Burkitt Lymphoma (BL) Therapeutics Market -Overview
Burkitt lymphoma, or small noncleaved cell lymphoma, is a highly aggressive B-cell non-Hodgkin lymphoma (NHL) characterized by the translocation and deregulation of the c-myc gene on chromosome 8. The 2016 World Health Organization (WHO) classification of lymphoid neoplasms recognizes Burkitt-like lymphoma with 11q aberration as a new provisional entity that lacks MYC rearrangements but resembles Burkitt lymphoma morphologically, to a large extent phenotypically, and by gene expression profiling.
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Burkitt lymphoma is named after British surgeon Denis Burkitt, who first identified this unusual disease in 1956 among children in Africa. In Africa, Burkitt lymphoma is common in young children who also have malaria and Epstein-Barr, the virus that causes infectious mononucleosis. One mechanism may be that malaria weakens the immune system’s response to Epstein-Barr, allowing it to change infected B-cells into cancerous cells. About 98% of African cases are associated with Epstein-Barr infection.
The symptoms of Burkitt lymphoma depend on the type. The endemic (African) variant usually starts as tumors of the jaw or other facial bones. It also can affect the gastrointestinal tract, ovaries, and breasts and can spread to the central nervous system, causing nerve damage, weakness, and paralysis.
Intensive intravenous chemotherapy — which usually involves a hospital stay — is the preferred treatment for Burkitt lymphoma. Because Burkitt lymphoma can spread to the fluid surrounding the brain and spinal cord, chemotherapy drugs also may be injected directly into the cerebrospinal fluid, a treatment known as intrathecal chemotherapy.
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Burkitt lymphoma (BL) may be defined as an aggressive rare disorder that constitutes approximately 1% to 2% of all non-Hodgkin lymphomas. Burkitt lymphoma is known as one of the most aggressive forms of lymphoma, with a doubling time of approximately 24 hours. The term was coined after an Irish surgeon named Denis Burkitt, who first described the lymphoma in 1958, and was the first to successfully compile description of significant number of cases with the disorder. A range of intensive therapy regimen is available to choose from which depends upon local practice.
The CODOX-M/IVAC is the most commonly administered regimen, first introduced as pediatric regimen in 1980 by Magrath et.al. Various regimen for Burkitt lymphoma (BL) are R-CODOX-M/IVAC regimen, BFM/GMALL/NHL regimen, Hyper-CVAD regimen, CALGB and LMB regimen, DA-EPOCH-R regimen, and other high-intensive regimens. Burkitt lymphoma is represented by three clinical sub-types: endemic Burkitt lymphoma (eBL), immunodeficiency-associated Burkitt lymphoma (iBL), and sporadic (sBL). These clinical sub-types share similar morphological and immunophenotypic features, while conform largely with respect to genetic makeup, and differ in exhibiting few clinical features as well as incidence pattern.
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Sporadic Burkitt lymphoma (sBL) is a rare disorder generally affecting all age groups. It accounts for approximately 1% of adult lymphomas and reported in areas lacking endemic malaria. Endemic Burkitt lymphoma (eBL) is predominantly a pediatric disease or disorder, accounting for about 30% to 50% of pediatric cancer in regions with holoendemic malaria. Endemic Burkitt lymphoma (eBL) clinically differs by affecting facial bones especially the jaw. Immunodeficiency-associated Burkitt lymphoma (iBL) accounts for 10% to 40% of all HIV (Human Immunodeficiency Virus) associated lymphomas and was the first to be recognized in HIV setting.
The CODOX-M/IVAC regimen includes cytarabine, cyclophosphamide, doxorubicin, etoposide, high-dose cytarabine, IT methotrexate, leucovorin, vincristine/ifosfamide. BFM/GMALL/NHL regimen generally consists of pre-phase treatment with prednisone and cyclophosphamide, which is then commenced by alternating chemotherapy with six cycles including doxorubicin, dexamethasone, ifosfamide, high-dose methotrexate, teniposide, cytarabine, and vincristine. It also includes triple IT therapy with cytarabine, dexamethasone, and methotrexate.
Hyper-CVAD regimen which was initially developed for acute lymphoblastic leukemia includes four cycles of hyperfractionated cyclophosphamide, dexamethasone, vincristine, and doxorubicin. CALGB and LMB regimens utilize prednisone and cyclophosphamide as a pre-phase cycle, followed by alternating chemotherapy cycles depending upon the risk. DA-EPOCH-R regimen consists of different approach with low concentration chemotherapy agents being exposed for long period of time as compared to that of other high-intensive regimen, comprising infusional EPOCH (cyclophosphamide, etoposide, doxorubicin, vincristine, and prednisone). This regimen is specifically used in order to provide continuous exposure of cytotoxic agents to tumor cells and to bring about decrease in toxicity. Many variations of short-cycle, high intensive regimens are being developed and used in addition to these regimens.
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The global Burkitt lymphoma (BL) therapeutics market can be segmented based on clinical type, drug therapy regimen, distribution channel, and region. In terms of clinical type, the market can be classified into endemic BL (eBL), immunodeficiency-associated BL (idBL), and sporadic variant (sBL). Based on drug therapy regimen, the global Burkitt lymphoma (BL) therapeutics market can be categorized into R-CODOX-M/IVAC regimen, BFM/GMALL/NHL regimen, Hyper-CVAD regimen, CALGB and LMB regimen, DA-EPOCH-R, and other high-intensive regimens. In terms of distribution channel, the market can be divided into drug stores, hospital pharmacy, online pharmacy, retail pharmacy, and others.
In terms of region, the global Burkitt lymphoma (BL) therapeutics market can be segmented into Asia Pacific, Europe, Middle East & Africa, North America, and Latin America.
Key players in the global Burkitt lymphoma (BL) therapeutics market are Bedford Laboratories, Sun Pharma, Seattle Genetics, Inc., Sanofi, Mayo Clinic, Pfizer, Inc., Merck KGaA, and Cellerant Therapeutics, Inc., among others.
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