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Blog, Non-Hodgkin’s Lymphoma

Non-Hodgkin’s Lymphoma Treatment


The Non-Hodgkin’s Lymphoma treatment depends on many factors, including but not limited to the type of NHL, stage of the disease, patient’s age, and performance status, along with other factors. Following are the preferred treatment approaches for different stages of NHL, but the final decision is taken after clinical assessment of the patient by an oncologist.

 

NHL Category NHL Type Preferred Treatment
Indolent Lymphomas Follicular lymphoma (FL) In patients with Stage I to II, Involved-site radiotherapy (ISRT) or Immunotherapy with or without chemotherapy are the preferred treatment options.

In patients with Stage III to IV, chemoimmunotherapy is considered the standard, if there is an indication for treatment. Maintenance therapy for up to 2 years with an immunotherapeutic agent (e.g. rituximab) may be considered in patients who receive chemoimmunotherapy.

 

Lymphoplasmacytic lymphoma (LPL)/ Waldenström’s macroglobulinemia (WM) In patients with symptomatic LPL/WM, chemoimmunotherapy is considered the standard treatment. Maintenance therapy for up to 2 years with an appropriate immunotherapeutic agent (e.g. rituximab) should be considered for patients achieving good response to initial therapy.
Marginal zone lymphoma (MZL) Treatment for MZL is somewhat similar to that of FL except below cases:

In patients with gastric MALT lymphoma positive for H. pylori infection, treatment options include an appropriate antibiotic in combination with proton pump inhibitors in some cases.

In patients with non-gastric MALT lymphoma and splenic MZL, surgical excision of the involved site may be employed for confirmative diagnosis and primary treatment. Also, in splenic MZL, anti-HCV therapy may be used in HCV positive cases.

Mycosis Fungoides (MF) and Sézary Syndrome (SS) In patients with limited disease, skin-directed therapies (topical corticosteroids, topical chemotherapy, topical retinoids, topical

imiquimod, and/or phototherapy) are generally employed. Local radiotherapy may be employed for limited skin lesions.

In patients with generalized skin involvement, total skin electron beam therapy (TSEBT), or systemic chemotherapy may be employed.

In patients with blood involvement, systemic therapy with or without skin-directed therapies may be employed.

In patients with solid organ involvement, systemic therapy with or without local radiotherapy is generally employed.

Palliative treatment for pruritus and secondary infections is generally provided along with primary treatment.

Aggressive Lymphomas Diffuse large B-cell lymphoma (DLBCL) In patients with Stage I to II, chemoimmunotherapy with or without radiotherapy is considered the standard treatment.

In patients with Stage III to IV, chemoimmunotherapy is considered the standard treatment.

Autologous SCT may be considered in some patients who achieve complete response to treatment.

Grey zone lymphomas are very hard to treat as no standard treatment option is available. They are generally treated with multi-agent chemotherapy.

Burkitt lymphoma (BL) Dose-intensive, multiagent chemotherapy with or without immunotherapy along with CNS prophylaxis with intrathecal or intravenous chemotherapy is the standard treatment for patients with BL.
Primary mediastinal large B-cell lymphoma (PMBL) Chemoimmunotherapy with or without radiotherapy is employed as the first-line treatment.
Mantle cell lymphoma (MCL) In patients with Stage I to II, radiotherapy or chemoimmunotherapy is considered the standard treatment.

In patients with Stage III to IV, chemoimmunotherapy is considered the standard treatment.

Consolidative treatment with autologous SCT may also be considered in some cases.

Maintenance therapy with an immunotherapeutic agent (e.g. rituximab) may be considered in patients who receive chemoimmunotherapy.

Post-transplantation lymphoproliferative disorder (PTLD) NHL treatment generally depends upon the type of NHL. Reduction in immunosuppression (RI) is generally employed in all cases and is the only treatment required for some early-stage NHLs.

In patients with polymorphic PTLD, RI with or without immunotherapy or chemoimmunotherapy should be employed.

 

In patients with monomorphic PTLD, RI with or without immunotherapy or chemoimmunotherapy should be employed.

AIDS-Related NHLs In patients with AIDS-related NHLs, treatment for both AIDS and NHL go hand in hand. NHL treatment generally depends upon the type of NHL. In most cases, chemotherapy with or without immunotherapy is employed as the primary treatment for NHL. Treatment decision is taken depending upon CD4 cell count.
Peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS) In patients with Stage I to IV PTCL-NOS, chemotherapy is considered the standard treatment. Patients who achieve response should be followed-up closely after treatment. Consolidative treatment with autologous SCT may also be considered in some cases.
Angioimmunoblastic T-cell lymphoma (AITL) In patients with Stage I to IV AITL, chemotherapy is considered the standard treatment. Consolidative treatment with autologous SCT may also be considered in some cases.
Anaplastic large cell lymphoma (ALCL) In patients with Stage I to II ALK-positive ALCL, chemotherapy with or without ISRT is considered the standard treatment.

In patients with Stage III to IV ALK-positive ALCL, chemotherapy is considered the standard treatment.

In ALK-negative ALCL, chemotherapy with or without ISRT is considered the standard treatment.

Consolidative treatment with autologous SCT may also be considered in some cases.

In patients with breast implant-associated ALCL (BIA-ALCL), surgical excision of involved breast tissue along with the removal of breast implant is generally recommended.  Radiotherapy or systemic therapy may be employed if surgery could not remove all involved tissue, and in cases of extended disease.

Enteropathy-associated T-cell lymphoma (EATL) In patients with Stage I to IV EATL, chemotherapy is considered the standard treatment. Consolidative treatment with autologous SCT may also be considered in some cases.
Adult T-cell leukemia/lymphoma (ATLL) In patients with chronic or smoldering ATLL, observation is generally recommended until the appearance of bothering symptoms. In the case of symptomatic disease, skin-directed therapies or chemotherapy may be employed, as appropriate.

 

In patients with acute or lymphoma ATLL, chemotherapy is considered the standard treatment.

Antiviral therapy may be considered for acute ATLL.

Allogeneic SCT may be considered in eligible cases.

  Extranodal NK-/T-cell lymphoma (ENKL) In patients with the limited nasal disease, radiotherapy with or without chemotherapy is considered the standard treatment.

In patients with nasal disease. Patients achieving a response can be observed after treatment.

In patients with an advanced nasal or extranasal disease, multiagent chemotherapy (pegaspargase-based) with or without radiotherapy should be employed.

 

Following is the brief description of various Non-Hodgkin’s Lymphoma treatment:

1.Chemotherapy: Chemotherapy is the mainstay of treatment for NHL. Chemotherapy means treatment with anti-cancer drugs that kill or decrease the growth of rapidly-growing cancer cells. Certain standard combination regimen involving multiple drugs are used for the treatment of NHL, for example, R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). Many pharmaceutical companies are conducting a number of clinical trials to find out new drugs and drug-combinations with increased efficacy and specificity to target HL cells. Chemotherapy may be associated with side effects due to its effect on normal body cells apart from cancerous cells.

2.Radiation Therapy: Radiation therapy (or radiotherapy) uses high-energy x-rays or other high-energy radiations which are directed to the affected area to kill cancer cells. It is generally employed for the treatment of NHL, especially in case of bulky disease limited to a part of the body. Radiation therapy alone is recommended for the treatment of some early-stage indolent lymphomas. Sometimes, it is used as palliative therapy to relieve pain, bleeding, and obstructive problems associated with the advanced-stage disease.

3.Monoclonal Antibodies: Monoclonal antibody is a targeted therapy which can be directed to certain protein characteristic of cancer cells.  For the treatment of NHL, Rituximab (that targets CD20 protein on many types of NHL cells) is generally employed. These drugs help immune cells to destroy the cancer cells. Rituximab is generally employed for the treatment of NHL in combination with chemotherapy.

4.Stem Cell Transplant (SCT): SCT can be considered for some patients with NHL who are the good candidate for the same (younger patients in good health) and are not responding or have achieved a complete response to chemoimmunotherapy and/or radiotherapy. Following are major types of SCT techniques used for the treatment of NHL:

-Autologous SCT: In this technique, the patient’s own stem cells are first collected from the bone marrow tissue or peripheral blood (preferred nowadays). Then, the patient receives high-dose chemotherapy with or without radiation therapy to kill the lymphoma cells. The collected stem cells are re-administered to the patient which slowly replenish the blood cells in the patient body.

-Allogeneic SCT: In this technique, healthy stem cells to be administered to the patient after high dose chemotherapy are obtained from another person known as the donor. It is very important that donor is a close blood relative (preferably a sibling) so that donor cells (HLA type) closely match with the patient’s cell types.

5.Supportive Care: Supportive care is a very important component of NHL therapy and may include management of infectious complications, use of myeloid growth factors or blood product transfusions, and management of tumor lysis syndrome. Certain viral infections and bacterial infections are common among NHL patients receiving treatment. Anti-viral and antibiotic treatment with curative intent is generally employed in such cases. Additionally, patients may frequently require transfusion of blood products or growth factor treatment to maintain an appropriate count of normal blood cells. Treatment with chemotherapy and other anti-cancer agents causes NHL cells to die abruptly which releases their intracellular contents in the blood that may lead to tumor lysis syndrome characterized by sudden metabolic and electrolyte changes. Rarely, it may cause cardiac arrhythmias, acute renal failure, seizures, and even death if not managed appropriately. The management of TLS mainly involves proper hydration and controlling hyperuricemia.

It is very important to assess the benefits of each treatment option versus the possible risks and side effects before making a treatment decision. Sometimes patient’s choice and health condition are also important to make a treatment choice. Following are ultimate goals for Non-Hodgkin’s Lymphoma treatment:

  • Prolongation of life.
  • Reduction of symptoms.
  • Improvement in quality of life.

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