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What is Diffuse Large B-Cell Lymphoma?

Diffuse large B-cell lymphoma (DLBCL) is the most common histologic subtype of non-Hodgkin lymphoma (NHL) accounting for approximately 25 percent of NHL cases.

Diffuse Large B-Cell lymphoma is the most common lymphoid neoplasm and accounts for approximately 25 percent of all NHLs.

Like most other NHLs, there is a male predominance. Incidence increases with age; the median age at presentation is 64 years for patients as a whole, but appears to be younger for Blacks than for Caucasian Americans. There does appear to be familial aggregation of patients with DLBCL and other non-Hodgkin lymphoma subtypes.  

 

Pathogenesis of Diffuse Large B-Cell Lymphoma?

 

DLBCL arises from a mature B-cell, usually a centroblast or immunoblast. The molecular pathogenesis of DLBCL is complex and includes both genetic lesions that are relatively specific for this disease (ie, rearrangements of BCL-6) and molecular alterations that are shared with other NHL variants. The pathogenesis of DLBCL is discussed in more detail separately.

Patients with DLBCL typically present with a rapidly enlarging symptomatic mass, most usually nodal enlargement in the neck or abdomen. Systemic "B" symptoms (ie, fever, weight loss, drenching night sweats) are observed in approximately 30 percent of patients, and the serum LDH is elevated in over one-half.

Extranodal extramedullary disease occurs in up to 40 percent of cases.  The most common site of extranodal involvement is the stomach/gastrointestinal trac, but the disease can arise in virtually any tissue, including the testis, bone, thyroid, salivary glands, tonsil, skin, liver, breast, adrenals, kidneys, nasal cavity, paranasal sinuses, uterine cervix, and central nervous system.

 

Prognosis of Diffuse Large B Cell Lymphoma:

 

Prognosis in DLBCL is highly associated with the International Prognostic Index (IPI) score, which was proposed in 1993 to assign prognosis to patients with aggressive non-Hodgkin lymphoma undergoing treatment with doxorubicin containing chemotherapeutic regimens. These findings have been confirmed in many series of patients with DLBCL and a revised version is available for patients treated with Rituximab-containing regimens.
 
Original IPI — 
 

Age >60

Serum lactate dehydrogenase (LDH) concentration greater than normal

ECOG performance status ≥2

Ann Arbor clinical stage III or IV

Number of involved extranodal disease sites >1
 
In this system, one point is given for each of the above characteristics present in the patient, for a total score ranging from zero to five, representing increasing degrees of risk
 

· Low risk — IPI score of zero or one

· Low intermediate risk — IPI score of two

· High intermediate risk — IPI score of three

· High risk — IPI score of four or five
 

At a median observation time exceeding 30 months, three-year estimates for event-free (EFS), progression-free (PFS), and overall (OS) survival as divided by IPI score were:

 

IPI score zero or one — rates of 81, 87, and 91 percent, respectively.

IPI score two — rates of 69, 75, and 81 percent, respectively.

IPI score three — rates of 53, 59, and 65 percent, respectively.

IPI score four or five — rates of 50, 56, and 59 percent, respectively.

 

Treatment of Diffuse Large B-Cell Lymphoma:

 

The initial treatment of DLBCL is dependent upon the extent of disease. This staging system focuses on the number of tumor sites (nodal and extranodal), location, and the presence or absence of systemic ("B") symptoms.  For treatment purposes, patients with DLBCL are generally classified as having either limited stage disease or advanced stage disease based upon whether or not the tumor can be contained within one irradiation field:

· Limited stage disease (usually Ann Arbor stage I or II) — Limited stage DLBCL can be contained within one irradiation field. This population accounts for 30 to 40 percent of patients with DLBCL. Limited stage DLBCL is treated primarily with combined modality therapy consisting of abbreviated systemic chemotherapy (three cycles), the recombinant           anti-CD20 antibody rituximab, and involved field radiation therapy. Alternatively, full course (six to eight cycles) systemic chemotherapy plus rituximab without radiation therapy may be used.

· Advanced stage disease (usually Ann Arbor stage III or IV) — Advanced stage DLBCL cannot be contained within one irradiation field. This population accounts for 60 to 70 percent of patients with DLBCL. Advanced stage DLBCL is treated primarily with systemic chemotherapy plus the recombinant anti-CD20 antibody rituximab.

Patients with bulky (>10 cm) stage II disease and patients with stage IIB disease have a less favorable prognosis than those with non-bulky stage II disease without systemic B symptoms. Many clinicians treat such patients in a similar fashion to those with advanced stage disease.

Chemotherapy regimen referred to as R-CHOP with cyclophosphamide, doxorubicin, vincristine, and prednisone as well we rituximab is the standard of care for diffuse large B cell lymphoma.

Here Dr. Tony Talebi discusses “What is diffuse large B cell lymphoma?” with Dr. Ann Mohrbacher, associate professor of medicine at the University of Southern California.  The discussion includes lymphoma prognosis, what causes lymphoma, signs of lymphoma, lymphoma symptoms, lymphoma, lymphoma stage 4, treatment of lymphoma, lymphoma association, stage four lymphoma, small cell lymphoma, symtoms of lymphoma, causes of lymphoma, lymphoma chemotherapy, what is lymphoma, lymphomas, lymphoma information, lymphoma prevention, stage 4 lymphoma, information about lymphoma, stage iv lymphoma, lymphoma signs, lymphoma symptom, is lymphoma curable, stage 3b lymphoma, lungs cancer symptoms, survival rates for lymphoma, symptoms of lymphoma, lymphoma survivors, lymphoma symptons, lymphoma survival, treatments of lymphoma, symptons of lymphoma, lymphoma statistics, non small cell lymphoma, chemo for lymphoma, lymphoma survival rate, large cell lymphoma, effects of lymphoma, lymphoma screening, lymphoma diagnosis, lymphoma society, lymphoma clinical trials, lymphoma metastasis, survival rate lymphoma, symptom of lymphoma, info on lymphoma, new treatments for lymphoma, how common is lymphoma.
 
 
 
 
 
 
 
 
Background:

Dr. Mohrbacher, a graduate of Harvard Medical School, is a devoted educator, researcher and medical practitioner. She has several hematology related research interests and activities, including bone marrow transplantation, and has received thousands of dollars in research grants. She has been published in a number of medical journals and has been invited to lecture on topics including Radioimmunotherapy of non-Hodgkin’s lymphoma and B-cell targeted therapy of rheumatoid arthritis. Dr. Mohrbacher is a member of the Southern California Lymphoma Group and currently belongs to a number of university related committees, such as the Post-Graduate Education Committee and the Clinical Investigations Committee.

Education:

  • Harvard and Radcliffe Colleges, Boston, MA, B.A., 1983
  • Doctor of Medicine, Harvard Medical School, Boston, MA, 1987

Internships:

  • Brigham and Women's Hospital, Boston, MA, 1983 - 1984

Residencies:

  • Brigham and Women's Hospital (Internal Medicine), Boston, MA, 1987 - 1990

Fellowships:

  • Clinical Medicine Fellow, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, 1990 - 1993
  • Clinical Oncology and Tumor Immunology Fellow, Dana-Farber Cancer Institute, Boston, MA, 1990 - 1993

Board Certification:

  • Medical Oncology
  • Internal Medicine

Professional Society Memberships:

  • Massachusetts Medical Society, 1985
  • American Society of Hematology, 1994
  • Southwest Oncology Group, 1994

Awards:

  • A.B. Magna Cum Laude, 1983
  • Harvard College Scholarship, 1983
  • ASCO career Development Award, 1995
  • Dr. Mohrbacher was voted a Top Doctor by Pasadena Magazine in 2011.