Lymphoma - home heath advice

Overview
Definition

Lymphomas, or malignant lymphocytic neoplasms of the immune system, may be broadly separated under categories of non-Hodgkin's, Hodgkin's, and cutaneous T-cell lymphoma. The largest group of immune system neoplasms, with an annual incidence of approximately 43,000 cases, is non-Hodgkin's lymphoma. It encompasses 10 distinct disease entities divided into indolent or aggressive categories. Hodgkin's is distinguished by the presence of the Reed-Sternberg (R-S) cells, a multinuclear giant cell, and largely affects the lymphatic system, spleen, and liver. Incidence rate for Hodgkin's in the U.S. is approximately 3 to 4 cases per 100,000. A less common lymphoma, cutaneous T cell, has numerous subtypes with vastly different clinical presentations.
Etiology

The etiology of lymphomas is unclear. Hodgkin's and non-Hodgkin's have correlation with known risks. Genetic or geographic clusters suggest possibility of causative infectious agent.
Risk Factors

Non-Hodgkin's:

* Congenital immunodeficiency (e.g., ataxia-telangietasia, Wiscott-Aldridge syndrome)
* Infections Epstein-Barr virus (EBV), Helicobacter pylori, Kaposi's sarcoma herpes virus (HIV-related lymphoma), human T-cell leukemia virus type 1 (HTLV-1)
* Immunosuppressive therapy following organ transplant
* Primary Sjogren's syndrome, or secondary to lupus erythematosus
* Prior chemotherapy or radiation exposure or therapy
* Exposure to herbicides, paint thinner, hair dyes, formaldehyde, lead arsenate, creosote, benzene

Hodgkin's:

* Immunodeficiency and autoimmune diseases
* Bimodal late teens to early adulthood, and then over 65
* EBV
* Employment in woodworking or wood-related industries (occupation most consistently associated with Hodgkin's disease)
* Mononucleosis
* HIV
* Tonsillectomy
* Higher education and socioeconomic status
* Genetic predisposition
* Whites > blacks
* Men > women
* Same sex siblings 10 times greater risk

Cutaneous T Cell:

* HTLV-1
* Exposure to certain chemicals or solvents

Signs and Symptoms

Non-Hodgkin's:

* Rubbery and mobile, or hard and fixed nodes, usually in cervical or supraclavicular area
* Painful masses unusual for low grade; may be warm and erythematous for intermediate and high grade
* Liver, spleen, bone marrow, extranodal involvement
* Low-grade nodes wax and wane; higher-grade nodes abruptly appear and enlarge

Hodgkin's:

* Initially painless enlarged lymph node in neck, groin, axilla
* Pain back, abdomen, chest (especially with alcohol consumption)
* Fever, fatigue, night sweats, weight loss
* Chronic pruritus
* Cough or shortness of breath relieved by sitting up

Cutaneous T Cell:

* Skin patches erythematous, scaly
* Plaques
* Secondary infections
* Tumors lymph nodes, spleen, liver, lung

Differential Diagnosis

* Collagen vascular disorders
* Lymphadenopathy from AIDS, mononucleosis, cytomegalovirus, tuberculosis, syphilis, sarcoidosis, and other causes

Diagnosis
Physical Examination

After a complete history, all lymph node areas are examined (e.g., preauricular, epitrochlear, and popliteal nodes plus Waldeyer's ring). Tests for suspected extranodal involvement are performed. Determination of presence of a mass in the liver, spleen, or abdomen is made.
Laboratory Tests

* Lymph nodes, bone marrow, gastroscopic, and/or skin biopsy
* Complete blood count
* Erythrocyte sedimentation rate
* Antibody tests for HTLV-1 or HIV
* Liver, renal function tests
* Cerebrospinal fluid or pleural cytology

Pathology/Pathophysiology

Non-Hodgkin's:

* B cell monoclonal surface immunoglobulin and B or T cell differentiation antigens in all histologic subtypes
* Enlarged nodes resulting in lymphedema, ureteral obstruction, epidural spinal cord compression
* Bulky lymph node masses (> 10 cm) compress the mesentry, mediastinum, and retroperitoneum
* Involvement of Waldeyer's ring

Hodgkin's:

* Classic malignant, large multinucleated Reed-Sternberg (RS) cells; tumors contain many T lymphocytes, eosinophils, neutrophils, and histiocytes; mononuclear or lacunar variants; nodular, diffuse, or interfollicular patterns; 40% to 70% are EBV-positive
* Nodular lymphocyte predominance rare form; lymphocytic and histiocytic tumor cell; popcorn cell; nodular pattern

Cutaneous T Cell:

* Malignant proliferation of T cells
* Mycosis fungoides cell lymphocytes with large, hyperchromatic, convoluted nuclei; scanty cytoplasm
* Sezary's syndrome a variant of the above, generalized redness and scaling of the skin with circulating atypical lyphocytes
* Plaques acanthosis and elongation of rete ridges
* Patchy band-like infiltrate of lymphocytes and histiocytes
* Pautrier's microabscesses

Imaging

* Chest, pelvic, intrathoracic area, abdominal computed tomography (CT) if indicated
* Chest radiography
* Bone scan or radiographs if indicated
* Lymphography especially with inguinal or iliac involvement
* Gallium scan images neoplasms especially in the liver, spleen, bone, skeleton

Other Diagnostic Procedures

* Excision of lymph node(s) for biopsy
* Staging for Hodgkin's, sometimes also used for non-Hodgkin's identifies sites of tumor involvement in relation to the diaphragm and best treatment strategy
* Staging laparotomy with splenectomy invasive; reduces need for chemotherapy; allows smaller radiation fields; unnecessary if chemotherapy is part of treatment plan

Treatment Options
Treatment Strategy

Treatment is based on diagnostic information involving history, histologic subtype, staging, tumor bulk, general health, and age.
Drug Therapies

Non-Hodgkin's:

* Regional radiation infrequently used due to early hematogenous spread
* Single-agent or multiagent chemotherapy with or without radiation for low-grade stage III or IV
* Chemotherapy initial response good then gradual resistance; higher doses needed to treat aggressive and recurrent disease
* Example of multiagent chemotherapy: cyclophosphamide (750 mg/m2 IV d 1), doxorubicin (50 mg/m2 IV d 1), vincristine (1.4 mg/m2 IV d 1), and prednisone (100 mg/m2 IV daily PO d 1 5) [CHOP] 21-day cycle
* Alpha interferon after initial chemotherapy reduces remission, may increase survival
* Bone marrow transplant salvage therapy

Hodgkin's:

* Radiation alone or with chemotherapy (based on presence of prognostic indicators) combination therapy increases freedom from recurrence but not survival rates
* Six cycles of multiagent chemotherapy followed by regional or mantle irradiation effective for large mediastinal adenopathy or bulky tumor
* Radiation side effects include dry mouth, pharyngitis, nausea, dermatitis, fatigue.
* Example of multiagent chemotherapy: doxorubicin (25 mg/m2 IV), bleomycin (10 mg/m2 IV), vinblastine (6 mg/m2 IV), and dacarbazine (375 mg/m2 IV) [ABVD], repeated every two weeks; side effects include nausea, vomiting, hair loss, fatigue, myelosuppression

Cutaneous T Cell:

* Emollients, moisturizers, topical steroids
* Topical chemotherapy mechlorethamine hydrochloride (nitrogen mustard HN2); long-term use causes squamous cell carcinoma
* Systemic chemotherapy (e.g., mechlorethamine, vincristine, prednisone, procarbazine)
* Psoralen plus ultraviolet A with chemotherapy relapses if not continued; risk of malignant melanoma
* Electron beam therapy penetrates only upper dermis
* Retinoids and interferon promising, especially in combination

Surgical Procedures

Surgical removal of neoplasm if indicated
Complementary and Alternative Therapies

Herbal therapies may be beneficial in supporting the lymph system as well as enhancing immunity. Diet and nutritional supplements optimize detoxification and antioxidant activities. Improved relaxation and decreased stress, through such activities as guided imagery, tai chi, yoga, and meditation are helpful in promoting a sense of well-being. Intimacy and support from others helps promote a positive and empowering attitude.
Nutrition

* Avoid foods that may interfere with optimal immune function, such as refined foods, sugar, alcohol, caffeine, and saturated fats (e.g., animal products).
* Use only organically-raised foods. Include foods that support detoxification, immunity, and are high in antioxidant nutrients, such as beets, carrots, artichokes, yams, onions, garlic, dark leafy greens, yellow and orange vegetables, shiitake mushrooms, green tea, and filtered water. Green tea may potentiate the effects of doxorubicin.
* Vitamin C (1,000 mg qid), vitamin E (400 IU bid), beta carotene (50,000 IU one to two times daily), coenzyme Q10 (100 mg tid), and zinc (30 mg/day) for antioxidant protection and immune support. Coenzyme Q10 and L-carnitine (600 mg tid) may protect against cardiac toxicity secondary to doxorubicin.
* Selenium (200 mcg bid) and glutathione (500 mg bid) to support detoxification.
* B-complex (50 to 100 mg) to reduce the effects of stress.
* Juicing: Combine equal parts romaine lettuce, green pepper, celery, parsley, cucumber, and apple or pear (for flavor). Use organic fruits and vegetables. Drink one glass per day.

Herbs

Herbs may be used as dried extracts (pills, capsules, or tablets), teas, or tinctures (alcohol extraction, unless otherwise noted). Dose is 1 heaping tsp. herb/cup water steeped for 10 minutes (roots need 20 minutes). Traditional alterative formulas may be employed in lymphoma for detoxification, tumor inhibition, and immune support. Choose one or more of the following.

* Commercial Hoxsey-like formulas or trifolium compounds include red clover (Trifolium pratense), burdock root (Arctium lappa), Oregon grape (Mahonia aquifolium), queen's delight (Stillingia sylvatica), barberry (Berberis vulgaris), licorice root (Glycyrrhiza glabra), poke root (Phytolacca americana), prickly ash bark (Xanthoxylum clava-herculis), and yellow dock (Rumex crispus). Take 60 drops bid to tid for six months or longer.
* Scudder's Alterative Compound: combine equal parts of corydalis tubers (Dicentra Canadensis), black tag alder (Alnus serrulata), mayapple root (Podophyllum peltatum), figwort (Scrophularia nodosa), and yellowdock (Rumex crispus). Take 30 to 40 drops tincture tid to qid for six months.
* Essiac: sheep sorrel (Rumex acetosella), burdock root (Arctium lappa), slippery elm inner bark (Ulmus fulva), and turkey rhubarb (Rheum palmatum). Another version, Flor-Essence includes additional herbs, such as watercress (Nasturtium officinale). Drink one cup tea bid, or take 2 tbs. formula bid for six months.

For general long-term immune and lymphatic support:

* Combine equal parts of astragalus (Astragalus membranaceus), lomatium root (Lomatium dissectum), marigold (Calendula officinalis), red clover (Trifolium pratense), blue flag (Iris versicolor), and cleavers (Galium aparine). Drink two to three cups of tea per day.
* Siberian ginseng (Eleutherococcus senticosus): 30 to 60 drops bid in the morning and at noon to increase stamina. Do not take after 3 pm as it may induce wakefulness at night.

Homeopathy

May be helpful in addressing symptomatic complaints and strengthening overall constitution. An experienced homeopath would consider the individual's constitution and may be effective at decreasing side effects from chemotherapy.
Physical Medicine

Contrast hydrotherapy may aid in enhancing immune function and facilitating the transport of nutrients and waste products. End hot showers with one to two minutes of cold water spray. Use less extremes of temperature with debility and weakness.
Acupuncture

May be helpful in strengthening immunity and detoxification activities. May decrease side effects from chemotherapy.
Patient Monitoring

After remission, monitoring for relapse is crucial.
Other Considerations
Prevention

Avoidance of known risks
Complications/Sequelae

* Hodgkin's may develop into non-Hodgkin's
* Late-appearing side effects of radiation and/or chemotherapy, including secondary malignancies
* Infection due to immunosuppression
* Pulmonary fibrosis

Prognosis

Non-Hodgkin's:

* Stage I and II disease expected five-year survival rate of 90%
* Localized, nonbulky stage I or II with regional radiation 50% long-term disease free
* Low grade stage III or IV 2.5- to 5-year median remission rate
* After eight years, with or without treatment, disease has a propensity to change from indolent to aggressive

Hodgkin's:

* Combined-modality therapy with staging laparotomy and splenectomy (LAP)10-year survival rate: 89%
* Combined-modality therapy without LAP 10-year survival rate: 87%
* Radiation with LAP 10-year survival rate: 84%
* Most relapses occur within three years
* Fever, night sweats, weight loss, large mediastinal adenopathy, age > 40 years poorer prognosis

Cutaneous T Cell:

* Prognosis varies vastly with subtype

Pregnancy

* Occurrence is one in every 1,000 to 6,000 pregnancies (40% Hodgkin's). Many of the diagnostic (e.g., laparotomy with splenectomy) and treatment (radiation, chemotherapy) procedures are contraindicated during pregnancy. Therapeutic abortion and watchful waiting (at least until the second trimester) are considered. Delay may affect mother's prognosis, and treatment may be teratogenic.

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