ICD-10: C83.72

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. The ICD-10-CM code C83.72 specifically refers to Burkitt lymphoma that is localized in the intrathoracic lymph nodes. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Burkitt Lymphoma

Overview

Burkitt lymphoma is classified as a type of B-cell lymphoma and is known for its fast growth rate. It is most commonly seen in children and young adults, although it can occur at any age. The disease is associated with chromosomal translocations, particularly involving the MYC gene, which plays a crucial role in cell proliferation and 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 often presents as an abdominal mass.
3. Immunodeficiency-associated Burkitt Lymphoma: Occurs in individuals with compromised immune systems, such as those with HIV/AIDS.

Symptoms

Patients with Burkitt lymphoma may present with various symptoms, including:
- Swollen lymph nodes, particularly in the neck, abdomen, or groin.
- Abdominal pain or swelling due to enlarged lymph nodes or masses.
- Fever, night sweats, and unexplained weight loss.
- Fatigue and weakness.

Intrathoracic Involvement

When Burkitt lymphoma affects the intrathoracic lymph nodes, it can lead to specific symptoms related to thoracic structures, such as:
- Coughing or difficulty breathing if the lymph nodes compress the airways.
- Chest pain or discomfort.
- Mediastinal masses that may be detected through imaging studies.

Diagnosis

Diagnosis of Burkitt lymphoma typically involves:
- Imaging Studies: CT scans or PET scans to identify the extent of lymph node involvement and any associated masses.
- Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph nodes, which reveals characteristic histological features, including a high proliferation index and the presence of starry sky appearance due to macrophage activity.
- Flow Cytometry and Cytogenetics: These tests help confirm the B-cell lineage and identify specific genetic abnormalities, such as MYC translocations.

Treatment

The treatment for Burkitt lymphoma is aggressive and often includes:
- Chemotherapy: Intensive multi-agent chemotherapy regimens are the cornerstone of treatment, aiming for rapid tumor reduction.
- Immunotherapy: In some cases, monoclonal antibodies may be used to target specific antigens on the lymphoma 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 vary based on several factors, including the stage of the disease at diagnosis, the patient's age, and the presence of any comorbid conditions. Generally, with appropriate treatment, the prognosis can be favorable, especially in pediatric cases.

In summary, ICD-10 code C83.72 specifically denotes Burkitt lymphoma localized to the intrathoracic lymph nodes, highlighting the need for prompt diagnosis and aggressive treatment to improve patient outcomes. Understanding the clinical features, diagnostic criteria, and treatment options is essential for healthcare providers managing this aggressive lymphoma type.

Burkitt lymphoma, particularly when it involves intrathoracic lymph nodes, presents a unique clinical picture characterized by specific signs, symptoms, and patient demographics. Understanding these aspects is crucial for accurate diagnosis and management.

Clinical Presentation of Burkitt Lymphoma

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma that primarily affects B-lymphocytes. It is known for its rapid growth and can manifest in various anatomical locations, including the abdomen, jaw, and lymph nodes, with intrathoracic involvement being less common but significant.

Signs and Symptoms

1. Lymphadenopathy:
   - Patients often present with enlarged lymph nodes, particularly in the neck, axilla, or groin. In cases where intrathoracic lymph nodes are involved, mediastinal lymphadenopathy may be observed, leading to respiratory symptoms[6].

2. Respiratory Distress:
   - Due to the location of the lymph nodes, patients may experience symptoms such as cough, dyspnea (shortness of breath), or wheezing, which can result from compression of the airways or lung tissue[6].

3. Fever and Night Sweats:
   - Systemic symptoms like fever, drenching night sweats, and unexplained weight loss are common in lymphoma patients, including those with Burkitt lymphoma[6].

4. Abdominal Symptoms:
   - If the lymphoma involves abdominal lymph nodes or organs, patients may report abdominal pain, distension, or changes in bowel habits due to mass effects[6].

5. Tumor Lysis Syndrome:
   - Given the aggressive nature of Burkitt lymphoma, patients may be at risk for tumor lysis syndrome, which can lead to metabolic abnormalities such as hyperuricemia, hyperkalemia, and acute kidney injury[6].

Patient Characteristics

1. Age:
   - Burkitt lymphoma 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].

2. Gender:
   - There is a slight male predominance in the incidence of Burkitt lymphoma, although it can affect individuals of any gender[6].

3. Immunocompromised Status:
   - Patients with compromised immune systems, such as those with HIV/AIDS, are at a higher risk for developing Burkitt lymphoma. The disease is also associated with certain genetic mutations and chromosomal translocations, particularly involving the MYC gene[6].

4. Geographic Variability:
   - The incidence of Burkitt lymphoma varies geographically, with higher rates observed in regions of Africa, where it is often associated with endemic forms linked to Epstein-Barr virus (EBV) infection. In contrast, sporadic cases are more common in Western countries[6].

Conclusion

Burkitt lymphoma, particularly with intrathoracic lymph node involvement, presents with a range of clinical signs and symptoms that can significantly impact patient health. Early recognition of these symptoms, along with an understanding of patient demographics, is essential for timely diagnosis and treatment. Given its aggressive nature, prompt intervention is critical to improve outcomes for affected individuals.

Burkitt lymphoma, classified under ICD-10 code C83.72, specifically refers to a type of non-Hodgkin lymphoma that primarily affects the intrathoracic lymph nodes. This aggressive form of lymphoma is characterized by rapid growth and is often associated with specific genetic mutations. 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 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 Epstein-Barr virus (EBV) infection.
3. Sporadic Burkitt Lymphoma: This variant occurs more frequently in non-endemic regions and is not linked to EBV as strongly as the endemic type.
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 where lymphocytes (a type of white blood cell) proliferate abnormally, including various types of lymphomas.
3. Intrathoracic Lymphadenopathy: This term describes the enlargement of lymph nodes within the thoracic cavity, which is a common presentation in cases of Burkitt lymphoma affecting the intrathoracic region.
4. 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 involved in the immune response.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.72 is crucial for accurate diagnosis, treatment planning, and communication among healthcare professionals. Burkitt lymphoma, with its aggressive nature and specific associations, requires careful consideration in clinical practice, particularly regarding its variants and the broader category of non-Hodgkin lymphomas.

Burkitt lymphoma, classified under ICD-10 code C83.72, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. The diagnosis of Burkitt lymphoma, particularly when it involves intrathoracic lymph nodes, relies on a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria and steps involved in diagnosing this condition.

Clinical Presentation

Symptoms

Patients with Burkitt lymphoma may present with various symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the neck, abdomen, or thoracic region.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many lymphomas.
- Abdominal Pain or Distension: Due to lymph node enlargement or involvement of abdominal organs.
- Respiratory Symptoms: If intrathoracic lymph nodes are involved, patients may experience cough, chest pain, or difficulty breathing.

Diagnostic Imaging

Imaging Techniques

1. CT Scan: A computed tomography (CT) scan of the chest, abdomen, and pelvis is often performed to identify enlarged lymph nodes and assess the extent of disease.
2. PET Scan: Positron emission tomography (PET) may be used to evaluate metabolic activity in lymph nodes and other tissues, helping to distinguish between active disease and residual masses post-treatment.

Histopathological Examination

Biopsy

A definitive diagnosis of Burkitt lymphoma requires a biopsy of the affected lymph node or tissue. The following histological features are critical:
- High Mitotic Index: A high number of dividing cells, indicative of aggressive proliferation.
- Starry Sky Appearance: This characteristic pattern is due to interspersed macrophages within the tumor, which phagocytize apoptotic cells and debris.
- Immunophenotyping: Flow cytometry or immunohistochemistry is used to identify specific markers. Burkitt lymphoma typically expresses:
- CD10
- CD19
- CD20
- CD22
- BCL-6
- Ki-67 (high proliferation index)

Genetic Testing

• MYC Gene Rearrangement: The presence of a translocation involving the MYC gene (most commonly t(8;14)) is a hallmark of Burkitt lymphoma and is crucial for diagnosis.

Laboratory Tests

Blood Tests

• Complete Blood Count (CBC): May show cytopenias or elevated white blood cell counts.
• Lactate Dehydrogenase (LDH): Elevated levels can indicate tumor burden and are often associated with aggressive lymphomas.

Conclusion

The diagnosis of Burkitt lymphoma, particularly with intrathoracic lymph node involvement (ICD-10 code C83.72), is a multifaceted process that includes clinical assessment, imaging studies, histopathological evaluation, and genetic testing. Early and accurate diagnosis is essential for effective treatment planning, as Burkitt lymphoma is highly responsive to chemotherapy but requires prompt intervention due to its aggressive nature. If you have further questions or need more specific information, feel free to ask!

Burkitt lymphoma, particularly when it involves intrathoracic lymph nodes, is a highly aggressive form of non-Hodgkin lymphoma (NHL) that requires prompt and effective treatment. The standard treatment approaches for this condition typically involve a combination of chemotherapy, immunotherapy, and sometimes radiation therapy. Below is a detailed overview of the standard treatment modalities for Burkitt lymphoma, specifically for cases coded under ICD-10 C83.72.

Overview of Burkitt Lymphoma

Burkitt lymphoma is characterized by the rapid proliferation of B-lymphoid cells and is classified into three main types: endemic, sporadic, and immunodeficiency-associated. The intrathoracic involvement indicates that the lymphoma has spread to the lymph nodes within the thoracic cavity, which can complicate treatment and prognosis.

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), and Methotrexate (CODOX-M), followed by Ifosfamide, Etoposide, and Cytarabine (IVAC). This intensive regimen is designed to achieve rapid tumor reduction and is often administered in cycles.

• Hyper-CVAD: This regimen consists of alternating cycles of Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone (CVAD) with high-dose Methotrexate and Cytarabine. Hyper-CVAD is particularly effective in treating aggressive lymphomas and is tailored to the patient's response and tolerance.

2. Immunotherapy

Immunotherapy has become an integral part of the treatment landscape for various lymphomas, including Burkitt lymphoma. The following agents may be utilized:

• Rituximab: This monoclonal antibody targets the CD20 antigen on B-cells and is often used in combination with chemotherapy to enhance treatment efficacy.

• CAR T-cell Therapy: For relapsed or refractory cases, Chimeric Antigen Receptor (CAR) T-cell therapy may be considered. This involves modifying a patient’s T-cells to better recognize and attack lymphoma cells.

3. Radiation Therapy

While chemotherapy is the primary treatment, radiation therapy may be employed in specific scenarios:

• Palliative Care: Radiation can be used to relieve symptoms in patients with bulky disease or those experiencing pain due to lymph node enlargement.

• Consolidation Therapy: In some cases, radiation may be used after chemotherapy to target residual disease, particularly if there are localized areas of concern.

4. Stem Cell Transplantation

For patients with high-risk features or those who experience relapse, autologous stem cell transplantation (ASCT) may be considered. This approach involves harvesting the patient’s stem cells, administering high-dose chemotherapy, and then reinfusing the stem cells to restore bone marrow function.

Conclusion

The treatment of Burkitt lymphoma, particularly with intrathoracic lymph node involvement, is complex and requires a multidisciplinary approach. The standard treatment typically involves aggressive chemotherapy regimens, often supplemented with immunotherapy and, in select cases, radiation therapy or stem cell transplantation. Given the aggressive nature of this lymphoma, timely diagnosis and initiation of treatment are crucial for improving patient outcomes. Regular follow-up and monitoring for potential complications or relapses are also essential components of care.

For the most effective treatment plan, it is vital for patients to work closely with a healthcare team specializing in hematologic malignancies to tailor the approach based on individual patient factors and disease characteristics.