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Lymphoma

Lymphatic Cancer

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.

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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.

Splenomegaly
  • A 65-year-old patient was diagnosed with DLBCL stage IIa with splenomegaly, transformed from a NLPHL, after a 30-year history of repeatedly enlarged inguinal lymph nodes. The patient initially rejected chemotherapy.[ncbi.nlm.nih.gov]
  • 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 observed in approximately 40% of patients; the spleen is rarely the only involved site at presentation.[emedicine.medscape.com]
Generalized Lymphadenopathy
  • When more than one area of lymph nodes is swollen it’s called generalized lymphadenopathy .[m.cancer.org]
Nasal Congestion
  • A chronic lymphocytic leukemia patient presented with nasal congestion. Nasal endoscopy identified a left nasal mass, and the pathology suggested extranodal NK/T-cell lymphoma (nasal type). The patient received a course of chemotherapy.[ncbi.nlm.nih.gov]
Fever
  • There was no history of fever, trauma, skin redness, or nipple discharge. The patient had a history of breast augmentation, with retropectoral silicone implants placed 2.5 years ago.[ncbi.nlm.nih.gov]
  • He does well during his first cycle, but shortly after develops a fever and suffers from violent chills. His wife drives him to the hospital.[chemotherapy.com]
  • The most common symptoms of lymphoma are: painless, swollen lymph nodes in the neck, groin or underarm tiredness night sweats fever itchy skin weight loss loss of appetite frequent infections bleeding or bruising easily swollen abdomen, as a result of[lymphoma.canceraustralia.gov.au]
  • Other signs and symptoms that could be specific to a subtype of lymphoma are outlined in the table below: Symptom General Location in the Body Painless lymph node enlargement 2cm most common often in the neck, underarm or groin Fevers, night sweats, tiredness[lymphoma.org.au]
  • While receiving stem cells, patients may experience pain, chills, fever, hives, chest pain or other symptoms.[hopkinsmedicine.org]
Weight Loss
  • Some Common Symptoms of Lymphoma can include: Swollen lymph nodes Fatigue Unexplained weight loss Night sweats Itching Our latest community service announcment highlights the most common signs and symptoms.[lymphoma.org.au]
  • Statistics provided by: Lymphoma Coalition 2014 Global Patient Survey (3,500 Respondents) Common Signs and Symptoms Painless swelling in a lymph node Chills/temperature swings Recurrent f evers and e xcessive sweating at night Unintentional weight loss[lymphomacoalition.org]
  • The most common symptoms of lymphoma are: painless, swollen lymph nodes in the neck, groin or underarm tiredness night sweats fever itchy skin weight loss loss of appetite frequent infections bleeding or bruising easily swollen abdomen, as a result of[lymphoma.canceraustralia.gov.au]
  • Symptoms appear at an advanced stage of the disease and can include fever, night sweats and weight loss. At diagnosis, patients usually present with adenopathy and, in 50% of cases, splenomegaly.[orpha.net]
  • In addition to swollen lymph nodes, symptoms may include fever and itching followed later by weight loss and fatigue. A microscopic examination of affected tissue, usually obtained from a lymph node , is required to confirm diagnosis .[britannica.com]
Hodgkin's Disease
  • Etiology of Hodgkin’s Disease Causes of Hodgkin’s disease To date, the etiology of Hodgkin’s disease is not fully understood.[lecturio.com]
  • A prognostic score for advanced Hodgkin's disease. International Prognostic Factors Project on Advanced Hodgkin's Disease. N Engl J Med . 1998 Nov 19. 339(21):1506-14. [Medline] . [Full Text] .[emedicine.medscape.com]
  • Hodgkin’s Disease The usual treatment for most patients with early stage Hodgkin’s disease is high-energy radiation of the lymph nodes.[healthcentral.com]
  • Most people who get Hodgkin's disease will survive for many years after diagnosis. That's why the goal of Hodgkin's disease is usually to cure the cancer. Treatment is determined by the stage and other specific characteristics of the cancer.[chemotherapy.com]
  • 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]
Chills
  • Statistics provided by: Lymphoma Coalition 2014 Global Patient Survey (3,500 Respondents) Common Signs and Symptoms Painless swelling in a lymph node Chills/temperature swings Recurrent f evers and e xcessive sweating at night Unintentional weight loss[lymphomacoalition.org]
  • While receiving stem cells, patients may experience pain, chills, fever, hives, chest pain or other symptoms.[hopkinsmedicine.org]
  • Symptoms of Non-Hodgkin's lymphoma include swollen lymph nodes, fever, night sweats, chills, fatigue, unexplained weight loss, and facial swelling.[cancercenter.com]
  • 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]
  • He does well during his first cycle, but shortly after develops a fever and suffers from violent chills. His wife drives him to the hospital.[chemotherapy.com]
Abdominal Mass
  • Patients with Burkitt lymphoma (occurring in the United States) often present with a large abdominal mass and symptoms of bowel obstruction.[emedicine.medscape.com]
  • 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]
  • 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]
  • Intermediate- and high-grade lymphomas may result in the following examination findings: Rapidly growing and bulky lymphadenopathy Splenomegaly Hepatomegaly Large abdominal mass: Usually in Burkitt lymphoma Testicular mass Skin lesions: Associated with[emedicine.medscape.com]
Hepatomegaly
  • Hepatomegaly. Intermediate- and high-grade lymphomas: Most patients present with rapidly growing and bulky lymphadenopathy.[patient.info]
  • 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]
  • Diagnosis Examination in patients with low-grade lymphomas may demonstrate peripheral adenopathy, splenomegaly, and hepatomegaly.[emedicine.medscape.com]
  • […] inguinal area (groin, 6-20%) Involvement of the Waldeyer ring (back of the throat, including the tonsils) or occipital (lower rear of the head) or epitrochlear (inside the upper arm near the elbow) areas is infrequently observed Splenomegaly and/or hepatomegaly[emedicine.medscape.com]
Retinal Lesion
  • We report an unusual case of primary central nervous system lymphoma (PCNSL) presenting as migrating retinal lesions with unique shapes. The diagnostic challenges are described and the clinical features of intraocular lymphoma are reviewed.[ncbi.nlm.nih.gov]
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]
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]

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]
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:

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].

Sex distribution
Age distribution

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

Article

  1. 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.
  2. 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.
  3. Goldin LR, Björkholm M, Kristinsson SY, et al. Highly increased familial risks for specific lymphoma subtypes. Br J Haematol 2009; 146:91.
  4. Lukes R, Butler J, Hicks E. Natural history of Hodgkin's disease as related to its pathological picture. Cancer 1966; 19:317.
  5. 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.
  6. 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.
  7. 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
  8. 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.
  9. 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.
  10. 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

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