Cancer is an abnormal growth resulting from uncontrolled division of cells. When this occurs in the lymphatic system, it is known as lymphatic cancer or lymphoma.
Presentation
The symptoms of lymphomas are varied. Patients most commonly present with painless swelling in a lymph node group, usually in the neck, axilla or inguinal group of nodes. Some present with abdominal swelling and there could also be pressure symptoms like paresthesia which results from the tumor pressing against a nerve. Other non-specific symptoms include fevers, weight loss, chills, rigors, night sweat, and easy fatigability.
Hematological
- Hemophilia A
Hemophilia is a congenital bleeding disorder characterized by deficiency of coagulation factor VIII or IX. Hemophilia B is a consequence of factor IX deficiency and has an incidence of 1 in 20,000 male births. [ncbi.nlm.nih.gov]
Immune System
- Splenomegaly
At diagnosis, patients usually present with adenopathy and, in 50% of cases, splenomegaly. [orpha.net]
However, the presence of extensive retroperitoneal lymphadenopathy and splenomegaly favours lymphoma, whereas adjacent fat infiltration supports adenocarcinoma. [radiopaedia.org]
Splenomegaly. Hepatomegaly. Intermediate- and high-grade lymphomas: Most patients present with rapidly growing and bulky lymphadenopathy. [patient.info]
Splenomegaly is clinically detectable in 50% of patients at presentation; it can cause abdominal pain or early satiety. Lymphadenopathy commonly occurs in the cervical, axillary, and supraclavicular areas. Inguinal adenopathy is rare. [web.archive.org]
Variable • severity: asymptomatic to extremely ill • time course: evolution over weeks, months, or years • Systemic manifestations • fever, night sweats (HL>NHL), weight loss, anorexia, pruritis (HL>NHL), LBP • Local manifestations • lymphadenopathy, splenomegaly [slideshare.net]
- Generalized Lymphadenopathy
When more than one area of lymph nodes is swollen it’s called generalized lymphadenopathy. [m.cancer.org]
Persistent Generalized Lymphadenopathy (PGL). [nap.edu]
- Massive Splenomegaly
T-cell CLL usually is a more aggressive disorder than B-cell CLL, with massive splenomegaly, marked neutropenia, skin infiltration, modest bone marrow infiltration, and a rapid clinical course leading to death in about 50% of these patients. [web.archive.org]
Entire Body System
- Hodgkin Lymphoma
Overview Lymphoma is a broad term for cancer that begins in cells of the lymph system. The two main types are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Hodgkin lymphoma can often be cured. The prognosis of NHL depends on the specific type. [cancer.gov]
They are broadly divided into two groups: Hodgkin's disease (Hodgkin lymphoma) and Non-Hodgkin lymphoma based on the presence of Reed Sternberg cells (RS cells). [symptoma.com]
There are two main types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. [mskcc.org]
[…] high-grade non-Hodgkin lymphoma. [cancer.ie]
lymphoma Classical Hodgkin lymphoma Nodular sclerosis classical Hodgkin lymphoma Lymphocyte-rich classical Hodgkin lymphoma Mixed cellularity classical Hodgkin lymphoma Lymphocyte-depleted classical Hodgkin lymphoma Histiocytic and dendritic cell neoplasms [labtestsonline.org]
- Fever
Fever Fever that is continuous or occurs intermittently over a period of time and doesn't seem related to a chest or urinary infection is a good sign that you should consult a doctor. [web.archive.org]
Occasionally, in those affected by Hodgkin lymphoma, a characteristic fever called Pel-Ebstein fever occurs. [lymphoma.about.com]
Other symptoms are fever, weight loss, pain, sweating at night and getting tired easily. [symptoma.com]
DO call your health care provider if you have pain, fever, or drainage from the incision after surgery. DO call your health care provider if you have shortness of breath, chest pain, or coughing with a fever after radiation. [spectrumhealth.org]
Fever is a natural response to an infection, but it may also be a sign of advanced lymphoma. Most lymphoma-related fevers are relatively low-grade. They’re often accompanied by chills. Night sweats may occur if you have a fever while asleep. [healthline.com]
- Weight Loss
Weight loss Weight loss usually occurs rapidly for no known reason. Often an individual can lose ten to fifteen pounds over a couple of months. [web.archive.org]
Unexplained Weight Loss Weight loss in the context of lymphoma usually occurs rapidly and it may have various causes. [lymphoma.about.com]
Other symptoms are fever, weight loss, pain, sweating at night and getting tired easily. [symptoma.com]
This can lead to sudden weight loss, especially since many lymphomas typically grow quickly. You should discuss any extensive and unintentional weight loss with your doctor. It can be a sign of a serious health problem. [healthline.com]
The letter B indicates the person has experienced one or more of the following B symptoms: drenching night sweats, unexplained fever or sudden weight loss. [cancercenter.com]
- Fatigue
They include: fatigue night sweats chills fever unexplained weight loss itching Fatigue as well as the lack of energy and interest can be symptoms of lymphoma. However, fatigue can also be a sign of insufficient sleep or poor diet. [healthline.com]
Other symptoms include fever, weight loss, fatigue, or night sweats. Also called Hodgkin disease. [web.archive.org]
The lymphoma patients presented significantly more often with fatigue and fever (p = 0.023 and p = 0.016 respectively) than did the CD subjects. [ncbi.nlm.nih.gov]
Side effects can depend on which drugs were given and how much, but include fatigue, hair loss, poor appetite, nausea or vomiting, diarrhea, or infertility. [hopkinsmedicine.org]
Lack of energy, tiredness or fatigue. Read on to find out the types of lymphoma and how the disease is diagnosed. Ref: S13 [healthxchange.sg]
- Chills
Common symptoms include: • Swelling of lymph nodes, which may or may not be painless • Fever • Unexplained weight loss • Sweating (often at night) • Chills • Lack of energy • Itching Most people who have these non-specific symptoms will not have lymphoma [web.archive.org]
While receiving stem cells, patients may experience pain, chills, fever, hives, chest pain or other symptoms. [hopkinsmedicine.org]
The most common symptoms of lymphoma are: Painless swelling of the lymph nodes in the neck, armpits or groin Persistent fatigue Unexplained weight loss Fever, night sweats and chills Leukemia, on the other hand, often produces bone and joint pain, anemia [moffitt.org]
Other non-specific symptoms include fevers, weight loss, chills, rigors, night sweat, and easy fatigability. There is need for laboratory tests, imaging and histologic investigation in patients with lymphoma. [symptoma.com]
Other common lymphoma symptoms include: Fever Fatigue or lethargy Loss of appetite or vomiting Sudden and dramatic weight loss Night sweats or chills Unexplained pain or swelling Difficulty moving body parts Lymphoma stages At CTCA®, a thorough and accurate [cancercenter.com]
Gastrointestinal
- Abdominal Pain
Patient 2: A 43-year-old male presented with abdominal pain and diarrhoea lasting one year. [ncbi.nlm.nih.gov]
Other signs may include fever, night sweats, fatigue, weight loss, abdominal pain or swelling, chest pain or trouble breathing, itchy skin. [publichealth.va.gov]
Sometimes triggered by alcohol Nausea, vomiting, abdominal pain If lymphoma is affecting the digestive tract Shortness of breath, cough If lymphoma is affecting the chest Headaches, vision changes, seizures If lymphoma is affecting the brain Anaemia If [lymphoma.org.au]
Sometimes there is nausea, vomiting, or abdominal pain. As lymphomas progress, the body is less able to fight infection. These symptoms are not sure signs of cancer, however. [healthcentral.com]
- Abdominal Mass
Burkitt's lymphoma: often presents with a large abdominal mass and symptoms of bowel obstruction. Differential diagnosis See also separate Generalised Lymphadenopathy and Splenomegaly and Hypersplenism articles. Hodgkin's lymphoma. [patient.info]
Patients with Burkitt lymphoma (occurring in the United States) often present with a large abdominal mass and symptoms of bowel obstruction. [emedicine.medscape.com]
The disease starts in the lymph nodes, although early involvement of the oropharyngeal lymphoid tissue or the bone marrow is common, as is abdominal mass or gastrointestinal involvement with complaints of vague back or abdominal discomfort." [1] "The [physio-pedia.com]
- Abdominal Distension
CASE PRESENTATION: An 85-year-old male complained of bowel movement pattern change, abdominal distension and loss of weight, without vomiting but with nausea. [ncbi.nlm.nih.gov]
Liver, Gall & Pancreas
- Hepatomegaly
Hepatomegaly. Intermediate- and high-grade lymphomas: Most patients present with rapidly growing and bulky lymphadenopathy. [patient.info]
Hepatomegaly may develop as the disease progresses. Jaundice usually suggests hemolysis, although biliary obstruction can result from periportal lymph node enlargement. Lymphocytic infiltration can occur in any organ. [web.archive.org]
Intermediate- and high-grade lymphomas may produce the following physical examination findings: Rapidly growing and bulky lymphadenopathy Splenomegaly Hepatomegaly Large abdominal mass : this usually occurs in Burkitt lymphoma Testicular mass Complications [emedicine.medscape.com]
Skin
- Ulcer
The patient also underwent esophagogastroduodenoscopy, which showed ulcerative tumors in the stomach. Both ascites cytology and pathology of the gastric tumors confirmed the diagnosis of B-cell lymphoma. [ncbi.nlm.nih.gov]
[…] conditions: An inherited immune system disorder An autoimmune disease, such as rheumatoid arthritis or psoriasis HIV/AIDS Human T-lymphotrophic virus Type I or Epstein-Barr virus Repeated bouts of Helicobacter pylori infection, which causes stomach ulcers [my.clevelandclinic.org]
Only a minority of people with CTCL develop advanced disease, with tumor formation, ulceration, involvement of lymph nodes, blood, and internal organs. [clfoundation.org]
Carcinoma कार्सिनोमा, कैंसर, कर्कट, कासीनोमा, कर्कट-रोग, Unlike carcinomas, metastatic sarcomas generally occur deeper and may not ulcerate the mucosa. [dictionary.studysite.org]
- Eczema
CTCL often mimics eczema, psoriasis, or other chronic dermatitis, and because of this it’s common that the diagnosis of CTCL is delayed, sometimes by years or decades. [clfoundation.org]
Pain Cirrhosis Cocaine Codependency College Colon Cancer Colorectal Cancer Coming Out Crohns Disease Cymbalta Cystic Fibrosis Dads Dementia Depression Diabetes Diverticulitis Divorce Dizziness Down Syndrome Drug Dyslexia EDNOS Eating Disorder Ecstasy Eczema [non-hodgkins-lymphoma.supportgroups.com]
It typically looks like an eczema rash and can affect parts of the body outside of the skin, such as the blood or internal organs in some cases. [utswmed.org]
These rashes often occur in skin folds and can be easily confused with other conditions like eczema. They can spread as the lymphoma progresses. Lymphoma can also form lumps or nodules within the skin. [healthline.com]
- Pruritus
[…] of 375 mg/m 2 administered as intravenous infusion. 7 However, to overcome the problem of recurrence, an additional maintenance regimen of treatment every two months for two years has been proposed. 8 Reported side effects include nausea, fever, and pruritus [aao.org]
Pel-Ebstein fever is observed (high fever for 1-2 wk, followed by an afebrile period of 1-2 wk) Chest pain, cough, shortness of breath, or a combination of those may be present due to a large mediastinal mass or lung involvement; rarely, hemoptysis occurs Pruritus [emedicine.medscape.com]
Symptoms of lymphoma may vary from patient to patient and may include one or more the following: Fevers Chills Unexplained weight loss Night sweats Lack of energy Itching (up to 25% of patients develop this itch [pruritus], most commonly in the lower [emedicinehealth.com]
elevated levels of serum lactose dehydrogenase or beta-2 microglobulin;[4] presence of localized bone lesions; kidney involvement; reduced levels of cicurlating blood platelets or any of the various types of white blood cells; onset of significant pruritus [en.wikipedia.org]
- Urticaria
UDPglucose Epimerase Ulcer Aphthous Rodent Stomach Ulnar Umbilical Hernia Undulant Unipolar Depression Upper Aerodigestive Tract Neoplasms Neck Respiratory Urea Cycle Urethral Stricture Urethritis Urinary Bladder Retention Urination Urogenital Urologic Urticaria [i2i.gi]
Face, Head & Neck
- Neck Swelling
Patients most commonly present with painless swelling in a lymph node group, usually in the neck, axilla or inguinal group of nodes. [symptoma.com]
Workup
There is need for laboratory tests, imaging and histologic investigation in patients with lymphoma.
Laboratory tests
- Complete blood count
- Erythrocyte sedimentation rate
- Lactate dehydrogenase level
- Serum creatinine
- Liver function test
- HIV screening
- Hepatitis B and C screening[6].
Imaging
- Plain radiograph
- CT scan
- PET scan which distinguishes between viable and non-viable tumor
Histology
- Excisional lymph node biopsy
- Lumber puncture to rule out CNS involvement
- Bone marrow biopsy [7]
X-Ray
- Anterior Mediastinal Mass
Clinical Evaluation As many as 75% of patients with T-cell lymphoblastic lymphoma will present with an anterior mediastinal mass, which may manifest as dyspnea, wheezing, stridor, dysphagia, or swelling of the head and neck. [9] B-cell subtypes usually [emedicine.medscape.com]
- Superior Mediastinal Mass
Lymphoblastic lymphoma, a high-grade lymphoma, often manifests with an anterior superior mediastinal mass, superior vena cava (SVC) syndrome, and leptomeningeal disease with cranial nerve palsies. [emedicine.medscape.com]
Treatment
Hodgkin's disease is generally considered to be curable. A combination of chemotherapy and radiotherapy is used in the treatment. Radiotherapy types include: Extended-field radiation, involved-field radiation and involved-node radiation.
Drugs used in chemotherapy include vincristine, adriamycin, doxorubicin, etopoxide, cyclophosphamide and many more are used in various combination therapies. The role of surgery is limited in the treatment of lymphomas. It is useful for specific types like gastrointestinal lymphoma [8].
Prognosis
For Non-Hodgkin lymphoma, patients generally have a 5 year survival rate of 63%. The prognosis is dependent on a number of factors like:
- Type of cells
- Size of the tumor
- Stage of the tumor
- Patient’s age
- Patient’s response to treatment
Hodgkin's disease has a 5 year survival rate of 85%. The outcome is dependent on factors like:
- Serum albumin less than 4g/dL
- Hemoglobin less than 10.5g/dL
- Male sex
- Age of patient 45years and above
- Ann Arbor stage IV disease
- WBC count greater than 15,000/mm3
- Absolute lymphocyte count less than 600/mm3
Each of this factors is assigned 1 point and the lower the total score, the better the prognosis [5].
Etiology
The causes of lymphoma are known carcinogens that have also been implicated in other cancers. They include:
- Infections which could be caused by viruses like Epstein-Barr virus, Hepatitis C virus and HIV and bacteria like Helicobacter pylori.
- Chemical pollutants like benzene, pesticides or hair dye.
- Chemotherapy and radiation exposure
- Drugs, especially tricyclic antidepressants (TCAs).
- Heavy metals [2]
- Chromosomal translocations
Epidemiology
Although lymphoma is currently ranked 7th in the leading types of cancers, the incidence is far becoming a major source of concern. The incidence of new cases has almost doubled since the early 70s and for the most part, there is no explanation for the rise although it might be due to new and better ways of making diagnosis.
The Non-Hodgkin type make up over 80% of all forms of lymphomas. Caucasians are at higher risk of developing this form of cancer compared to Blacks and Asians.
The incidence is generally higher in men. They are generally seen in individuals above 50 years although some subtypes are more prevalent in childhood [3].
Pathophysiology
Non-Hodgkin lymphoma results from a continuous clonal expansion of B or T cells and/or NK cells which arise from a buildup of lesions affecting protooncogenes or tumor suppressor genes leading to cell immortalization. Most of them are of B cell origin and their cell sizes and pattern of growth are determinants of tumor aggressiveness.
The presence of RS cells is the hallmark of the Hodgkin's disease. There are four established subtypes namely:
- Lymphocyte-rich Hodgkin lymphoma show typical RS cells.
- Lymphocyte depleted Hodgkin lymphoma is responsible for very few cases of Hodgkin lymphoma. An increased amount of RS cells and unusual sarcomatous modifications are present.
- The most common one is the nodular sclerosing Hodgkin lymphoma and it constitutes 3 or 4 out of every 5 incidence of Hodgkin lymphoma. It is nodular with a thickened capsule and the distinguishing cell type is the lacunar type Reed Sternberg cell.
- The fourth type is the mixed-cellularity type. Here, the RS cells give a classical appearance. Patients usually have advanced stage disease and it is the commonest type seen in patients with HIV infection [4].
A fifth type, which is now regarded as a different entity is the nodular lymphocyte-predominant Hodgkin lymphoma. It constitutes 5% of cases. Typical RS cells, if seen, are usually infrequent. There are instead lymphocytic and histiocytic cells.
Prevention
The best way to prevent this disease is to avoid the risk factors like viruses, chemical pollutants and drugs that have been implicated in its etiology [9].
Summary
Lymphatic cancer is a cancer that affects the lymphatic system. This is the system responsible for immunity. Lymphatic cancer is not a single entity but a collection of many types of cancers. They are broadly divided into two groups: Hodgkin's disease (Hodgkin lymphoma) and Non-Hodgkin lymphoma based on the presence of Reed Sternberg cells (RS cells) [1].
Patient Information
Definition
Lymphatic cancer, also known as lymphoma is a group of cancers that affects the organs responsible for immunity in the body. It is mostly seen in the extremes of age, young children and older adults.
Cause
It could be caused by long-term exposure to poisonous chemicals like benzene and insecticides. It could also be caused by viruses like the HIV virus. Other causes include drugs and heavy metals.
Symptoms
Some of the symptoms include swelling in the neck, swelling under the armpits, in the groin, and in the stomach. Other symptoms are fever, weight loss, pain, sweating at night and getting tired easily.
Diagnosis
The doctor would ask for a lot of investigations including blood tests like complete blood count to know the hemoglobin level and the number of white blood cells and platelets. HIV and hepatitis screening could be ordered as well as specific chemical blood tests. Other imaging techniques like CT scan also help in the diagnosis. A sample of the swelling would be taken to the lab to determine what type of lymphoma it is [10].
Treatment
Treatment of this disease involves radiotherapy and chemotherapy. The chemotherapy can be done on an out-patients basis. Surgery may also be needed for some patients like those who have the mass inside their abdomen.
References
- Tinguely M, Vonlanthen R, Müller E, et al. Hodgkin's disease-like lymphoproliferative disorders in patients with different underlying immunodeficiency states. Mod Pathol 1998; 11:307.
- Nieters A, Rohrmann S, Becker N, et al. Smoking and lymphoma risk in the European prospective investigation into cancer and nutrition. Am J Epidemiol 2008; 167:1081.
- Goldin LR, Björkholm M, Kristinsson SY, et al. Highly increased familial risks for specific lymphoma subtypes. Br J Haematol 2009; 146:91.
- Lukes R, Butler J, Hicks E. Natural history of Hodgkin's disease as related to its pathological picture. Cancer 1966; 19:317.
- von Wasielewski R, Werner M, Fischer R, et al. Lymphocyte-predominant Hodgkin's disease. An immunohistochemical analysis of 208 reviewed Hodgkin's disease cases from the German Hodgkin Study Group. Am J Pathol 1997; 150:793.
- Schmitz R, Hansmann ML, Bohle V, et al. TNFAIP3 (A20) is a tumor suppressor gene in Hodgkin lymphoma and primary mediastinal B cell lymphoma. J Exp Med 2009; 206:981.
- Horning SJ, Hoppe RT, Breslin S, Bartlett NL, Brown BW, Rosenberg SA. Stanford V and radiotherapy for locally extensive and advanced Hodgkin's disease: mature results of a prospective clinical trial. J Clin Oncol. Feb 1 2002;20(3):630-7
- Aparicio J, Segura A, Garcerá S, Oltra A, Santaballa A, Yuste A, et al. ESHAP is an active regimen for relapsing Hodgkin's disease. Ann Oncol. May 1999;10(5):593-5.
- Rummel MJ, et al. Bendamustine plus rituximab is superior in respect to progression free survival and CR rate when compared to CHOP plus rituximab as first-line treatment in patients with advanced follicular, indolent, and mantle cell lymphomas. Blood. 2009;114:405.
- Horvath B, Demeter J, Eros N, et al. Intravascular large B-cell lymphoma: Remission after rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy. J Am Acad Dermatol. Jul 24 2009