Refractory High grade B-cell lymphoma Burkitt-like lymphoma

Overview of Refractory High Grade B-cell Lymphoma Burkitt-like Lymphoma

Refractory High Grade B-Cell Lymphomas (RHBLL) is a subgroup of B-Cell non-Hodgkin’s lymphomas, a type of cancer that affects the lymphatic system. They are characterized by rapid, aggressive growth and high grade malignancy. RHBLL is further classified as part of the Burkitt-like Lymphoma (B-LL) group which includes three subtypes: Burkitt’s, Burkitt-like Acute Lymphoblastic Leukemia (ALL), and High Grade B-cell Lymphoma Burkitt-like Lymphoma (HGBL).

RHBLL subtypes share many common characteristics which include an aggressive, rapidly growing course, early systemic spread, and late nodal involvement. Patients often present with symptoms of fever, night sweats, weight loss, and swollen lymph nodes. radiographically, the disease is characterized by multiple, large, and arterial enhancement on contrast-enhanced CT scans. Histologically, the tumors are composed of large, round neoplastic cells that express B-cell markers. The hallmark of the disease is the MYC translocation, t(8;14)(q24;q32), which results in deregulation of MYC expression leading to cell proliferation.

Treatment of Refractory High Grade B-cell Lymphoma Burkitt-like Lymphoma

Treatment for RHBLL is typically initiated with a multi-drug chemotherapy regimen with a focus on intensive induction followed by consolidation. In general, regimens are chosen with drugs that have the widest spectrum of activity to target RHBLL cells. Common chemotherapy agents used in treatment include doxorubicin, vincristine, prednisone, cyclophosphamide, daunorubicin, and liposomal doxorubicin, among others. Depending on the patient’s response to chemotherapy, a bone marrow/stem cell transplant may be recommended.

Radiotherapy is used for local control of tumor bulk and in the setting of relapse or refractory disease. However, this is not a frontline therapy as radiation can be damaging to normal tissues. Additionally, targeted therapy is used in select cases to target the underlying genetic driver of the disease, namely, the MYC translocation. Drugs such as ibrutinib and venetoclax are being studied in clinical trials as potential treatments for RHBLL.

Prognosis of Refractory High Grade B-cell Lymphoma Burkitt-like Lymphoma

The overall prognosis for RHBLL is generally poor with a median survival of 6-9 months. However, response to treatment is variable and can depend on the patient’s age, comorbidities, and response to therapy. Patients who respond to induction chemotherapy have higher long-term survival rates compared to those who do not respond. Additionally, patients who receive bone marrow/stem cell transplants have higher survival rates compared to those who do not. However, the morbidity associated with transplant is high and must be weighed against potential benefit.