Last updated date: 03-Mar-2023
Originally Written in English
Malignant Lymphoma is a kind of blood and lymph malignancy caused by lymphocytes. In modern usage, the term generally refers to just the malignant variants of such tumors, rather than all such tumors. Enlarged lymph nodes, fever, drenching sweats, unintentional weight loss, itching, and fatigue are all possible signs and symptoms. Usually, swollen lymph nodes are painless. Sweating is especially prevalent at night.
What is Malignant lymphoma?
Malignant lymphoma is a kind of cancer that affects lymphocytes, which are white blood cells that are part of the immune system. Lymphocyte abnormalities lead them to expand uncontrolled, resulting in tumor formation. This also prevents them from fulfilling their role of protecting the body from sickness and infection.
Because lymph nodes are situated throughout the body, lymphoma can begin anywhere. Tumors can spread throughout the body and harm the organs, leading to the development of other types of cancer, such as leukemia.
There are several subtypes of malignant lymphoma. Lymphomas are classified into two types:
- Non-Hodgkin lymphoma (NHL) (90% of cases) and
- Hodgkin lymphoma (HL) (10%).
HL is further subdivided into classical and non-classical varieties, whereas NHL is subdivided into B-cell, T-cell, and natural killer (NK) cell types. Lymphoma is classified clinically as aggressive (high grade) or indolent (low grade).
Although they both come from lymphoid tissues, their pathophysiology differs significantly. If a type of aberrant cell known as a Reed-Sternberg cell is seen, the lymphoma is categorized as Hodgkin If the aforementioned cell is absent, the lymphoma is characterized as non- Hodgkin B-cell lymphoma and follicular lymphoma are examples of this kind of lymphoma.
Is Malignant lymphoma a common cancer?
From 2009 to 2013, the incidence of lymphoma in the United States was 22/ 100,000, accounting for roughly 5% of all malignancies; it doubled between 1970 and 1990 and has been steady thereafter. The average age upon diagnosis is 63. At five years, overall survival is predicted to be 72 percent, and it is improving.
Different stresses, including as infectious, inflammatory, and toxic stimuli, interact in a complicated way with the genetic composition of the human host to cause lymphomagenesis. Being on long-term immunosuppressive medications, which make the innate immune system less capable of detecting and destroying cancer cells or warding off infections that might lead to cancer, is one of the commonly acknowledged principles of lymphomagenesis.
Malignant Lymphoma Causes
Different environmental, infectious, and genetic factors have been identified, which predispose to Malignant lymphoma.
- Occupational exposure: herbicides, pesticides
- Infectious organisms: Helicobacter pylori (MALT lymphoma), Borrelia burgdorferi, Chlamydia psittaci, Campylobacter jejuni, human T-cell lymphotropic virus (adult T-cell leukemia/lymphoma), hepatitis C (lymphoplasmacytic lymphoma, diffuse large B-cell lymphoma, and marginal zone lymphoma) (primary effusion lymphoma and Castleman disease). Lymphoma formation is also increased by chronic activation of lymphoid tissue. Persistent infection with viruses such as Epstein Barr virus and CMV also predisposes to lymphoma development.
- Immunodeficiency: Those with HIV, transplant recipients, and genetic immunodeficiency diseases (severe combined immunodeficiency and common variable immunodeficiency).
- Drugs: Tumor necrosis factor-alpha inhibitors have been linked to T-cell lymphoma in particular. Chronic immunosuppression raises the likelihood of lymphoma in post-transplant patients (both solid organ transplant and bone marrow transplant recipients).
- Autoimmune diseases: Inflammatory bowel disease (enteropathy associated lymphoma), rheumatoid arthritis and, Sjögren’s syndrome (diffuse large B-cell lymphoma)
- Geographic location: Extranodal NK/T- cell lymphoma incidence is high in Southern Asia and some parts of Latin America.
NHLs are cancers that develop from lymphoid tissues, primarily lymph nodes. Each of the biological components of antigen-stimulated lymphoid follicles corresponds to a different neoplastic tumor cell line.
NHL is defined as a gradual clonal growth of B cells, T cells, and/or NK cells caused by an accumulation of lesions affecting proto-oncogenes or tumor suppressor genes, which results in cell immortalization. These oncogenes can be triggered by chromosomal translocations (the genetic characteristic of lymphoid malignancies), or tumor suppressor sites can be inactivated through chromosomal deletion or mutation.
Furthermore, several oncogenic viruses can modify the genome of some lymphoma subtypes by introducing foreign genes. Several chromosomal abnormalities are linked to certain NHLs, indicating the existence of unique diagnostic markers in subclassifying diverse NHL subtypes.
Almost 85 percent of NHLs are B-cell-derived; just 15 % are T/NK-cell-derived, and the remaining are macrophage-derived. These tumors are distinguished by their amount of differentiation, the size of the cell of origin, the rate of proliferation of the origin cell, and the histologic pattern of growth.
The pattern of growth and cell size may be key factors of tumor aggressiveness in several B-cell NHL subtypes. Tumors that proliferate in a nodular pattern, which resembles normal B-cell lymphoid follicular structures, are less aggressive than lymphomas that proliferate in a diffuse form. Small lymphocyte lymphomas normally progress more slowly than big lymphocyte lymphomas, which can be intermediate- or high-grade aggressive. Small cell morphology, on the other hand, distinguishes several subgroups of high-grade lymphomas.
Malignant Lymphoma symptoms
- Hodgkin Lymphoma
Painless, superficial enlarged lymphadenopathy that includes adjacent lymph node chains in a predictable manner as the illness spreads. Hematogenous spread with vascular invasion happens later in the disease's course. Only 3-7 % of individuals have isolated infra-diaphragmatic illness, while the majority of patients have supradiaphragmatic disease.
In the absence of symptoms, 60 to 70 % of patients have cervical and/or supraclavicular lymphadenopathy, 30 percent have axillary disease, and 50 to 60 percent have mediastinal involvement observed on radiography. Although para-aortic lymph nodes are involved in infra diaphragmatic illness, abdominal organ involvement is infrequent. Only 10-15% of HL patients develop extranodal illness, with bone, bone marrow, lung, and liver being the most often afflicted organs. Although CNS involvement is uncommon, para-aortic adenopathy that extends into the epidural space might cause neurological symptoms.
Approximately 25% of previously undetected HL patients show systemic symptoms before to the onset of lymphadenopathy, known as B symptoms (fevers, drenching night sweats, and unintentional weight loss). The occurrence of these symptoms is associated with a poor prognosis in both early and advanced stages of illness. Other symptoms include severe pruritis and alcohol-induced discomfort that occurs minutes after drinking alcohol and is localized to lymphadenopathy areas. Cerebellar degeneration and stiff-person syndrome are two rare neurological syndromes that have been reported.
- Non-Hodgkin Lymphoma
The most prevalent complaint is a symptomatic, expanding lymph nodal mass, either centrally or peripherally situated. Twenty percent of patients have stage I illness, around 40% have disease localized to one side of the diaphragm (stage II), another 20% have involvement above and below the diaphragm, and 40% have extensive disease with extranodal involvement.
The lung, liver, kidney, and bone marrow are common sites of extranodal spread. In contrast, the gastrointestinal tract, thyroid, bone, brain, testis, kidney, liver, breast, and skin are the predominant extranodal sites of genesis for Diffuse large B-cell lymphoma (DLBCL). Around 30% of patients will exhibit B symptoms as well as less specific symptoms such as malaise and tiredness.
- Follicular Lymphoma
The most typical manifestation is subacute or chronic asymptomatic peripheral lymphadenopathy, which can last for years or wax and wane. Lymphadenopathy in the abdomen, pelvis, or retroperitoneum can be bulky without presenting gastrointestinal or genitourinary symptoms, and nodal masses are not locally invasive and destructive. The lymph nodes are not involved in an organized fashion, and early hematogenous spread is prevalent. B-symptoms are found in fewer than 20% of individuals and should suggest consideration for DLBCL transformation.
- Marginal Zone Lymphoma
Symptoms associated to the locations of involvement (stomach, lung, ocular adnexa, breast, thyroid, intestine, skin, and soft tissue) and B-symptoms are uncommon and should raise the possibility of transformed lymphoma.
- Mantle Cell Lymphoma
Seventy to ninety percent of individuals have detectable stage 4 illness. In certain series, bone marrow involvement is widespread, and a leukemic phase is found as frequently as 75% of the time. The gastrointestinal tract is commonly implicated and might show with symptoms ranging from widespread lymphomatous polyposis to a normal lumen with microscopic illness seen on biopsy. The spleen and Waldyer's ring are two more typical sites of involvement.
Endemic Burkitt Lymphoma (BL) manifests in children as a tumor of the jaw or facial bones and extends hematogenously to extranodal organs such as the kidney, testis or ovary, CNS, or meninges early in the course. Sporadic BL manifests as a bloated abdominal condition affecting the stomach, caecum, or small intestine, with ascites. Kidneys, ovarian or testicular organs, the CNS, or the meninges are frequently involved. Immunodeficiency-related BL often manifests as lymph node involvement, although it can also include the bone marrow, CNS, or meninges and, in rare cases, progress to leukemia.
Malignant lymphoma Diagnosis
Following confirmation of lymphoma by tissue biopsy, subsequent examination entails determining the tissue with the highest disease activity. PET/CT scans that evaluate the uptake of radiolabeled fluorodeoxyglucose (FDG) are used to assess lymphoma biological activity. Staging is done prior to the start of lymphoma treatment.
The Ann Arbor staging approach is used for both HL and NHL clinical staging. The presence or absence of B symptoms (chronic fever, weight loss of more than 10% of body weight over six months, or night sweats) is considered in lymphoma staging. Lactate dehydrogenase, complete blood counts with differential, a comprehensive metabolic panel, and uric acid are all included in the blood work.
Whole-body PET/CT imaging is preferred over CT of the chest, abdomen, and pelvis. Bone marrow biopsy is often performed for staging but may be omitted for stage III DLBCL and HL because detection of stage IV disease over stage III does not change the treatment. In some cases where lymphoma is judged to be high risk, standard staging is supplemented by cerebrospinal fluid testing.
Malignant lymphoma Management
The treatment you have depends on the type and subtype of lymphoma that you have. This can vary widely.
Other things taken into consideration include:
- Overall health, age
- Fitness status
- Goals of treatment
- Personal preferences
If you have very slow-growing lymphomas, called indolent lymphoma, and aren’t having symptoms, you may be simply monitored.
Hodgkin's Lymphoma Treatment
The vast majority of Hodgkin's lymphoma patients may be cured. If you have just been diagnosed, your treatment may include both radiation and chemotherapy, or only chemotherapy. ABVD is an abbreviation for one of the most regularly utilized chemotherapy regimens for HL.
- Adriamycin (doxorubicin)
- Blenoxane (bleomycin)
- Velban or Alkaban-AQ (vinblastine)
- DTIC-Dome (dacarbazine)
If you have relapsed or unresponsive HL, your treatment may include chemotherapy, radiotherapy, new medicines, and stem cell transplantation. Immunotherapy and clinical trial participation may potentially be options.
Non-Hodgkin's Lymphoma Treatment
For those with aggressive B-cell NHL, chemotherapy is often recommended, in a regimen called R-CHOP, which includes:
- Rituxan (rituximab)
- Neosar (cyclophosphamide)
- Adriamycin or Rubex (doxorubicin)
- Oncovin or Vincasar Pfs (vincristine sulfate)
- Deltasone, Liquid Pred, Meticorten, and Orasone (prednisone)
You may also receive alternative treatments like as radiation or surgery. Treatment options include targeted medicines, immunotherapy, CAR T-cell therapy, stem cell transplantation, and radiotherapy.
The prognosis for lymphoma is determined by the kind of lymphoma, as well as its stage and other prognostic variables. Other factors, such as your age and overall health, might also influence your prognosis.
Overall, the five-year survival rate for HL is 87 %. This indicates that persons diagnosed with HL are around 87 % more likely than those who do not have the diagnosis to live five years following diagnosis.
If you break the five-year survival rate for HL down by stage, it looks like this:
- Localized (one lymph node/one organ outside lymph system/one lymphoid organ): 92%
- Regional (spread to one organ near a lymph node area/two or more lymph node areas on the same side of diaphragm/bulk disease): 94%
- Distant (in bone marrow/spread to distant parts of the body/spread to above and below the diaphragm): 82%
Because there are so many distinct forms of NHL, the five-year survival rate varies. The total five-year relative survival rate for NHL is 73%. Because prognosis is affected by so many individual circumstances and illness features, discuss your prognosis with your treatment team.
When Should You See an Oncologist?
Oncologists are doctors that specialize in cancer treatment. If you have any of the symptoms of lymphoma for an extended length of time, such as longer than two weeks, you should consult your doctor. You will be sent to an oncologist if it has been determined that the tumors are malignant.
A medical oncologist specializes in cancer therapy and has specific skill in developing the best treatment plan for you. Oncologists may also specialize in areas such as radiation oncology, which treats patients who require radiation therapy, and pediatric oncology, which treats children with cancer.
Lymphoma is a collection of malignant lymphocyte neoplasms with over 90 subtypes. Non-Hodgkin or Hodgkin lymphoma is the most common classification. Tobacco use and obesity are significant modifiable risk factors, with genetic, viral, and inflammatory etiologies all playing a role.
Malignant lymphoma often manifests as painless adenopathy, with systemic symptoms such as fever, unexplained weight loss, and night sweats appearing later in the disease's progression. For diagnosis, an open lymph node biopsy is preferable. To stage lymphoma, the Lugano classification system integrates symptoms and the extent of the illness as indicated by positron emission tomography/computed tomography, which is then used to select therapy.
Chemotherapy treatment plans differ between the main subtypes of lymphoma. Non-Hodgkin lymphoma is treated with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) with or without rituximab (R-CHOP), bendamustine, and lenalidomide. Hodgkin lymphoma is treated with combined chemotherapy with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine), Stanford V (a chemotherapy regimen consisting of mechlorethamine, doxorubicin, vinblastine, vincristine, bleomycin, etoposide, and prednisone) with radiotherapy.
Subsequent chemotherapy toxicities include neuropathy, cardiotoxicity, and secondary cancers such as lung and breast, and should be considered in the shared decision-making process to select a treatment regimen. Once remission is achieved, patients need routine surveillance to monitor for complications and relapse, in addition to age-appropriate screenings recommended by the U.S.