ICD-10: C83.70

Burkitt lymphoma, classified under ICD-10 code C83.70, 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 the MYC gene translocation, which plays a critical role in its pathogenesis. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Burkitt Lymphoma

Overview

Burkitt lymphoma is categorized as a B-cell neoplasm and is one of the fastest-growing human tumors. It is most commonly seen in children and young adults, although it can occur at any age. The disease is named after Dr. Denis Burkitt, who first described it in African children in the 1950s, where it often presents as a jaw lesion.

Types of Burkitt Lymphoma

There are three main types of Burkitt lymphoma:
1. Endemic Burkitt Lymphoma: Predominantly found in Africa, often associated with Epstein-Barr virus (EBV) infection, and typically presents with jaw or facial bone lesions.
2. Sporadic Burkitt Lymphoma: More common in the United States and Europe, this type usually presents with abdominal masses and is less frequently 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 may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, often in the neck, abdomen, or groin.
- Abdominal Pain: Due to enlarged lymph nodes or masses in the abdomen.
- Fever and Night Sweats: Common systemic symptoms associated with lymphoma.
- Weight Loss: Unintentional weight loss can occur due to the disease's aggressive nature.
- Fatigue: Generalized weakness and fatigue are frequent complaints.

Diagnosis

Diagnosis of Burkitt lymphoma typically involves:
- Histopathological Examination: A biopsy of the 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 (e.g., CD19, CD20).
- Cytogenetic Analysis: Detection of MYC gene translocation (most commonly t(8;14)) is crucial for confirming the diagnosis.

Treatment

The treatment of Burkitt lymphoma is aggressive and often includes:
- Chemotherapy: Intensive multi-agent chemotherapy regimens, such as the CODOX-M/IVAC protocol, are commonly used.
- Immunotherapy: Agents like rituximab may be included in treatment regimens to target CD20-positive B-cells.
- Stem Cell Transplantation: In cases of relapse or refractory disease, hematopoietic stem cell transplantation may be considered.

Prognosis

The prognosis for Burkitt lymphoma can be favorable with early diagnosis and aggressive treatment, with overall survival rates significantly improved in pediatric cases compared to adults. However, the disease's aggressive nature necessitates prompt intervention to prevent rapid progression.

Conclusion

ICD-10 code C83.70 refers to Burkitt lymphoma, unspecified site, highlighting the need for precise diagnosis and treatment strategies tailored to the individual patient. Understanding the clinical features, diagnostic criteria, and treatment options is essential for healthcare providers managing this aggressive lymphoma. Early recognition and intervention are critical to improving patient outcomes in Burkitt lymphoma.

Burkitt lymphoma, classified under ICD-10 code C83.70, 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 treatment.

Clinical Presentation

General Overview

Burkitt lymphoma is primarily a B-cell lymphoma that can manifest in various forms, including endemic (often associated with malaria in Africa), sporadic (more common in the Western world), and immunodeficiency-associated types. The clinical presentation can vary significantly based on the subtype and the affected sites.

Common Signs and Symptoms

1. Lymphadenopathy: Patients often present with swollen lymph nodes, which may be painless. This can occur in various regions, including cervical, axillary, or inguinal areas.
2. Abdominal Mass: In many cases, especially in the sporadic form, patients may develop a palpable abdominal mass due to lymphadenopathy or involvement of the gastrointestinal tract, leading to symptoms such as abdominal pain or distension.
3. B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many lymphomas and indicate systemic involvement.
4. Bone Marrow Involvement: Patients may experience symptoms related to bone marrow infiltration, such as fatigue, anemia, or increased susceptibility to infections due to leukopenia.
5. CNS Involvement: In some cases, particularly in immunocompromised patients, Burkitt lymphoma can involve the central nervous system, leading to neurological symptoms such as headaches, seizures, or changes in mental status.

Patient Characteristics

• Age: Burkitt lymphoma predominantly affects children and young adults, although it can occur at any age. The endemic form is more common in children, while the sporadic form is often seen in adolescents and young adults.
• Gender: There is a slight male predominance in the incidence of Burkitt lymphoma.
• Geographic Variation: The endemic form is primarily found in Africa, often associated with Epstein-Barr virus (EBV) infection, while the sporadic form is more prevalent in developed countries.
• Immunocompromised Status: Patients with weakened immune systems, such as those with HIV/AIDS, are at a higher risk for developing Burkitt lymphoma.

Conclusion

Burkitt lymphoma, coded as C83.70 in the ICD-10 classification, presents with a range of clinical features that can vary based on the patient's age, immune status, and the specific subtype of the disease. Early recognition of symptoms such as lymphadenopathy, abdominal masses, and systemic B symptoms is essential for prompt diagnosis and treatment. Understanding the patient characteristics associated with this aggressive lymphoma can aid healthcare providers in identifying at-risk individuals and implementing appropriate management strategies.

Burkitt lymphoma, classified under the ICD-10-CM code C83.70, is a type of non-Hodgkin lymphoma characterized by the rapid growth of tumors, primarily affecting B-lymphocytes. 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 spelling that retains the same meaning.
2. Endemic Burkitt Lymphoma: 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 term describes the variant that occurs outside of endemic regions, typically in non-tropical areas, and is more common in children and young adults.
4. AIDS-related Burkitt Lymphoma: This designation 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. B-cell Lymphoma: Since Burkitt lymphoma originates from B-lymphocytes, it is often categorized under B-cell lymphomas.
3. Lymphoproliferative Disorders: This broader category includes conditions characterized by the excessive proliferation of lymphocytes, including various types of lymphomas.
4. Lymphoma, Non-follicular: Burkitt lymphoma is classified as a non-follicular lymphoma, distinguishing it from follicular lymphoma, another subtype of non-Hodgkin lymphoma.

Clinical Context

Burkitt lymphoma is notable for its aggressive nature and rapid progression, often requiring prompt diagnosis and treatment. The distinction between endemic and sporadic forms is crucial for understanding epidemiology and potential treatment approaches. Additionally, recognizing its association with immunocompromised states, such as in AIDS-related cases, is vital for patient management and therapeutic strategies.

In summary, the ICD-10-CM code C83.70 for Burkitt lymphoma, unspecified site, encompasses various alternative names and related terms that reflect its classification, epidemiology, and clinical significance. Understanding these terms can facilitate better communication among healthcare professionals and improve patient care outcomes.

Burkitt lymphoma, classified under ICD-10 code C83.70, is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. The diagnosis of Burkitt lymphoma involves a combination of clinical evaluation, imaging studies, laboratory tests, and histopathological examination. Below are the key criteria used for diagnosing this specific type of lymphoma.

Clinical Presentation

1. Symptoms: Patients often present with symptoms such as:
   - Rapidly enlarging lymph nodes, particularly in the abdomen or neck.
   - Abdominal pain or swelling due to lymphadenopathy or organ involvement.
   - Fever, night sweats, and weight loss (B symptoms) may also be present, although they are less common in Burkitt lymphoma compared to other lymphomas[3].

2. Age and Demographics: Burkitt lymphoma is more prevalent in children and young adults, although it can occur at any age. The endemic form is often associated with Epstein-Barr virus (EBV) infection and is more common in African children[3].

Laboratory and Imaging Studies

1. Blood Tests:
   - Complete blood count (CBC) may show anemia, thrombocytopenia, or leukopenia.
   - Elevated lactate dehydrogenase (LDH) levels are often indicative of high tumor burden[3].

2. Imaging:
   - CT scans or MRI may be used to identify the extent of lymphadenopathy or any abdominal masses.
   - PET scans can help assess metabolic activity and staging of the disease[3].

Histopathological Examination

1. Biopsy: A definitive diagnosis of Burkitt lymphoma requires a tissue biopsy, typically from an enlarged lymph node or mass. The biopsy should be processed for histological examination.

2. Microscopic Features: Pathologists look for:
   - A high proliferation index, often assessed by Ki-67 staining, which is typically >95%.
   - A characteristic "starry sky" appearance due to interspersed macrophages among the neoplastic cells[3].

3. Immunophenotyping: Flow cytometry or immunohistochemistry is used to confirm the B-cell lineage and to identify specific markers:
   - Positive for CD19, CD20, and CD10.
   - Negative for CD5 and CD23, which helps differentiate it from other types of B-cell lymphomas[3].

Genetic Testing

1. Cytogenetic Analysis: The presence of the MYC gene rearrangement is a hallmark of Burkitt lymphoma. This can be detected through:
   - Fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) techniques, which confirm the diagnosis by identifying the t(8;14) translocation[3].

Conclusion

The diagnosis of Burkitt lymphoma (ICD-10 code C83.70) is multifaceted, relying on clinical presentation, laboratory findings, imaging studies, histopathological examination, and genetic testing. Each of these components plays a crucial role in establishing an accurate diagnosis and guiding treatment decisions. If you have further questions or need more specific information, feel free to ask!

Burkitt lymphoma, classified under ICD-10 code C83.70, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid tumor growth. It primarily affects children and young adults, although it can occur at any age. The treatment for Burkitt lymphoma typically involves a combination of chemotherapy, targeted therapy, and sometimes radiation, 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 (Ara-C) and etoposide. This regimen is designed to achieve rapid tumor reduction and is often administered in cycles over several months.

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

2. Targeted Therapy

Targeted therapies may be used in conjunction with chemotherapy to improve outcomes. Rituximab, a monoclonal antibody that targets the CD20 protein on B-cells, is often included in treatment regimens for Burkitt lymphoma, especially in cases where the lymphoma expresses CD20. This can enhance the effectiveness of chemotherapy and reduce the risk of relapse[1].

3. Radiation Therapy

Radiation therapy is not typically the primary treatment for Burkitt lymphoma but may be used in specific situations, such as:

• Localized Disease: If the lymphoma is localized and has not spread extensively, radiation may be used to target the tumor directly.
• Palliative Care: In cases where the disease is advanced and causing symptoms, radiation can help alleviate pain or other complications.

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, potentially leading to better long-term outcomes[2].

5. Clinical Trials

Given the aggressive nature of Burkitt lymphoma, participation in clinical trials may be an option for patients. These trials often explore new treatment combinations, novel agents, or innovative approaches to improve survival rates and reduce side effects.

Conclusion

The treatment of Burkitt lymphoma (ICD-10 code C83.70) is multifaceted, primarily relying on aggressive chemotherapy regimens, with the potential addition of targeted therapies and radiation in select cases. The choice of treatment is influenced by various factors, including the patient's age, overall health, and specific disease characteristics. Ongoing research and clinical trials continue to refine these approaches, aiming to enhance outcomes for patients diagnosed with this challenging malignancy. For the most current treatment protocols, consultation with a hematologist or oncologist specializing in lymphomas is essential[3].

[1] Billing and Coding: Biomarkers for Oncology (A52986)
[2] HCT for Non-Hodgkin Lymphoma
[3] Medical Necessity Tool for Flow Cytometry