ICD-10: C83.73

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. The ICD-10 code C83.73 specifically refers to Burkitt lymphoma that primarily affects intra-abdominal 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 non-Hodgkin lymphoma and is known for its fast growth and aggressive nature. 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 with abdominal masses and is not associated with EBV.
3. Immunodeficiency-associated Burkitt Lymphoma: Occurs in individuals with compromised immune systems, such as those with HIV/AIDS.

Symptoms

Patients with Burkitt lymphoma affecting intra-abdominal lymph nodes may present with a variety of symptoms, including:
- Abdominal pain or swelling due to enlarged lymph nodes.
- Intestinal obstruction or perforation if the lymphoma involves the gastrointestinal tract.
- Systemic symptoms such as fever, night sweats, and weight loss.
- Lymphadenopathy in other regions, although the primary focus is in the abdomen.

Diagnosis

Diagnosis of Burkitt lymphoma typically involves:
- Imaging Studies: CT scans or MRIs to identify the extent of lymph node involvement and any abdominal masses.
- Biopsy: A definitive diagnosis is made through histological examination of tissue samples, which reveals the characteristic high proliferation of B-cells.
- Cytogenetic Analysis: Testing for MYC gene translocations can confirm the diagnosis.

Treatment

The treatment for Burkitt lymphoma is aggressive and often includes:
- Chemotherapy: Intensive multi-agent chemotherapy regimens are the standard approach, aiming for rapid tumor reduction.
- Supportive Care: Management of complications such as tumor lysis syndrome, which can occur due to rapid cell turnover during treatment.

ICD-10 Code C83.73

The ICD-10 code C83.73 specifically denotes "Burkitt lymphoma, intra-abdominal lymph nodes." This classification is crucial for accurate medical coding, billing, and epidemiological tracking. It falls under the broader category of non-Hodgkin lymphoma, which includes various subtypes based on histological and clinical features.

Importance of Accurate Coding

Accurate coding is essential for:
- Clinical Management: Ensuring appropriate treatment protocols are followed.
- Research and Epidemiology: Understanding the prevalence and outcomes of this aggressive lymphoma type.
- Insurance and Billing: Facilitating reimbursement for healthcare services provided to patients.

Conclusion

Burkitt lymphoma, particularly when involving intra-abdominal lymph nodes, is a critical condition requiring prompt diagnosis and aggressive treatment. The ICD-10 code C83.73 serves as an important tool for healthcare providers in managing this disease effectively. Understanding the clinical features, diagnostic criteria, and treatment options is essential for improving patient outcomes in those affected by this aggressive lymphoma.

Burkitt lymphoma, classified under ICD-10 code C83.73, specifically refers to a type of non-Hodgkin lymphoma that primarily affects the intra-abdominal lymph nodes. This aggressive form of lymphoma is characterized by rapid cell growth and is often associated with the Epstein-Barr virus (EBV). 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 the spelling of the name, often used interchangeably.
2. Endemic Burkitt Lymphoma: This term refers to the form of Burkitt lymphoma that is prevalent in certain regions of Africa, often associated with malaria and EBV.
3. Sporadic Burkitt Lymphoma: This variant occurs more commonly in non-endemic regions and is not linked to geographic factors.
4. Immunodeficiency-associated Burkitt Lymphoma: This type occurs in individuals with compromised immune systems, such as those with HIV/AIDS.

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. B-cell Lymphoma: Burkitt lymphoma is classified as a B-cell lymphoma, indicating that it originates from B-lymphocytes, a type of immune cell.
4. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.
5. C83.7: This is the broader ICD-10 code category for Burkitt lymphoma, which includes other variants beyond intra-abdominal lymph node involvement.

Clinical Context

Burkitt lymphoma is notable for its rapid progression and is often diagnosed in children and young adults. The intra-abdominal variant, specifically coded as C83.73, indicates that the lymphoma is primarily affecting lymph nodes located in the abdominal region, which can lead to symptoms such as abdominal pain, swelling, and gastrointestinal issues.

Understanding these alternative names and related terms is crucial for healthcare professionals involved in diagnosis, treatment planning, and coding for insurance purposes. Accurate coding and terminology ensure proper communication among medical teams and facilitate research and epidemiological studies related to this aggressive lymphoma type.

Burkitt lymphoma, classified under ICD-10 code C83.73, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. The diagnosis of Burkitt lymphoma, particularly when it involves intra-abdominal lymph nodes, relies on a combination of clinical evaluation, imaging studies, laboratory tests, and histopathological examination. Below is a detailed overview of the criteria used for diagnosing this condition.

Clinical Presentation

Symptoms

Patients with Burkitt lymphoma may present with various symptoms, including:
- Abdominal pain or swelling: Due to the enlargement of intra-abdominal lymph nodes or masses.
- B symptoms: These include fever, night sweats, and unexplained weight loss, which are common in lymphoproliferative disorders.
- Lymphadenopathy: Swelling of lymph nodes, which may be palpable in the abdomen or other regions.

Medical History

A thorough medical history is essential, focusing on:
- Previous malignancies or immunosuppressive conditions.
- Family history of lymphomas or other hematological disorders.

Diagnostic Imaging

Imaging Studies

Imaging plays a crucial role in identifying the extent of the disease:
- CT Scan: A computed tomography scan of the abdomen and pelvis is typically performed to assess the size and location of lymphadenopathy and any associated masses.
- MRI: Magnetic resonance imaging may be used in certain cases to provide detailed images of soft tissues.

Laboratory Tests

Blood Tests

• Complete Blood Count (CBC): This test may reveal anemia, thrombocytopenia, or leukopenia, which can be indicative of bone marrow involvement.
• Lactate Dehydrogenase (LDH): Elevated levels of LDH can suggest a high tumor burden and are often associated with aggressive lymphomas.

Cytogenetic and Molecular Studies

• Cytogenetic Analysis: 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).
• Immunophenotyping: Flow cytometry can be used to analyze the surface markers on the lymphoma cells, typically showing a B-cell phenotype (CD19+, CD20+, CD10+, and often CD5-).

Histopathological Examination

Biopsy

A definitive diagnosis of Burkitt lymphoma requires a tissue biopsy:
- Excisional Biopsy: This is the preferred method for obtaining a representative sample of the lymph node or mass.
- Histological Features: The biopsy will typically show a high proliferation index (often >90% Ki-67 positivity), a starry sky appearance due to interspersed macrophages, and a diffuse infiltrate of atypical lymphoid cells.

Diagnostic Criteria Summary

To summarize, the diagnosis of Burkitt lymphoma, particularly with intra-abdominal lymph node involvement, is based on:
1. Clinical Symptoms: Presence of abdominal pain, lymphadenopathy, and B symptoms.
2. Imaging Studies: CT or MRI findings indicating lymph node enlargement.
3. Laboratory Tests: CBC abnormalities, elevated LDH, and cytogenetic findings.
4. Histopathological Confirmation: Biopsy showing characteristic histological features and immunophenotyping results.

Conclusion

The diagnosis of Burkitt lymphoma, especially when it involves intra-abdominal lymph nodes, is multifaceted, requiring a combination of clinical, imaging, laboratory, and histopathological evaluations. Early and accurate diagnosis is crucial for effective treatment planning and improving patient outcomes. If you have further questions or need more specific information regarding treatment options or prognosis, feel free to ask!

Burkitt lymphoma, particularly when it involves intra-abdominal lymph nodes, is a highly aggressive form of non-Hodgkin lymphoma (NHL) that requires prompt and effective treatment. The ICD-10 code C83.73 specifically designates Burkitt lymphoma with involvement of intra-abdominal lymph nodes, indicating the need for targeted therapeutic strategies. Below is a comprehensive overview of the standard treatment approaches for this condition.

Overview of Burkitt Lymphoma

Burkitt lymphoma is characterized by rapid tumor growth and is often associated with a translocation involving the MYC gene. It can present in various forms, including endemic (often seen in Africa), sporadic (more common in the Western world), and immunodeficiency-associated types. The intra-abdominal presentation can lead to complications such as bowel obstruction or perforation, necessitating urgent intervention.

Standard Treatment Approaches

1. Chemotherapy

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

• CODOX-M/IVAC: This regimen combines Cyclophosphamide, Vincristine, Doxorubicin (Adriamycin), and Methotrexate (CODOX-M) with Ifosfamide, Etoposide, and Cytarabine (IVAC). This intensive treatment is designed to achieve rapid tumor reduction and is typically administered in cycles over several months.

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

2. Intrathecal Chemotherapy

Given the high risk of central nervous system (CNS) involvement in Burkitt lymphoma, intrathecal chemotherapy (administration of chemotherapy directly into the cerebrospinal fluid) is often included in treatment protocols. Agents such as Methotrexate, Cytarabine, and Hydrocortisone are commonly used to prevent or treat CNS disease.

3. Targeted Therapy

While traditional chemotherapy remains the primary treatment, targeted therapies are being explored. For instance, Rituximab, a monoclonal antibody targeting CD20, may be used in some cases, particularly in patients with co-existing conditions or those who are not candidates for intensive chemotherapy. However, its role in Burkitt lymphoma is less established compared to other types of NHL.

4. Stem Cell Transplantation

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

5. Supportive Care

Supportive care is crucial in managing the side effects of aggressive chemotherapy. This includes:

• Antiemetics to control nausea and vomiting.
• Growth factors such as G-CSF (Granulocyte Colony-Stimulating Factor) to support white blood cell recovery.
• Infection prophylaxis due to the immunocompromised state of patients undergoing intensive chemotherapy.

Conclusion

The treatment of Burkitt lymphoma, particularly with intra-abdominal lymph node involvement, is aggressive and multifaceted, primarily relying on intensive chemotherapy regimens. Early diagnosis and prompt initiation of treatment are critical for improving outcomes. As research continues, the integration of targeted therapies and advancements in supportive care will likely enhance the management of this challenging malignancy. For patients diagnosed with C83.73, a multidisciplinary approach involving oncologists, hematologists, and supportive care teams is essential to optimize treatment efficacy and patient quality of life.

Burkitt lymphoma, particularly when it involves intra-abdominal lymph nodes, is a highly aggressive form of non-Hodgkin lymphoma. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for timely diagnosis and management. Below is a detailed overview of these aspects related to ICD-10 code C83.73.

Clinical Presentation

Overview of Burkitt Lymphoma

Burkitt lymphoma is 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 intra-abdominal variant often presents with significant abdominal symptoms due to lymphadenopathy and associated complications.

Signs and Symptoms

Patients with Burkitt lymphoma affecting intra-abdominal lymph nodes may exhibit a range of signs and symptoms, including:

• Abdominal Pain: Often due to the enlargement of lymph nodes or masses in the abdomen, leading to pressure on surrounding organs.
• Abdominal Distension: This can occur as a result of large lymphadenopathy or ascites (fluid accumulation in the abdominal cavity).
• Nausea and Vomiting: These symptoms may arise from gastrointestinal obstruction or irritation caused by enlarged lymph nodes.
• Weight Loss: Unintentional weight loss is common and may be attributed to decreased appetite or increased metabolic demands of the tumor.
• Fever and Night Sweats: Systemic symptoms such as fever, chills, and night sweats may be present, indicating a systemic illness.
• Lymphadenopathy: While intra-abdominal lymph nodes are affected, patients may also have peripheral lymphadenopathy, particularly in the cervical and axillary regions.

Additional Symptoms

• Bowel Obstruction: Due to the mass effect of enlarged lymph nodes, patients may experience constipation or changes in bowel habits.
• Splenomegaly: Enlargement of the spleen can occur, contributing to abdominal discomfort and fullness.
• Hepatomegaly: Liver involvement may also be noted, leading to further abdominal symptoms.

Patient Characteristics

Demographics

• Age: Burkitt lymphoma predominantly affects children and young adults, with a peak incidence between ages 5 and 10. However, it can also occur in adults, particularly in endemic forms associated with Epstein-Barr virus (EBV) infection.
• Gender: There is a slight male predominance in cases of Burkitt lymphoma.

Risk Factors

• Immunocompromised State: Patients with weakened immune systems, such as those with HIV/AIDS, are at increased risk for developing Burkitt lymphoma.
• Geographic Variability: Endemic Burkitt lymphoma is more common in regions of Africa, often associated with malaria and EBV, while sporadic cases are more prevalent in Western countries.

Clinical History

• Rapid Onset: Symptoms typically develop quickly, often over weeks, reflecting the aggressive nature of the disease.
• Previous Health Issues: A history of autoimmune diseases or prior malignancies may be relevant in some patients.

Conclusion

Burkitt lymphoma, particularly when involving intra-abdominal lymph nodes, presents with a distinct set of clinical features that require prompt recognition and intervention. The combination of abdominal pain, distension, systemic symptoms, and demographic factors can guide clinicians in diagnosing this aggressive lymphoma. Early identification and treatment are critical to improving patient outcomes, given the rapid progression associated with this disease. Understanding these clinical presentations and patient characteristics is essential for healthcare providers managing suspected cases of Burkitt lymphoma.