ICD-10: C83.76

Burkitt lymphoma, classified under ICD-10 code C83.76, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. It primarily affects B-lymphocytes and is often associated with a chromosomal translocation involving the MYC gene. The treatment for Burkitt lymphoma, particularly when it involves intrapelvic lymph nodes, typically follows a multi-faceted approach that includes chemotherapy, immunotherapy, and sometimes radiation therapy.

Standard Treatment Approaches

1. Chemotherapy

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

• CODOX-M/IVAC: This is a combination chemotherapy regimen that includes Cyclophosphamide, Vincristine, Doxorubicin (Adriamycin), and Methotrexate (CODOX-M), followed by Ifosfamide, Etoposide, and Cytarabine (IVAC). This regimen is designed to achieve rapid tumor reduction and is often administered in cycles.

• Hyper-CVAD: Another effective regimen is Hyper-CVAD, which consists of alternating cycles of Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone (CVAD) with high-dose Methotrexate and Cytarabine. This approach is particularly beneficial for patients with high tumor burden or those presenting with central nervous system involvement.

2. Immunotherapy

Immunotherapy has emerged as a significant adjunct to traditional chemotherapy. The use of monoclonal antibodies, such as Rituximab, can enhance the effectiveness of chemotherapy by targeting CD20-positive B-cells. While Rituximab is more commonly associated with other types of B-cell lymphomas, its role in Burkitt lymphoma is being explored, especially in cases where the disease is refractory to standard chemotherapy.

3. Radiation Therapy

Radiation therapy is not typically the primary treatment for Burkitt lymphoma due to its aggressive nature and the systemic approach required. However, it may be utilized in specific scenarios, such as:

• Palliative Care: For patients with localized disease or those experiencing significant symptoms from bulky disease, radiation can provide symptomatic relief.

• Consolidation Therapy: In some cases, radiation may be used after chemotherapy to target residual disease, particularly if there are concerns about local control.

4. Stem Cell Transplantation

For patients with 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 tolerated otherwise, with the goal of eradicating the disease.

5. Clinical Trials

Given the aggressive nature of Burkitt lymphoma and the ongoing research in the field, participation in clinical trials may be an option for patients. These trials often explore novel therapies, including targeted agents and new combinations of existing treatments.

Conclusion

The treatment of Burkitt lymphoma, particularly when it involves intrapelvic lymph nodes, is complex and requires a multidisciplinary approach. The standard treatment primarily revolves around intensive chemotherapy regimens, with potential roles for immunotherapy and radiation in specific contexts. Given the aggressive nature of this lymphoma, timely diagnosis and treatment initiation are crucial for improving patient outcomes. As research continues, new therapies and combinations are likely to emerge, offering hope for better management of this challenging disease.

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. The ICD-10 code C83.76 specifically refers to Burkitt lymphoma that is located in the intrapelvic lymph nodes. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Burkitt Lymphoma

Overview

Burkitt lymphoma is a type of B-cell lymphoma that is classified as a high-grade malignancy. It is most commonly seen in children and young adults, although it can occur at any age. The disease is associated with a chromosomal translocation involving the MYC gene, which leads to uncontrolled cell growth.

Types of Burkitt Lymphoma

There are three main types of Burkitt lymphoma:
1. Endemic Burkitt Lymphoma: Commonly found in Africa, often associated with Epstein-Barr virus (EBV) infection, and typically presents as a jaw lesion in children.
2. Sporadic Burkitt Lymphoma: More prevalent in the United States and Europe, this form usually presents as an abdominal mass.
3. Immunodeficiency-associated Burkitt Lymphoma: Occurs in individuals with HIV/AIDS or other immunocompromised states.

Symptoms

Patients with Burkitt lymphoma may present with various symptoms, including:
- Swollen lymph nodes, particularly in the abdomen or pelvis.
- Abdominal pain or discomfort.
- Fever, night sweats, and weight loss (B symptoms).
- Intestinal obstruction or perforation if the lymphoma involves the gastrointestinal tract.

Diagnosis

Diagnosis typically involves:
- Imaging Studies: CT scans or MRIs to assess lymph node involvement and staging.
- Biopsy: A tissue sample from the affected lymph nodes is essential for histological examination and confirmation of Burkitt lymphoma.
- Immunophenotyping: Flow cytometry may be used to identify specific cell markers associated with B-cell lymphomas.

Intrapelvic Lymph Nodes Involvement

The designation of C83.76 indicates that the lymphoma is specifically affecting the intrapelvic lymph nodes. This can lead to:
- Pelvic Mass Effect: Compression of surrounding structures, potentially causing urinary or gastrointestinal symptoms.
- Lymphadenopathy: Enlarged lymph nodes in the pelvic region, which may be palpable on physical examination.

Treatment

The treatment for Burkitt lymphoma is aggressive and typically includes:
- Chemotherapy: Intensive multi-agent chemotherapy regimens are the standard of care, often including drugs such as cyclophosphamide, doxorubicin, vincristine, and methotrexate.
- Immunotherapy: In some cases, monoclonal antibodies may be used to target specific cancer cells.
- Stem Cell Transplant: For relapsed or refractory cases, hematopoietic stem cell transplantation may be considered.

Prognosis

The prognosis for patients with Burkitt lymphoma can be favorable with early diagnosis and aggressive treatment. The overall survival rate varies based on factors such as age, stage at diagnosis, and response to therapy.

Conclusion

ICD-10 code C83.76 identifies Burkitt lymphoma localized to the intrapelvic lymph nodes, a condition that requires prompt diagnosis and treatment due to its aggressive nature. Understanding the clinical features, diagnostic methods, and treatment options is crucial for managing this type of lymphoma effectively. Early intervention can significantly improve outcomes for affected patients.

Burkitt lymphoma, particularly when it involves intrapelvic lymph nodes, presents with a range of clinical features, signs, and symptoms that are critical for diagnosis and management. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with ICD-10 code C83.76, which specifically refers to Burkitt lymphoma affecting the intrapelvic lymph nodes.

Clinical Presentation

Overview of Burkitt Lymphoma

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. It is most commonly seen in children and young adults but can also occur in adults. The disease is often associated with the presence of the MYC gene rearrangement, which plays a significant role in its pathogenesis[6].

Signs and Symptoms

The clinical presentation of Burkitt lymphoma can vary based on the location of the disease, but when it involves the intrapelvic lymph nodes, the following signs and symptoms may be observed:

• Abdominal Pain or Discomfort: Patients may experience pain in the lower abdomen due to the enlargement of lymph nodes in the pelvic region, which can exert pressure on surrounding structures[6].
• Swelling or Mass Effect: Palpable abdominal masses may be noted during physical examination, particularly in the lower abdomen, indicating lymphadenopathy[6].
• B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many lymphomas, including Burkitt lymphoma[6].
• Gastrointestinal Symptoms: Patients may present with nausea, vomiting, or changes in bowel habits if the lymphoma affects the gastrointestinal tract or causes obstruction[6].
• Lymphadenopathy: Generalized lymphadenopathy may be present, with specific enlargement of pelvic lymph nodes being a hallmark of this subtype[6].

Patient Characteristics

Burkitt lymphoma has distinct demographic and clinical characteristics:

• Age: It predominantly affects children and young adults, with a peak incidence in the age group of 5 to 10 years. However, it can also occur in adults, particularly in immunocompromised individuals[6].
• Gender: There is a slight male predominance in the incidence of Burkitt lymphoma[6].
• Immunocompromised Status: Patients with compromised immune systems, such as those with HIV/AIDS, are at a higher risk for developing Burkitt lymphoma[6].
• Geographic Variability: The endemic form of Burkitt lymphoma is more common in Africa, often associated with malaria, while the sporadic form is more prevalent in Western countries[6].

Conclusion

Burkitt lymphoma, particularly when involving intrapelvic lymph nodes, presents with a range of symptoms that can significantly impact patient quality of life. Early recognition of these signs and symptoms is crucial for timely diagnosis and treatment. Given its aggressive nature, prompt intervention is essential to improve outcomes for affected individuals. Understanding the patient characteristics and clinical presentation can aid healthcare providers in making informed decisions regarding diagnosis and management strategies for this challenging malignancy.

Burkitt lymphoma, particularly when it involves the intrapelvic lymph nodes, is classified under the ICD-10 code C83.76. This specific code falls within the broader category of non-Hodgkin lymphoma, which encompasses various types of lymphatic cancers. Below are alternative names and related terms associated with this condition:

Alternative Names for Burkitt Lymphoma

1. Burkitt's Lymphoma: This is a common variation in spelling and is often used interchangeably with Burkitt lymphoma.
2. Endemic Burkitt Lymphoma: This form is typically associated with malaria-endemic regions and is characterized by a specific genetic mutation.
3. Sporadic Burkitt Lymphoma: This variant occurs more commonly in non-endemic areas and is not linked to malaria.
4. AIDS-related Burkitt Lymphoma: This type is associated with HIV infection and is more aggressive in nature.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): Burkitt lymphoma is classified as a type of non-Hodgkin lymphoma, which includes a diverse group of blood cancers.
2. Lymphoproliferative Disorders: This term encompasses a range of conditions, including lymphomas, where there is an abnormal proliferation of lymphocytes.
3. B-cell Lymphoma: Burkitt lymphoma is a subtype of B-cell lymphoma, indicating that it originates from B-lymphocytes.
4. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with Burkitt lymphoma.
5. C83.7: This is the broader ICD-10 code for Burkitt lymphoma, which includes various sites of involvement, not limited to the intrapelvic lymph nodes.

Conclusion

Understanding the alternative names and related terms for Burkitt lymphoma, particularly in the context of intrapelvic lymph nodes, is essential for accurate diagnosis, treatment planning, and coding in medical records. This knowledge aids healthcare professionals in communicating effectively about the condition and ensures that patients receive appropriate care based on their specific type of lymphoma.

Burkitt lymphoma, classified under ICD-10 code C83.76, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. The diagnosis of Burkitt lymphoma, particularly when it involves intrapelvic lymph nodes, relies on a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria and methods used for diagnosis:

Clinical Presentation

1. Symptoms: Patients may present with symptoms such as:
   - Swollen lymph nodes, particularly in the abdomen or pelvis.
   - Abdominal pain or discomfort.
   - Fever, night sweats, and weight loss (B symptoms).
   - Gastrointestinal symptoms if the lymphoma affects the intestines.

2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy or splenomegaly, which are common in lymphoma cases.

Imaging Studies

1. CT Scans: Computed tomography (CT) scans of the abdomen and pelvis are crucial for visualizing enlarged lymph nodes and assessing the extent of the disease. They help in identifying the location and size of lymph nodes involved.

2. PET Scans: Positron emission tomography (PET) scans may be used to evaluate metabolic activity in lymph nodes and other tissues, aiding in the differentiation between benign and malignant processes.

Histopathological Examination

1. Biopsy: A definitive diagnosis of Burkitt lymphoma requires a biopsy of the affected lymph node or tissue. The biopsy can be performed through:
   - Excisional biopsy: Removal of an entire lymph node.
   - Core needle biopsy: Removal of a small core of tissue.

2. Microscopic Analysis: Pathological examination of the biopsy specimen is performed to identify characteristic features, including:
   - High proliferation index (often >95% Ki-67 positivity).
   - Presence of atypical lymphoid cells with a starry sky appearance due to interspersed macrophages.

3. Immunophenotyping: Flow cytometry or immunohistochemistry is used to determine the cell surface markers. Burkitt lymphoma typically expresses:
   - CD19, CD20, and CD10.
   - It is usually negative for CD5 and CD23, which helps differentiate it from other types of lymphoma.

4. Genetic Testing: The presence of the MYC gene rearrangement is a hallmark of Burkitt lymphoma. This can be identified through techniques such as fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR).

Conclusion

The diagnosis of Burkitt lymphoma, particularly when it involves intrapelvic lymph nodes, is a multifaceted process that combines clinical assessment, imaging studies, and detailed histopathological analysis. The integration of these diagnostic criteria is essential for accurate identification and subsequent management of this aggressive lymphoma type. If you have further questions or need more specific information, feel free to ask!