large

Precursor T    T cell lymphoblastic lymphoma Mature T (and NK cell)    Anaplastic T cell lymphoma    Peripheral T cell lymphoma    Cutaneous T-cell lymphoma (mycosis fungoides)

Lymphoma spread to Spleen

Clinical Findings Symptoms and Signs Patients with indolent lymphomas usually present with : painless lymphadenopathy, which may be isolated or widespread. lymph nodes may be peripherally or centrally The indolent lymphomas are usually disseminated at the time of diagnosis, and bone marrow involvement is frequent. constitutional symptoms such as fever, drenching night sweats, and weight loss (referred to as "B" symptoms).

On examination, lymphadenopathy may be isolated, or extranodal sites of disease (skin, gastrointestinal tract, liver) may be found. Patients with Burkitt lymphoma are noted to have abdominal pain or abdominal fullness because of the predilection of the disease for the abdomen.

Laboratory Findings The peripheral blood is usually normal even with extensive bone marrow involvement by lymphoma. Circulating lymphoma cells in the blood is uncommon.

Bone marrow involvement is manifested as paratrabecular monoclonal lymphoid aggregates In some high-grade lymphomas, the meninges are involved and malignant cells are found with cerebrospinal fluid cytology. The chest radiograph may show a mediastinal mass in some lymphomas. The serum LDH has been shown to be a useful prognostic marker and is now incorporated in risk stratification of treatment.

The diagnosis of lymphoma is made by tissue biopsy.

Treatment The treatment of indolent lymphoma depends on the stage of disease and the clinical status of the patient. A small number of patients have limited disease with only one abnormal lymph node group and may be treated with localized irradiation with curative intent.

Most patients with indolent lymphoma have disseminated disease at the time of diagnosis and are not curable. When the disease is not bulky and the patient not symptomatic, no initial therapy is required. Some patients will have spontaneous remissions.

Treatment with the anti-CD20 monoclonal antibody rituximab (intravenous) is a commonly used treatment because of its very low toxicity and avoidance of chemotherapy. Combinations of rituximab with chemotherapy may also be used. Common chemotherapy regimens include fludarabine; the combination of cyclophosphamide, vincristine, and prednisone (R-CVP); and cyclophosphamide, doxorubicin, vincristine, prednisone (R-CHOP).

Prognosis The median survival of patients with indolent lymphomas is 10–15 years..

HODGKIN DISEASE Essentials of Diagnosis : Painless lymphadenopathy. Constitutional symptoms may or may not be present. Pathologic diagnosis by lymph node biopsy.

General Considerations Hodgkin disease is a group of cancers characterized by Reed–Sternberg cells in an appropriate reactive cellular background The malignant cell is derived from B lymphocytes of germinal center origin.

Reed-Sternberg Cell

Hodgkins lymphoma

Hodgkins lymphoma cells

Clinical Findings There is a bimodal age distribution, with one peak in the 20s and a second over age 50 years. painless mass, commonly in the neck. constitutional symptoms such as fever, weight loss, or drenching night sweats, or generalized pruritus. An unusual symptom of Hodgkin disease is pain in an involved lymph node following alcohol ingestion.

An important feature of Hodgkin disease is its tendency to arise within single lymph node areas and spread in an orderly fashion to contiguous areas of lymph nodes. Only late in the course of the disease will vascular invasion lead to widespread hematogenous dissemination.

Hodgkin disease is divided into several subtypes: classic Hodgkin (nodular sclerosis, mixed cellularity, and lymphocyte depleted) and non-classic Hodgkin (lymphocyte predominant). Hodgkin disease should be distinguished pathologically from other malignant lymphomas and may occasionally be confused with reactive lymph nodes seen in infectious mononucleosis, cat-scratch disease, or drug reactions (eg, phenytoin).

STAGING The staging nomenclature (Ann Arbor) is as follows: stage I, one lymph node region involved; stage II, involvement of two or more lymph node regions on one side of the diaphragm; stage III, lymph node regions involved on both sides of the diaphragm; and stage IV, disseminated disease with extranodal involvement. In addition, patients’ stages are qualified as "A" if they lack constitutional symptoms or as "B" if 10% weight loss over 6 months, fever, or drenching night sweats are present.

Staging of Lymphoma

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