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Non-Hodgkin's lymphoma

  1. Incidence. Non-Hodgkin's lymphomas (espe­cially CNS lymphomas) are more common in patients with acquired immunodefi­ciency states and in patients receiving immunosuppressive drugs, such as renal and heart transplant patients.
  2. Etiology
    1. Cytogenetic abnormalities, such as chromosome translocations, are commonly ob­ served in lymphoma cells.
    2. Viral infection
      1. The Epstein-Barr virus (LQ 2012) has been linked to Burkitt's lymphoma.
      2. An aggressive T-cell leukemia or lymphoma is associated with human T Lymphotropic virus type I (HTLV-I) infec­tion.
  3. Pathology. histologic classification literature
Classification of the Morphological Subgroups of Non-Hodgkin's Lymphomas
  1. Low-grade malignant lymphoma
    1. Small lymphocytic CLL
    2. Follicular-predominantly small-cleaved cell                                                                                                                             
    3. Follicular-mixed (small cleaved and large cell)
  2. Intermediate-grade malignant lymphoma
    1. Follicular-predominantly large cell
    2. Diffuse-small-cleaved cell
    3. Diffuse-mixed (small and large cell)
    4. Diffuse-large cell
  3. High-grade malignant lymphoma
    Large cell
    1. Convoluted lymphoblastic ALL
    2. Small noncleaved cell (Burkitt's)
Extra Edge: Important translocation to be remembered
  1. Clinical features
    1. Most patients are asymptomatic. Or may have fever, night sweats, or weight loss.
    2. Patients with indolent lymphomas may have waxing and waning adenopathy for several months before diagnosis, although persistent nodal enlargement is more common.
    3. Extranodal disease most often involve the stomach, lung, and bone, resulting in symptoms characteristic of the affected organ. Involvement of Oropharyngeal lymphoid tissue (Waldeyer Ring) causes sore throat and obstructive breathing.
    4. If bone marrow is involve that lead to pancytopenia.
    5. Skin is involve in T cell lymphoma. 
  2. Staging and diagnosis
Table - International Prognostic Index for NHL
Five clinical risk factors:
  Age ³60 years
  Serum lactate dehydrogenase levels elevated
  Performance status ³2 (ECOG) or £70 (Karnofsky)
  Ann Arbor stage III or IV
  >1 site of extranodal involvement
Patients are assigned a number for each risk factor they have
Patients are grouped differently based upon the type of lymphoma
For diffuse large B cell lymphoma:
  0, 1 factor = low risk: 35% of cases; 5-year survival, 73%
  2 factors = low-intermediate risk: 27% of cases; 5-year survival, 51%
  3 factors = high-intermediate risk: 22% of cases; 5-year survival, 43%
  4, 5 factors = high risk: 16% of cases; 5-year survival, 26%
For diffuse large B cell lymphoma treated with R-CHOP:
  0 factor = very good: 10% of cases; 5-year survival, 94%
  1, 2 factors = good: 45% of cases; 5-year survival, 79%
  3, 4, 5 factors = poor: 45% of cases; 5-year survival, 55%
  1. Therapy
    1. Radiation therapy. Non-Hodgkin's lymphomas are very radiosensitive.
      1. In localized disease, radiation should be targeted to the affected site (4000cGy to the involved field). The nodal site and draining lymphatics should be included in the radiation field.
      2. Radiation therapy is used palliatively in disseminated disease or to "consoli­date" a complete response to chemotherapy in areas of bulky disease.
      3. Electron beam therapy has been used in the management of cutaneous lym­phomas, such as the early stages of mycosis fungoides.
    2. Chemotherapy
      1. Low-grade indolent lymphomas may not require treatment for many years. When therapy is indicated, chlorambucil or cyclophosphamide, with or with­out prednisone, is the agent of choice.
      2. Stage I or II intermediate and high-grade lymphomas often respond to combi­nation chemotherapy, with or without radiation therapy. Cure rates approach 80%-90%.
        The anti-CD20 antibody, rituximab, is useful in relapsed patients, as is the nucleoside analog, fludarabine.
      3. Aggressive intermediate or high-grade lymphomas (e.g., lymphoblastic or Bur­kitt's lymphoma) require immediate combination chemotherapy. Prophylactic intrathecal chemotherapy may also be given. Salvage combination chemother­apy produces second complete or partial remissions, but is rarely curative un­less the patient undergoes bone marrow transplantation.
        1. Common regimens include CHOP (cyclophosphamide, doxorubicin, vin­cristine, and prednisone), PROMACE-CytaBOM (cyclophosphamide, doxo­rubicin, etoposide, prednisone, cytarabine, bleomycin, vincristine, metho­trexate, and leukovorin), and MACOP-B (methotrexate, leukovorin, doxorubicin, cyclophosphamide, vincristine, bleomycin, and prednisone).
        2. Colony-stimulating factors hasten granulocyte recovery and may permit higher doses and better cure rates. 
Recent Advance - New Drug
Ibritumomab: It is useful in the treatment of relapsed or refractory low-grade, follicular or transformed B-cell NHL (Non-Hodgkin’s lymphoma).
Clinical differences between Hodgkin and non Hodgkin lymphomas
Hodgkin lymphoma Non-Hodgkin lymphoma
More often localized to a single axial group of nodes (cervical, mediastinal, para-aortic) More frequent involvement of multiple peripheral nodes
Orderly spread by contiguity Noncontiguous spread
Mesenteric nodes and Waldeyer ring rarely involved Mesenteric nodes and Waldeyer ring commonly involved
Extranodal involvement uncommon Extranodal involvement common
Burkitt lymphoma  is a cancer of the lymphatic system (in particular, B lymphocytes).


Burkitt's lymphoma is associated with c-myc gene translocation. This gene is found at 8q24.
The most common variant is t(8;14)(q24;q32). 

Types of Burkitt’s Lymphoma
TYPE                                         AGE                         SEX                          SITE ( most common to least common)
a. Endemic                             8-15                        M>>>F                   Face, abdomen, CNS, LN,BM
b. Sporadic                             8-15                       M>>>F                   abdomen > face
c. AIDS associated                Young Adults        M=F                        LN,BM

PBF -  Starry sky appearance
Highly proliferative lymphoma-  Doubling time – 60 hours ( most rapid)

Malignant B cell characteristics

  1. Normal B cells possess rearranged immunoglobulin heavy and light chain genes and each isolated B-cell possesses a unique IgH gene rearrangement.
  2. Since Burkitt lymphoma and other B-cell lymphomas are a clonal proliferative process, all tumor cells from one patient are supposed to possess identical IgH genes.
  3. When the DNA of tumor cells is analyzed using electrophoresis, a clonal band can be demonstrated since identical IgH genes will move to the same position.


  1. Cyclophosphamide                                 
  2. Doxorubicin                                     
  3. Vincristine                                        
  4. Methotrexate                                          
  5. Cytarabine                                       
  6. Ifosfamide
  7. Etoposide                                                 
  8. Rituximab
Other treatments are immunotherapy, bone marrow transplants, surgery to remove the tumor, and radiotherapy.

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