The Lymphomas

The Lymphomas



Jennifer K. Simpson



Hodgkin Lymphoma



DEFINITION


Lymphomas are cancers of the B or T lymphocytes or natural killer cells that originate in the lymphatic system. The lymphomas are divided into two major categories: Hodgkin lymphoma (HL) and all other lymphomas, called non-Hodgkin lymphomas. HL is an uncommon neoplasm arising from B lymphocytes and marked by a diagnostic tumor cell called the Reed-Sternberg cell. HL is also commonly referred to as Hodgkin disease.



INCIDENCE




• HL will represent about 11.4% of all lymphomas diagnosed in 2007.


• 8,190 (4,470 males and 3,720 females) new cases in the United States are expected to be diagnosed in 2007, with 1,070 deaths from the disease


• Bimodal distribution with the first peak at 15 to 34 years of age and the second at >50 years of age



ETIOLOGY AND RISK FACTORS




• The etiology of HL is uncertain.


• History of infectious mononucleosis caused by the Epstein-Barr virus is associated with nearly half of cases. Risk can be up to 20 years after infection and is more frequently associated with childhood and older adult cases.


• Infection with human immunodeficiency virus (HIV) also has an increased probability of development of HL.


• Measles virus exposure in childhood increases risk in young adults.


• Personal history of autoimmune conditions may be linked to increased risk.


• Familial HL represents approximately 5% of new cases


˚ First-degree relative with HL results in a threefold increased risk. Risk is stronger at younger age, in males, and in siblings.


SIGNS AND SYMPTOMS




• Approximately one third of patients are symptomatic.


• B symptoms (absence or presence is key in staging):


˚ Unexplained loss of more than 10% of body weight in the 6 months before diagnosis

˚ Unexplained fever with temperatures >101.5°g F (38° C) for more than 3 days

˚ Drenching night sweats

• Pruritus


• Less common: pain in involved areas after ingestion of alcohol


• Physical findings:


˚ Painless adenopathy, commonly in the supraclavicular or cervical areas

˚ Splenomegaly


DIAGNOSTIC WORKUP




• History and physical examination


• Laboratory tests:


˚ Complete blood cell count with differential

˚ Erythrocyte sedimentation rate

˚ Serum chemistries

˚ HIV

• Bone marrow aspirate and biopsy


˚ Biopsy indicated in the presence of constitutional B symptoms or anemia, leukopenia, or thrombocytopenia (bone marrow involvement occurs in 5% of patients)

• Imaging studies:


˚ Chest x-ray film

˚ Skeletal survey

˚ Neck, thoracic, and abdominal/pelvic computerized tomographic scans

˚ Positron emission tomography scans, sometimes combined with computed tomographic scans, have replaced gallium scans and lymphangiography for clinical staging.

˚ Magnetic resonance imaging may provide information on bone marrow involvement.


HISTOLOGY


The World Health Organization modification of the Revised European-American Lymphoma classification is currently used. Two clinicopathological entities are described: nodular lymphocyte predominance HL (NLPHL) and classical HL (CHL). NLPHL is distinct from CHL: the profile for NLPHL is CD15−, CD20+, CD30−, CD45+, whereas the profile for CHL is CD15+, CD20−, CD30+, CD45−.



• NLPHL


˚ Approximately 5% of HL cases

˚ Typically diagnosed in asymptomatic young men with cervical or inguinal lymph nodes without mediastinal involvement

˚ Typical immunophenotype is CD15−, CD20+, CD30−, CD45+ B cell lymphocytes.

˚ Patients usually diagnosed with earlier-stage disease, longer survival, and fewer treatment failures

• CHL


˚ Accounts for 95% of cases

˚ Typical immunophenotype is CD15+, CD20−, CD30+, CD45− B cell lymphocytes.

˚ Four subtypes:

image Nodular sclerosis (grade I and II)
image 75% of HL cases in United States

image Equal distribution between sexes

image More prominent in patients <50 years of age

image Common anterior mediastinal involvement

image Grade I, 75% of nodules contain scattered H-RS cells

image Grade 2, 25% contain numerous H-RS cells surrounded with necrosis

image Mixed cellularity
image More prominent in males

image Frequently associated with HIV and Epstein-Barr virus infection

image Classic H-RS cells with inflammatory rich background of lymphocytes, plasma cells, and eosinophils

image Lymphocyte rich
image More common in males

image Most with stage I or II disease with rare B symptoms

image Infrequent H-RS cells background of small lymphocytes

image Lymphocyte depleted
image Least common subtype with <5% of cases

image More common in men

image Typically advanced stage disease and B symptoms at presentation

image H-RS cells predominant in background of depleted lymphocytes.


STAGING


The staging classification that is currently used was adopted in 1971 at the Ann Arbor Conference with some modifications at the Cotswolds meeting 18 years later. This staging system is based on the number and location of involved lymph nodes and extranodal involvement. The current practice is to assign a clinical stage on the basis of the findings of the clinical evaluation and a pathological stage on the basis of the findings of invasive procedures. Pathological confirmation of noncontiguous extralymphatic involvement is strongly suggested.


Stages I, II, III, and IV can be subclassified into A and B categories: A for those without B symptoms and B for those with defined B symptoms. The E designation is also used in stages I-III. The E designation notes the presence of extralymphatic disease resulting from direct extension of an involved lymph node region. It is not appropriate to use this designation in the presence of widespread disease or diffuse extralymphatic disease.



• Stage I. Involvement of a single lymph node region (I) or localized involvement of a single extralymphatic organ or site (IE).


• Stage II. Involvement of more than two lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph node(s) with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


• Stage III. Involvement of lymph node regions on both sides of the diaphragm (III), which may also be accompanied by localized involvement of an associated extralymphatic organ or site (IIIE).


• Stage IV. Disseminated (multifocal) involvement of one or more extralymphatic organs, with or without associated lymph node involvement, or isolated extralymphatic organ involvement with distant (nonregional) nodal involvement.


A prognostic staging system can also be used that is particularly useful in clinical trials. This risk system uses three major groups on the basis of prognostic features. The European Organization for Research and Treatment of Cancer defines favorable and unfavorable features for stage I and II disease and the International Prognostic Factors Project has developed an International Prognostic Index with seven adverse factors for advanced stages (stage III and IV). These are described in the box on right.



TREATMENT




• Treatment is based on stage and prognostic factors.


• Standard treatment is intensive combination chemotherapy and involved-field radiation therapy, which yields a >90% cure rate.


• Front-line chemotherapy agents are usually given in combination and may include the following:


˚ Cyclophosphamide

˚ Procarbazine

˚ Vincristine or vinblastine

˚ Prednisone or dexamethasone

˚ Doxorubicin

˚ Bleomycin

˚ Dacarbazine

˚ Etoposide

˚ Methotrexate

˚ Cytosine arabinoside

˚ Mechlorethamine

• Ritux imab is being investigated, particularly for patients with NLPHL (CD20+).


• Salvage treatments:


˚ In late recurrences the same regimen used for front-line therapy may be effective. Early recurrence should be treated with different agents.

˚ A number of chemotherapy drugs not generally used in the initial treatment have demonstrated efficacy in recurrent disease, including:

image Moderate- or high-dose cytarabine

image Carboplatin/cisplatin

image Ifosfamide

image Etoposide

image Vinorelbine

image Gemcitabine

image Vinblastine.

˚ Radiation to sites not previously irradiated



Risk-Based Staging System



EARLY FAVORABLE (EUROPEAN ORGANIZATION FOR RESEARCH AND TREATMENT OF CANCER PROGNOSTIC DEFINITIONS)




• <50 years of age


• Clinical stage I and II


• No B symptoms or elevated erythrocyte sedimentation rate


• B symptoms + erythrocyte sedimentation rate <30 mm/h


• Involvement of <3 lymph node areas


• Mediastinal involvement <33% of the thoracic width on the chest x-ray, <10 cm on computed tomographic scan

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Mar 1, 2017 | Posted by in NURSING | Comments Off on The Lymphomas

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