ICD-10: C83.7

Burkitt lymphoma, classified under ICD-10 code C83.7, is a highly aggressive form of non-Hodgkin lymphoma (NHL) characterized by the rapid proliferation of B-lymphoid cells. This lymphoma is notable for its association with specific genetic abnormalities, particularly the translocation of the MYC oncogene, which plays a crucial role in cell growth and division.

Clinical Features

Epidemiology

Burkitt lymphoma is most commonly diagnosed in children and young adults, although it can occur at any age. It is particularly prevalent in certain regions of Africa, where it is often associated with endemic forms linked to malaria and Epstein-Barr virus (EBV) infection. In non-endemic forms, it can occur sporadically and is more frequently seen in immunocompromised individuals, such as those with HIV/AIDS[2][5].

Symptoms

Patients with Burkitt lymphoma may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, often in the abdomen, neck, or jaw.
- Abdominal Pain: Due to the involvement of the gastrointestinal tract or retroperitoneal lymph nodes.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many lymphomas.
- Bone Marrow Involvement: This can lead to cytopenias, resulting in fatigue, increased susceptibility to infections, and bleeding tendencies[1][4].

Diagnosis

Diagnosis of Burkitt lymphoma typically involves:
- Histopathological Examination: A biopsy of affected tissue is essential for diagnosis, revealing a high proliferation index and characteristic starry sky appearance due to interspersed macrophages.
- Immunophenotyping: Flow cytometry or immunohistochemistry is used to confirm the B-cell lineage and assess the expression of surface markers such as CD19, CD20, and CD10.
- Cytogenetic Analysis: Detection of MYC gene translocation (most commonly t(8;14)) is a hallmark of Burkitt lymphoma and aids in confirming the diagnosis[3][6].

Treatment

The treatment of Burkitt lymphoma is aggressive and typically involves:
- Chemotherapy: Intensive multi-agent chemotherapy regimens, such as the CODOX-M/IVAC protocol, are commonly employed. These regimens aim to achieve rapid tumor reduction and are often followed by central nervous system prophylaxis due to the risk of CNS involvement.
- Stem Cell Transplantation: In cases of relapsed or refractory disease, autologous or allogeneic stem cell transplantation may be considered[2][5].

Prognosis

The prognosis for patients with Burkitt lymphoma can be favorable with early diagnosis and appropriate treatment. The overall survival rates can exceed 80% in children and young adults, but outcomes may vary based on factors such as age, stage at diagnosis, and response to initial therapy[1][4].

Conclusion

Burkitt lymphoma, designated by ICD-10 code C83.7, is a highly aggressive B-cell malignancy that requires prompt diagnosis and treatment. Understanding its clinical features, diagnostic criteria, and treatment options is crucial for improving patient outcomes. Ongoing research into targeted therapies and the biology of Burkitt lymphoma continues to evolve, offering hope for better management strategies in the future.

Burkitt lymphoma, classified under ICD-10 code C83.7, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid tumor growth. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for timely diagnosis and management.

Clinical Presentation

Types of Burkitt Lymphoma

Burkitt lymphoma can be categorized into three main types:
1. Endemic Burkitt Lymphoma: Commonly found in African children, often associated with Epstein-Barr virus (EBV) infection. It typically presents as a jaw lesion.
2. Sporadic Burkitt Lymphoma: More prevalent in the United States and Europe, this form usually presents with abdominal masses.
3. Immunodeficiency-associated Burkitt Lymphoma: Occurs in individuals with compromised immune systems, such as those with HIV/AIDS.

Signs and Symptoms

The clinical signs and symptoms of Burkitt lymphoma can vary based on the type and location of the disease:

• Abdominal Pain and Swelling: Often due to large abdominal masses, which may cause discomfort or distension[1].
• Lymphadenopathy: Swollen lymph nodes, particularly in the neck, abdomen, or groin, are common[2].
• Fever and Night Sweats: Patients may experience systemic symptoms such as fever, drenching night sweats, and unexplained weight loss, indicative of lymphoma[3].
• Jaw Lesions: In endemic cases, jaw swelling or lesions are a hallmark sign, particularly in children[4].
• Bone Marrow Involvement: Symptoms may include fatigue, weakness, and increased susceptibility to infections due to bone marrow infiltration[5].
• Tumor Lysis Syndrome: This potentially life-threatening condition can occur due to rapid cell turnover, leading to metabolic abnormalities[6].

Patient Characteristics

Demographics

• Age: Burkitt lymphoma predominantly affects children and young adults, with a peak incidence in those aged 5 to 10 years for endemic cases. Sporadic cases are more common in adolescents and young adults[7].
• Gender: There is a slight male predominance in both endemic and sporadic forms of Burkitt lymphoma[8].

Risk Factors

• Infectious Agents: The association with EBV is significant, particularly in endemic cases. Immunocompromised individuals, such as those with HIV, are at higher risk for developing Burkitt lymphoma[9].
• Geographic Location: Endemic Burkitt lymphoma is more common in equatorial Africa, while sporadic cases are found worldwide, with higher incidence rates in developed countries[10].

Clinical Course

Burkitt lymphoma is known for its rapid progression, often requiring immediate treatment upon diagnosis. The aggressive nature of the disease necessitates a multidisciplinary approach to management, including chemotherapy and supportive care to prevent complications such as tumor lysis syndrome[11].

Conclusion

Burkitt lymphoma, represented by ICD-10 code C83.7, is a highly aggressive lymphoma with distinct clinical presentations based on its type. Recognizing the signs and symptoms, along with understanding patient demographics and risk factors, is essential for effective diagnosis and treatment. Early intervention is critical due to the rapid progression of the disease, highlighting the importance of awareness among healthcare providers.

Burkitt lymphoma, classified under the ICD-10 code C83.7, is a type of non-Hodgkin lymphoma characterized by the rapid growth of tumors, often in the abdomen or lymph nodes. Understanding the alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below are some of the key alternative names and related terms associated with Burkitt lymphoma.

Alternative Names for Burkitt Lymphoma

1. Burkitt's Lymphoma: This is a common variation in naming, often used interchangeably with Burkitt lymphoma.
2. Endemic Burkitt Lymphoma: This term refers to the form of Burkitt lymphoma that is commonly found in Africa, often associated with malaria and Epstein-Barr virus (EBV) infection.
3. Sporadic Burkitt Lymphoma: This variant occurs outside of endemic regions and is more prevalent in Western countries. It typically presents with abdominal masses.
4. AIDS-related Burkitt Lymphoma: This term is used when Burkitt lymphoma occurs in patients with HIV/AIDS, highlighting its association with immunocompromised states.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): Burkitt lymphoma is a subtype of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers that include various lymphomas.
2. Lymphoproliferative Disorders: This broader category includes conditions like Burkitt lymphoma, where there is an abnormal proliferation of lymphocytes.
3. B-cell Lymphoma: Burkitt lymphoma is classified as a B-cell lymphoma, indicating that it originates from B-lymphocytes, a type of white blood cell.
4. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes Burkitt lymphoma as a specific type.
5. C83.7: The specific ICD-10 code for Burkitt lymphoma, which is essential for medical billing and coding.

Conclusion

Understanding the alternative names and related terms for Burkitt lymphoma is crucial for healthcare professionals, researchers, and patients alike. These terms not only facilitate better communication but also enhance the accuracy of medical records and treatment plans. If you have further questions or need more detailed information about Burkitt lymphoma or its classifications, feel free to ask!

Burkitt lymphoma, classified under ICD-10 code C83.7, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. The diagnosis of Burkitt lymphoma involves a combination of clinical evaluation, histopathological examination, and specific laboratory tests. Below are the key criteria used for its diagnosis:

Clinical Presentation

1. Symptoms: Patients often present with symptoms such as:
   - Swollen lymph nodes (lymphadenopathy)
   - Abdominal pain or swelling due to enlarged organs (hepatosplenomegaly)
   - Fever, night sweats, and weight loss (B symptoms) which are common in lymphomas[4].

2. Age and Demographics: Burkitt lymphoma has distinct age-related peaks, commonly affecting children and young adults, but it can also occur in older adults[6].

Histopathological Criteria

1. Tissue Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph node or tissue. The histopathological examination typically reveals:
   - A high proliferation index, often assessed by Ki-67 staining, which is usually greater than 90%[4].
   - A characteristic "starry sky" appearance due to the presence of numerous macrophages engulfing apoptotic cells and debris[4].

2. Cell Type: The lymphoma is composed predominantly of B-lymphoid cells, which can be confirmed through immunohistochemistry. The cells typically express:
   - CD19, CD20, and CD10 markers, while being negative for other markers such as CD5 and CD23, which helps differentiate it from other types of lymphomas[4].

Genetic and Molecular Criteria

1. Cytogenetic Analysis: The presence of specific chromosomal abnormalities is crucial for diagnosis:
   - The most common genetic alteration in Burkitt lymphoma is the translocation involving the MYC gene, typically t(8;14), which leads to the overexpression of the MYC oncogene[4][5].

2. Molecular Testing: Fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) can be used to detect MYC rearrangements and confirm the diagnosis[4].

Imaging Studies

1. Radiological Assessment: Imaging studies such as CT scans or PET scans are often employed to assess the extent of the disease and to identify any extranodal involvement, which is common in Burkitt lymphoma[4].

Conclusion

The diagnosis of Burkitt lymphoma (ICD-10 code C83.7) is multifaceted, relying on clinical presentation, histopathological findings, genetic testing, and imaging studies. Early and accurate diagnosis is critical due to the aggressive nature of this lymphoma, which necessitates prompt treatment. If you have further questions or need more detailed information on specific aspects of Burkitt lymphoma, feel free to ask!

Burkitt lymphoma, classified under ICD-10 code C83.7, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid tumor growth. Understanding the standard treatment approaches for this condition is crucial for effective management and improved patient outcomes. Below, we explore the treatment modalities typically employed for Burkitt lymphoma.

Overview of Burkitt Lymphoma

Burkitt lymphoma is categorized into three main types: endemic (African), sporadic (non-endemic), and immunodeficiency-associated. Each type may present differently and can influence treatment decisions. The disease is marked by the translocation of the MYC gene, leading to uncontrolled cell proliferation, and it often presents with a high tumor burden at diagnosis[2].

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains the cornerstone of treatment for Burkitt lymphoma. The aggressive nature of the disease necessitates intensive chemotherapy regimens. Commonly used regimens include:

• CODOX-M/IVAC: This regimen combines cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate (CODOX-M), followed by ifosfamide, etoposide, and cytarabine (IVAC). This approach is often used for both pediatric and adult patients due to its efficacy in achieving remission[3].

• R-CHOP: While not the first-line treatment, the R-CHOP regimen (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) may be considered in certain cases, particularly for patients with less aggressive forms or those who cannot tolerate more intensive regimens[4].

2. Targeted Therapy

Targeted therapies are increasingly being integrated into treatment protocols. Rituximab, a monoclonal antibody targeting CD20, is sometimes used in combination with chemotherapy to enhance treatment efficacy, especially in cases where the lymphoma expresses CD20[5].

3. Stem Cell Transplantation

For patients with relapsed or refractory Burkitt lymphoma, autologous stem cell transplantation (ASCT) may be considered. This approach involves harvesting the patient's stem cells, administering high-dose chemotherapy to eradicate the lymphoma, and then reinfusing the stem cells to restore bone marrow function[6].

4. Radiation Therapy

Radiation therapy is not typically a primary treatment for Burkitt lymphoma but may be used in specific scenarios, such as localized disease or to manage complications like bulky disease or symptomatic masses. It can also be employed as a palliative measure in advanced cases[7].

Considerations and Prognosis

The prognosis for Burkitt lymphoma can be favorable with timely and appropriate treatment, with overall survival rates significantly improved through intensive chemotherapy regimens. However, the aggressive nature of the disease means that early diagnosis and intervention are critical. Factors such as age, performance status, and the presence of central nervous system involvement can influence treatment decisions and outcomes[8].

Conclusion

In summary, the standard treatment approaches for Burkitt lymphoma (ICD-10 code C83.7) primarily involve intensive chemotherapy regimens, with options for targeted therapy and stem cell transplantation in specific cases. The management of this aggressive lymphoma requires a multidisciplinary approach to optimize patient outcomes and address the unique challenges posed by the disease. Continuous advancements in treatment protocols and supportive care are essential for improving survival rates and quality of life for affected individuals.