Burkitt's Lymphoma Overview

Study Notes

Burkitt's Lymphoma

Pathophysiology

Highly aggressive B-cell non-Hodgkin lymphoma.
Characterized by the translocation of the MYC gene, often t(8;14).
Associated with rapid cell proliferation leading to tumor growth.
Can be categorized into three forms: endemic (African), sporadic (American), and immunodeficiency-associated (HIV+).

Diagnosis Criteria

Histological Examination: Presence of monomorphic medium-sized B-cells with high mitotic activity.
Immunophenotyping: Positive for B-cell markers (CD19, CD20, CD10) and negative for CD5.
Genetic Testing: Detection of MYC gene translocation through FISH or PCR.
Imaging Studies: CT or PET scans to assess disease extent.

Treatment Options

Chemotherapy: Intensive regimens like CODOX-M/IVAC are standard.
Immunotherapy: Rituximab may be added to chemotherapy to target CD20.
Stem Cell Transplant: Considered in relapsed or high-risk cases.
Supportive Care: Management of tumor lysis syndrome and infections.

Prognosis Factors

Age: Younger patients generally have better outcomes.
Stage of Disease: Early-stage disease has a better prognosis.
Response to Treatment: Rapid and complete response to initial therapy is favorable.
CNS Involvement: Presence of central nervous system involvement indicates poorer prognosis.

Genetic Mutations

MYC gene translocation is the hallmark mutation.
Additional mutations may involve TP53 and BCL2, influencing tumor behavior and treatment response.
Genetic profiling may guide personalized therapeutic approaches.

Signs and Symptoms

Lymphadenopathy: Painless swelling of lymph nodes, especially in the abdomen or neck.
Abdominal Mass: Often presents with abdominal pain or fullness.
Systemic Symptoms: Fever, night sweats, and weight loss.
Bone Marrow Involvement: May lead to anemia, thrombocytopenia, and leukopenia.

Burkitt's Lymphoma

Pathophysiology

Highly aggressive type of B-cell non-Hodgkin lymphoma.
Defined by translocation of the MYC gene, primarily t(8;14).
Characterized by rapid cell proliferation resulting in significant tumor growth.
Can be classified into three main forms:
- Endemic (African)
- Sporadic (American)
- Immunodeficiency-associated (commonly seen in HIV+ patients)

Diagnosis Criteria

Histological Examination: Identification of monomorphic medium-sized B-cells exhibiting high mitotic activity.
Immunophenotyping: Positive expression of B-cell surface markers such as CD19, CD20, and CD10; negative for CD5.
Genetic Testing: Presence of MYC gene translocation evaluated using techniques like FISH or PCR.
Imaging Studies: Utilization of CT or PET scans to determine the extent of the disease.

Treatment Options

Chemotherapy: Utilization of intensive regimens, notably CODOX-M/IVAC, as standard treatment.
Immunotherapy: Inclusion of Rituximab in treatment plans to target CD20-positive cells.
Stem Cell Transplant: Consideration for patients with relapsed disease or those at high risk of recurrence.
Supportive Care: Implementing strategies to manage complications like tumor lysis syndrome and infections.

Prognosis Factors

Age: Generally, younger patients exhibit better treatment outcomes.
Stage of Disease: Early-stage diagnoses correlate with improved prognoses.
Response to Treatment: A rapid and complete treatment response is a positive prognostic indicator.
CNS Involvement: Central nervous system presence negatively impacts prognosis.

Genetic Mutations

MYC translocation serves as the hallmark genetic mutation associated with Burkitt's lymphoma.
Additional mutations may include TP53 and BCL2, affecting tumor behavior and influencing treatment efficacy.
Genetic profiling aids in tailoring personalized therapeutic strategies for patients.

Signs and Symptoms

Lymphadenopathy: Characterized by painless swelling of lymph nodes, predominantly in the abdomen or neck.
Abdominal Mass: Patients often present with abdominal pain or a sensation of fullness.
Systemic Symptoms: Common manifestations include fever, night sweats, and unintentional weight loss.
Bone Marrow Involvement: Leads to hematological issues such as anemia, thrombocytopenia, and leukopenia.

Description

This quiz covers the pathophysiology, diagnosis criteria, and treatment options for Burkitt's Lymphoma, a highly aggressive B-cell non-Hodgkin lymphoma. It delves into the genetic characteristics, histological findings, and various therapeutic approaches available for this disease.