ICD-10: C83.79

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid growth and a distinct genetic profile. The ICD-10 code C83.79 specifically refers to Burkitt lymphoma that occurs in extranodal and solid organ sites, distinguishing it from other forms of the disease that may present in lymph nodes or other typical locations.

Clinical Description of Burkitt Lymphoma

Overview

Burkitt lymphoma is classified as a B-cell neoplasm and is known for its association with the MYC gene translocation, which plays a crucial role in cell proliferation. This lymphoma 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 often linked to Epstein-Barr virus (EBV) infection.

Symptoms

Patients with Burkitt lymphoma may present with a variety of symptoms, which can include:
- Abdominal pain or swelling: Often due to the involvement of the gastrointestinal tract or kidneys.
- Lymphadenopathy: Swelling of lymph nodes, although in extranodal cases, this may be less prominent.
- Fever and night sweats: Common systemic symptoms associated with lymphoma.
- Weight loss: Unintentional weight loss can occur due to the aggressive nature of the disease.
- Fatigue: Generalized fatigue is frequently reported.

Extranodal Involvement

In the context of ICD-10 code C83.79, the term "extranodal" refers to the lymphoma's occurrence outside of the lymphatic system. Common extranodal sites for Burkitt lymphoma include:
- Gastrointestinal tract: Particularly the ileocecal region.
- Bone marrow: Involvement can lead to cytopenias.
- Central nervous system (CNS): CNS involvement is a critical concern due to the potential for neurological symptoms.
- Testes: Testicular involvement can occur, particularly in males.

Diagnosis

Diagnosis of Burkitt lymphoma typically involves:
- Histopathological examination: A biopsy of the affected tissue is essential for diagnosis, revealing characteristic high-grade B-cell proliferation.
- Immunophenotyping: Flow cytometry is used to identify specific B-cell markers (e.g., CD19, CD20).
- Cytogenetic analysis: Detection of MYC gene translocation is a hallmark of Burkitt lymphoma.
- Imaging studies: CT scans or PET scans may be utilized to assess the extent of disease and identify extranodal sites.

Treatment

The treatment of Burkitt lymphoma is aggressive and often includes:
- Chemotherapy: Intensive multi-agent chemotherapy regimens are standard, with protocols such as CODOX-M/IVAC being commonly used.
- Supportive care: Management of complications such as tumor lysis syndrome is critical due to the rapid cell turnover associated with treatment.
- Stem cell transplantation: In cases of relapsed or refractory disease, hematopoietic stem cell transplantation may be considered.

Conclusion

Burkitt lymphoma, particularly in its extranodal and solid organ manifestations as denoted by ICD-10 code C83.79, represents a challenging clinical entity requiring prompt diagnosis and aggressive treatment. Understanding the clinical features, diagnostic criteria, and treatment options is essential for healthcare providers managing patients with this aggressive lymphoma. Early intervention can significantly improve outcomes, underscoring the importance of awareness and timely action in the face of this rapidly progressing disease.

Burkitt lymphoma, particularly in its extranodal and solid organ manifestations, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid tumor growth. The clinical presentation, signs, symptoms, and patient characteristics associated with ICD-10 code C83.79 (Burkitt lymphoma, extranodal and solid organ sites) are crucial for diagnosis and management.

Clinical Presentation

General Characteristics

Burkitt lymphoma is known for its fast proliferation and is often seen in two main forms: endemic (associated with malaria and common in Africa) and sporadic (more prevalent in Western countries). The sporadic form typically presents in adolescents and young adults, while the endemic form is more common in children.

Extranodal Involvement

When Burkitt lymphoma presents in extranodal sites, it can affect various organs, including:

• Gastrointestinal Tract: Abdominal pain, distension, and obstruction may occur due to lymphoid tissue infiltration.
• Jaw and Facial Structures: Particularly in endemic cases, patients may present with jaw swelling or facial deformities.
• Central Nervous System (CNS): Neurological symptoms such as headaches, seizures, or altered mental status may arise if the lymphoma infiltrates the CNS.
• Kidneys and Liver: Symptoms may include flank pain, hepatomegaly, or renal dysfunction.

Signs and Symptoms

Common Symptoms

Patients with Burkitt lymphoma may exhibit a range of symptoms, including:

• Lymphadenopathy: Swollen lymph nodes, particularly in the neck, abdomen, or groin.
• Fever: Often low-grade but can be persistent.
• Night Sweats: Commonly reported by patients.
• Weight Loss: Unintentional weight loss is frequently observed.
• Fatigue: Generalized weakness and fatigue are common complaints.

Specific Signs

• Abdominal Mass: Palpable masses in the abdomen may indicate significant lymphoid infiltration.
• CNS Symptoms: Signs of increased intracranial pressure, such as papilledema, may be present if the CNS is involved.
• Skin Lesions: Occasionally, cutaneous manifestations may occur, presenting as nodules or plaques.

Patient Characteristics

Demographics

• Age: Burkitt lymphoma predominantly affects children and young adults, with a peak incidence in those aged 5-10 years for endemic cases and 20-30 years for sporadic cases.
• Gender: There is a slight male predominance in the incidence of Burkitt lymphoma.

Risk Factors

• Immunocompromised States: Patients with HIV/AIDS or those on immunosuppressive therapy are at increased risk.
• Geographic Location: Endemic Burkitt lymphoma is more common in regions with high malaria prevalence, particularly in sub-Saharan Africa.

Clinical Behavior

Burkitt lymphoma is characterized by a high proliferation index, often with a high Ki-67 labeling index, indicating aggressive behavior. The disease can rapidly progress, necessitating prompt diagnosis and treatment.

Conclusion

Understanding the clinical presentation, signs, symptoms, and patient characteristics of Burkitt lymphoma, particularly in its extranodal and solid organ forms, is essential for timely diagnosis and management. The aggressive nature of this lymphoma type requires healthcare providers to be vigilant in recognizing its manifestations, especially in at-risk populations. Early intervention can significantly improve patient outcomes, making awareness of these clinical features critical in clinical practice.

Burkitt lymphoma, classified under ICD-10 code C83.79, refers specifically to a type of non-Hodgkin lymphoma that primarily affects extranodal sites and solid organs. 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 this diagnosis.

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: This term is often used to describe the form of Burkitt lymphoma that is prevalent in certain regions, particularly in Africa, where it is associated with malaria and Epstein-Barr virus (EBV) infection.
3. Sporadic Burkitt Lymphoma: This variant occurs less frequently and is not geographically restricted, typically affecting individuals in non-endemic areas.
4. AIDS-related Burkitt Lymphoma: This term is used when the lymphoma is associated with HIV/AIDS, highlighting the increased risk in immunocompromised patients.

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 lymphoid tissues.
2. Extranodal Lymphoma: This term refers to lymphomas that occur outside of the lymph nodes, which is characteristic of Burkitt lymphoma, particularly in solid organ sites.
3. Solid Organ Lymphoma: This term is used to describe lymphomas that manifest in solid organs, such as the liver, spleen, or kidneys, which can be affected in cases of Burkitt lymphoma.
4. Lymphoproliferative Disorders: This broader category includes various conditions characterized by the proliferation of lymphocytes, including Burkitt lymphoma.
5. B-cell Lymphoma: Since Burkitt lymphoma originates from B-cells, this term is relevant in the context of its classification within lymphomas.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.79 is essential for accurate diagnosis, treatment planning, and communication among healthcare professionals. These terms not only facilitate better understanding of the condition but also aid in the classification and coding processes necessary for effective patient management and billing. If you need further information or specific details about Burkitt lymphoma, feel free to ask!

Burkitt lymphoma, classified under ICD-10 code C83.79, is a highly aggressive form of non-Hodgkin lymphoma that can manifest in extranodal and solid organ sites. The diagnosis of Burkitt lymphoma involves a combination of clinical evaluation, imaging studies, laboratory tests, and histopathological examination. Below is a detailed overview of the criteria used for diagnosing this specific type of lymphoma.

Clinical Presentation

Symptoms

Patients with Burkitt lymphoma may present with various symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, which may be localized or generalized.
- Abdominal Pain or Distension: Often due to the involvement of the gastrointestinal tract or solid organs.
- Fever, Night Sweats, and Weight Loss: These systemic symptoms are common in many lymphomas and indicate a more advanced disease state.
- Rapidly Growing Tumors: Burkitt lymphoma is characterized by its aggressive nature, leading to rapid tumor growth.

Diagnostic Criteria

1. Histopathological Examination

• Biopsy: A tissue biopsy is essential for diagnosis. The histological examination typically reveals a high proliferation of atypical B-lymphoid cells with a 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 specific markers, such as CD19, CD20, and CD10, while typically lacking CD5 and CD23.

2. Cytogenetic Analysis

• Chromosomal Abnormalities: 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).

3. Imaging Studies

• CT or PET Scans: Imaging studies are crucial for assessing the extent of the disease, particularly in extranodal sites. These scans help identify lymphadenopathy, organ involvement, and any potential complications such as bowel obstruction.

4. Laboratory Tests

• Blood Tests: Complete blood counts (CBC) may show cytopenias or elevated lactate dehydrogenase (LDH) levels, which can indicate tumor burden.
• Bone Marrow Biopsy: This may be performed to assess for bone marrow involvement, which is common in advanced cases.

Differential Diagnosis

It is important to differentiate Burkitt lymphoma from other types of lymphomas and malignancies, such as:
- Diffuse Large B-cell Lymphoma (DLBCL): This can present similarly but typically has a different clinical course and histological features.
- Acute Lymphoblastic Leukemia (ALL): Particularly in pediatric cases, where the distinction can be challenging.

Conclusion

The diagnosis of Burkitt lymphoma, particularly in extranodal and solid organ sites, relies on a comprehensive approach that includes clinical evaluation, histopathological confirmation, cytogenetic analysis, and imaging studies. The aggressive nature of this lymphoma necessitates prompt diagnosis and treatment to improve patient outcomes. If you have further questions or need more specific information, feel free to ask!

Burkitt lymphoma, particularly the variant classified under ICD-10 code C83.79, refers to a highly aggressive form of non-Hodgkin lymphoma that can manifest in extranodal and solid organ sites. This type of lymphoma is characterized by rapid growth and requires prompt and effective treatment strategies. Below, we explore the standard treatment approaches for Burkitt lymphoma, focusing on the specific considerations for extranodal and solid organ involvement.

Overview of Burkitt Lymphoma

Burkitt lymphoma is a B-cell neoplasm that is often associated with the MYC gene translocation. It is classified into three main types: endemic, sporadic, and immunodeficiency-associated. The sporadic form, which is more common in adults, can present in various extranodal sites, including the gastrointestinal tract, central nervous system, and other solid organs[1].

Standard Treatment Approaches

1. Chemotherapy

The cornerstone of treatment for Burkitt lymphoma is intensive chemotherapy. The most commonly used regimens include:

• CODOX-M/IVAC: This regimen combines Cyclophosphamide, Vincristine, Doxorubicin, and Methotrexate (CODOX-M) followed by Ifosfamide, Etoposide, and Cytarabine (IVAC). This approach is particularly effective due to its ability to target rapidly dividing cells, which is a hallmark of Burkitt lymphoma[2].

• Hyper-CVAD: This regimen involves alternating cycles of Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone (CVAD) with high-dose Methotrexate and Cytarabine. Hyper-CVAD is often used for patients with more advanced disease or those presenting with CNS involvement[3].

2. Central Nervous System Prophylaxis

Given the high risk of CNS involvement in Burkitt lymphoma, especially in cases with extranodal presentation, CNS prophylaxis is critical. This may involve:

• Intrathecal chemotherapy: Administering chemotherapy directly into the cerebrospinal fluid (CSF) to prevent or treat CNS involvement. Common agents include Methotrexate, Cytarabine, and Hydrocortisone[4].

3. Targeted Therapy

While traditional chemotherapy remains the primary treatment, targeted therapies are being explored. For instance:

• Rituximab: Although primarily used in other types of B-cell lymphomas, Rituximab may be considered in combination with chemotherapy for some patients, particularly those with CD20-positive Burkitt lymphoma[5].

4. Stem Cell Transplantation

For patients with high-risk disease or those who do not achieve remission with initial therapy, hematopoietic stem cell transplantation (HSCT) may be considered. This approach is particularly relevant for relapsed or refractory cases, providing a potential curative option[6].

5. Supportive Care

Supportive care is essential throughout the treatment process. This includes:

• Management of tumor lysis syndrome: Due to the rapid cell turnover associated with Burkitt lymphoma, patients are at risk for tumor lysis syndrome, necessitating careful monitoring and management[7].

• Infection prophylaxis: Given the immunosuppressive nature of chemotherapy, patients require prophylactic antibiotics and antifungals to prevent infections.

Conclusion

The treatment of Burkitt lymphoma, particularly in cases involving extranodal and solid organ sites, necessitates a comprehensive and aggressive approach. Intensive chemotherapy regimens, CNS prophylaxis, and potential use of targeted therapies are central to management. Additionally, supportive care plays a crucial role in optimizing patient outcomes. As research continues, the integration of novel therapies and personalized medicine may further enhance treatment efficacy for this aggressive lymphoma subtype.

For patients diagnosed with Burkitt lymphoma, it is essential to work closely with a multidisciplinary team specializing in hematologic malignancies to tailor the treatment plan to individual needs and disease characteristics.