ICD-10: C83.71

Burkitt lymphoma, classified under the ICD-10-CM code C83.71, is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. This specific code pertains to cases where the lymphoma primarily affects the lymph nodes located in the head, face, and neck regions.

Clinical Description of Burkitt Lymphoma

Overview

Burkitt lymphoma is a type of B-cell lymphoma that is known for its fast growth and is often associated with the Epstein-Barr virus (EBV). It is most commonly seen in children and young adults, although it can occur at any age. The disease is categorized into three main types: endemic (African), sporadic (non-African), and immunodeficiency-associated, with the endemic form being more prevalent in regions where malaria is common.

Symptoms

Patients with Burkitt lymphoma may present with various symptoms, including:

• Swollen Lymph Nodes: The most common initial presentation is the enlargement of lymph nodes, particularly in the head, face, and neck area, which can lead to visible swelling.
• Abdominal Pain: In cases where the lymphoma involves abdominal organs, patients may experience pain or discomfort.
• Fever and Night Sweats: Systemic symptoms such as fever, night sweats, and weight loss may also be present.
• Fatigue: Generalized fatigue and weakness are common due to the disease's aggressive nature and the body's response to the malignancy.

Diagnosis

Diagnosis of Burkitt lymphoma typically involves:

• Histopathological Examination: A biopsy of the affected lymph nodes is essential for confirming the diagnosis. The histological features include a high proliferation index and the presence of starry sky appearance due to interspersed macrophages.
• Immunophenotyping: Flow cytometry is used to identify the specific B-cell markers (CD19, CD20, CD10) that characterize Burkitt lymphoma.
• Cytogenetic Analysis: The presence of the MYC gene rearrangement is a hallmark of Burkitt lymphoma, often involving translocation t(8;14).

Treatment

The treatment for Burkitt lymphoma is aggressive and typically includes:

• Chemotherapy: Intensive multi-agent chemotherapy regimens are the cornerstone of treatment. Common regimens include the use of cyclophosphamide, doxorubicin, vincristine, and methotrexate.
• Supportive Care: Due to the aggressive nature of the disease and the side effects of treatment, supportive care is crucial, including management of tumor lysis syndrome and infections.

Prognosis

The prognosis for patients with Burkitt lymphoma can be favorable with early diagnosis and aggressive treatment. The overall survival rates are significantly improved with appropriate therapy, although the disease's rapid progression necessitates prompt intervention.

Conclusion

ICD-10 code C83.71 specifically identifies Burkitt lymphoma affecting the lymph nodes of the head, face, and neck, highlighting the need for timely diagnosis and treatment. Understanding the clinical presentation, diagnostic criteria, and treatment options is essential for healthcare providers managing patients with this aggressive lymphoma type. Early intervention can lead to better outcomes, underscoring the importance of awareness and education regarding this condition.

Burkitt lymphoma, classified under ICD-10 code C83.71, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid tumor growth. This lymphoma primarily affects lymphoid tissues, including lymph nodes, and can present with a variety of clinical features. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with Burkitt lymphoma is crucial for timely diagnosis and management.

Clinical Presentation

Common Signs and Symptoms

1. Lymphadenopathy: The most prominent feature of Burkitt lymphoma is lymphadenopathy, particularly in the head, face, and neck regions. Patients may present with swollen lymph nodes that are firm and non-tender[1].

2. Facial Swelling: Due to the involvement of lymph nodes in the head and neck, patients often exhibit noticeable facial swelling, which can lead to asymmetry[1].

3. Abdominal Symptoms: In some cases, Burkitt lymphoma may also involve abdominal lymph nodes, leading to symptoms such as abdominal pain, distension, or a palpable mass[1].

4. Systemic Symptoms: Patients may experience systemic symptoms such as fever, night sweats, and unexplained weight loss, which are common in many lymphomas[1].

5. Bone Marrow Involvement: If the disease progresses, it can affect the bone marrow, leading to symptoms of anemia (fatigue, pallor), thrombocytopenia (easy bruising or bleeding), and leukopenia (increased susceptibility to infections)[1].

Specific Characteristics

• Age Group: Burkitt lymphoma is most commonly diagnosed in children and young adults, particularly those aged 5 to 25 years. However, it can occur in older adults as well[1].
• Gender: There is a slight male predominance in the incidence of Burkitt lymphoma, with males being affected more frequently than females[1].

Diagnostic Considerations

Histological Features

Burkitt lymphoma is characterized histologically by a high proliferation index, often assessed using Ki-67 staining, which is typically greater than 90% in this type of lymphoma. The presence of "starry sky" appearance due to interspersed macrophages is a classic histological finding[1].

Imaging Studies

Imaging studies, such as CT or MRI scans, are essential for assessing the extent of lymphadenopathy and any potential involvement of other organs. These imaging modalities help in staging the disease and planning treatment[3].

Conclusion

Burkitt lymphoma, particularly when affecting the lymph nodes of the head, face, and neck, presents with distinctive clinical features that necessitate prompt recognition and intervention. The combination of lymphadenopathy, facial swelling, and systemic symptoms, along with the demographic characteristics of affected patients, plays a critical role in the diagnosis and management of this aggressive lymphoma. Early detection and treatment are vital for improving patient outcomes, given the rapid progression associated with this disease.

Burkitt lymphoma, classified under ICD-10 code C83.71, specifically refers to a type of non-Hodgkin lymphoma that primarily affects the lymph nodes in the head, face, and neck regions. This aggressive form of lymphoma is characterized by rapid cell growth and is most commonly seen in children and young adults. Below are alternative names and related terms associated with this condition.

Alternative Names for Burkitt Lymphoma

1. Endemic Burkitt Lymphoma: This variant is commonly found in Africa and is often associated with Epstein-Barr virus (EBV) infection. It typically presents as a jaw lesion in children.

2. Sporadic Burkitt Lymphoma: This form occurs worldwide and is not linked to EBV. It usually presents as an abdominal mass or lymphadenopathy.

3. Immunodeficiency-associated Burkitt Lymphoma: This variant occurs in individuals with compromised immune systems, such as those with HIV/AIDS.

4. Burkitt's Lymphoma: A common shorthand for the condition, often used interchangeably with the full name.

5. B-cell Lymphoma: Since Burkitt lymphoma arises from B-cells, it may be referred to in the context of B-cell malignancies.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): Burkitt lymphoma is a subtype of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers.

2. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with Burkitt lymphoma.

3. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes Burkitt lymphoma as a specific type.

4. C83.7: This is the broader ICD-10 code category for Burkitt lymphoma, which includes various subtypes and locations.

5. CD20 Positive Lymphoma: Burkitt lymphoma is typically characterized by the expression of the CD20 antigen, which is relevant for targeted therapies.

6. MYC Gene Rearrangement: Burkitt lymphoma is often associated with chromosomal translocations involving the MYC oncogene, which is a key factor in its pathogenesis.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.71 is crucial for healthcare professionals involved in the diagnosis and treatment of Burkitt lymphoma. This knowledge aids in accurate coding, effective communication among medical teams, and enhances patient care by ensuring that all aspects of the disease are considered. If you need further information on treatment options or clinical guidelines related to Burkitt lymphoma, feel free to ask!

Burkitt lymphoma, classified under ICD-10 code C83.71, is a highly aggressive form of non-Hodgkin lymphoma that primarily affects lymphoid tissue. The diagnosis of Burkitt lymphoma, particularly when it involves the lymph nodes of the head, face, and neck, is based on a combination of clinical, histological, and cytogenetic criteria. Below is a detailed overview of the criteria used for diagnosis.

Clinical Presentation

Symptoms

Patients with Burkitt lymphoma may present with:
- Swollen Lymph Nodes: Enlarged lymph nodes in the head, face, and neck region are common.
- Systemic Symptoms: Fever, night sweats, and weight loss may occur, although they are less common in Burkitt lymphoma compared to other lymphomas.
- Abdominal Symptoms: In cases where the abdomen is involved, patients may experience pain or swelling due to lymphadenopathy or organ involvement.

Physical Examination

A thorough physical examination is essential to assess the extent of lymphadenopathy and any associated symptoms. The presence of rapidly enlarging masses in the head and neck region is a significant indicator.

Histological Criteria

Biopsy

A definitive diagnosis of Burkitt lymphoma requires a tissue biopsy. The histological examination typically reveals:
- High-Grade Lymphoma: The presence of a high proliferation index, often assessed using Ki-67 staining, which is usually greater than 90%.
- Cell Morphology: The characteristic appearance of the lymphoma cells, which are typically medium-sized with a high nuclear-to-cytoplasmic ratio and prominent nucleoli.

Immunophenotyping

Immunohistochemical staining is performed to identify specific markers:
- B-cell Markers: Positive for CD19, CD20, and CD10.
- Negative for T-cell Markers: Typically negative for CD3 and CD5.

Cytogenetic Criteria

Genetic Testing

Cytogenetic analysis is crucial for confirming the diagnosis:
- MYC Gene Rearrangement: The hallmark of Burkitt lymphoma is the translocation involving the MYC gene, most commonly t(8;14), which juxtaposes the MYC oncogene with the immunoglobulin heavy chain locus.
- Additional Chromosomal Abnormalities: Other translocations may also be present, but the MYC translocation is definitive for diagnosis.

Imaging Studies

Radiological Assessment

Imaging studies, such as CT or MRI scans, may be utilized to evaluate the extent of disease:
- Lymph Node Involvement: Imaging can help visualize enlarged lymph nodes and any potential extranodal involvement, particularly in the abdomen or central nervous system.

Conclusion

The diagnosis of Burkitt lymphoma (ICD-10 code C83.71) involves a multifaceted approach that includes clinical evaluation, histological examination, immunophenotyping, cytogenetic analysis, and imaging studies. The combination of these criteria ensures an accurate diagnosis, which is critical for determining the appropriate treatment strategy. If you have further questions or need additional information on this topic, feel free to ask!

Burkitt lymphoma, classified under ICD-10 code C83.71, is a highly aggressive form of non-Hodgkin lymphoma that primarily affects lymphoid tissue, including the lymph nodes of the head, face, and neck. The treatment for Burkitt lymphoma typically involves a combination of chemotherapy, targeted therapy, and sometimes radiation therapy, depending on the specific characteristics of the disease and the patient's overall health.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy is the cornerstone of treatment for Burkitt lymphoma. The most commonly used regimens include:

• CODOX-M/IVAC: This is a combination regimen that includes Cyclophosphamide, Vincristine, Doxorubicin (Adriamycin), Methotrexate, and Ifosfamide, followed by high-dose Cytarabine and Etoposide. This regimen is designed to deliver intensive treatment over a short period, which is crucial given the aggressive nature of the disease[1].

• R-CHOP: While not the standard for Burkitt lymphoma, some patients may receive R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) if they have features that overlap with other types of non-Hodgkin lymphoma. However, R-CHOP is generally less effective for Burkitt lymphoma compared to more intensive regimens[2].

2. Targeted Therapy

Rituximab, a monoclonal antibody that targets the CD20 protein on B-cells, is often included in treatment regimens for Burkitt lymphoma. It can enhance the effectiveness of chemotherapy by specifically targeting malignant B-cells[3].

3. Radiation Therapy

Radiation therapy may be used in specific cases, particularly if there is localized disease or if the lymphoma is causing significant symptoms due to mass effect. However, it is not typically the primary treatment modality for Burkitt lymphoma, as systemic therapy is preferred due to the disease's aggressive nature[4].

4. Stem Cell Transplantation

In cases of relapsed or refractory Burkitt lymphoma, high-dose chemotherapy followed by autologous stem cell transplantation may be considered. This approach allows for the administration of higher doses of chemotherapy than would be tolerable without the support of stem cell rescue[5].

5. Clinical Trials

Given the aggressive nature of Burkitt lymphoma, patients are often encouraged to consider participation in clinical trials. These trials may offer access to novel therapies or combinations that are not yet widely available but could provide additional options for treatment[6].

Conclusion

The treatment of Burkitt lymphoma, particularly in the lymph nodes of the head, face, and neck, is complex and requires a multidisciplinary approach. The standard treatment typically involves intensive chemotherapy regimens, often supplemented with targeted therapies like Rituximab. Radiation therapy may be utilized in select cases, and stem cell transplantation is an option for relapsed disease. Given the rapid progression of this lymphoma type, timely diagnosis and treatment initiation are critical for improving patient outcomes. For the most current treatment options, consulting with a healthcare provider or oncologist specializing in hematologic malignancies is essential.