ICD-10: C83.77

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. The ICD-10 code C83.77 specifically refers to Burkitt lymphoma that is localized to the spleen. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Burkitt Lymphoma

Overview

Burkitt lymphoma is classified 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 associated with chromosomal translocations, particularly involving the MYC gene, which plays a crucial role in cell growth and division.

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, often involving the ileocecal region.
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:
- Abdominal pain or swelling due to splenic involvement.
- Rapidly enlarging lymph nodes.
- Fever, night sweats, and weight loss (B symptoms).
- Increased susceptibility to infections due to immune system compromise.

Diagnosis

Diagnosis typically involves:
- Histopathological Examination: A biopsy of the affected tissue, often revealing a high proliferation index (Ki-67).
- Immunophenotyping: Identifying specific markers (e.g., CD19, CD20, CD10) on the surface of the lymphoma cells.
- Cytogenetic Analysis: Detecting MYC gene translocations, which are characteristic of Burkitt lymphoma.

Treatment

The treatment for Burkitt lymphoma is aggressive and often includes:
- Chemotherapy: Intensive regimens such as the CODOX-M/IVAC protocol are commonly used.
- Immunotherapy: Targeted therapies may be employed, especially in relapsed cases.
- Stem Cell Transplantation: Considered in high-risk patients or those with relapsed disease.

ICD-10 Code C83.77: Burkitt Lymphoma, Spleen

Specifics of the Code

• ICD-10 Code: C83.77
• Description: This code specifically denotes Burkitt lymphoma that is localized to the spleen, indicating that the disease has a primary site of involvement in this organ.

Clinical Implications

The designation of C83.77 is crucial for clinical documentation, billing, and treatment planning. Accurate coding ensures that patients receive appropriate care and that healthcare providers are reimbursed correctly for the services rendered.

Prognosis

The prognosis for patients with Burkitt lymphoma can vary significantly based on several factors, including:
- The stage of the disease at diagnosis.
- The patient's age and overall health.
- The response to initial treatment.

With aggressive treatment, many patients can achieve remission, but the risk of relapse remains a concern, necessitating close monitoring.

Conclusion

Burkitt lymphoma, particularly when localized to the spleen (ICD-10 code C83.77), is a serious and rapidly progressing malignancy that requires prompt diagnosis and aggressive treatment. Understanding the clinical features, diagnostic criteria, and treatment options is essential for healthcare providers managing patients with this condition. Regular follow-up and monitoring are critical to ensure the best possible outcomes for affected individuals.

Burkitt lymphoma, particularly when it involves the spleen, 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 management.

Clinical Presentation

Overview of Burkitt Lymphoma

Burkitt lymphoma is classified as a B-cell neoplasm and is known for its association with the MYC gene translocation. It can present in various forms, including endemic (often seen in Africa), sporadic (more common in the Western world), and immunodeficiency-associated types. The involvement of the spleen, denoted by the ICD-10 code C83.77, indicates a specific manifestation of the disease that can complicate the clinical picture.

Signs and Symptoms

Patients with Burkitt lymphoma involving the spleen may exhibit a range of signs and symptoms, including:

• Abdominal Pain or Discomfort: Due to splenic enlargement or involvement, patients often report pain or a feeling of fullness in the abdomen.
• Splenomegaly: An enlarged spleen is a common physical finding, which may be palpable during a physical examination.
• Lymphadenopathy: Patients may present with swollen lymph nodes, particularly in the cervical, axillary, or inguinal regions.
• Fever: Persistent or intermittent fevers may occur, often associated with systemic symptoms.
• Night Sweats: Patients frequently experience drenching night sweats.
• Weight Loss: Unintentional weight loss is common and can be significant.
• Fatigue: A general sense of fatigue or malaise is often reported.

Additional Symptoms

In some cases, patients may also experience:

• Cytopenias: Due to bone marrow involvement, leading to anemia, thrombocytopenia, or leukopenia.
• Gastrointestinal Symptoms: Such as nausea or vomiting, particularly if there is abdominal involvement.
• Respiratory Symptoms: If there is mediastinal involvement, patients may experience cough or difficulty breathing.

Patient Characteristics

Demographics

• Age: Burkitt lymphoma predominantly affects children and young adults, although it can occur at any age. The sporadic form is more common in older individuals.
• Gender: There is a slight male predominance in cases of Burkitt lymphoma.

Risk Factors

• Immunocompromised State: Patients with HIV/AIDS or those on immunosuppressive therapy are at increased risk for developing Burkitt lymphoma.
• Geographic Location: Endemic Burkitt lymphoma is more prevalent in regions of Africa, often associated with malaria and Epstein-Barr virus (EBV) infection.

Clinical Course

Burkitt lymphoma is known for its rapid progression, and patients may present with advanced disease at the time of diagnosis. The aggressive nature of the lymphoma necessitates prompt treatment, often involving chemotherapy regimens that are intensive and may include agents such as cyclophosphamide, doxorubicin, vincristine, and methotrexate.

Conclusion

Burkitt lymphoma involving the spleen (ICD-10 code C83.77) presents with a distinct set of clinical features, including abdominal pain, splenomegaly, and systemic symptoms like fever and weight loss. Understanding these characteristics is essential for healthcare providers to facilitate early diagnosis and initiate appropriate treatment strategies. Given the aggressive nature of this lymphoma, timely intervention is critical to improve patient outcomes.

Burkitt lymphoma, classified under the ICD-10 code C83.77, is a type of non-Hodgkin lymphoma characterized by the rapid growth of tumors, often affecting the lymphatic system. Understanding 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, particularly focusing on its presentation in the spleen.

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 and typically presents as a jaw lesion in children.
3. Sporadic Burkitt Lymphoma: This variant occurs more frequently in non-endemic regions and is characterized by abdominal masses, including splenic involvement.
4. AIDS-related Burkitt Lymphoma: This term is used when Burkitt lymphoma occurs in patients with HIV/AIDS, reflecting 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. Lymphoma: A broader term that refers to cancers that originate in the lymphatic system, including both Hodgkin and non-Hodgkin lymphomas.
3. B-cell Lymphoma: Burkitt lymphoma is classified as a B-cell lymphoma, indicating that it arises from B-lymphocytes, a type of white blood cell.
4. Lymphadenopathy: This term describes the enlargement of lymph nodes, which can be a clinical feature of Burkitt lymphoma.
5. Splenic Lymphoma: This term may be used to describe lymphomas that primarily affect the spleen, including Burkitt lymphoma when it presents with splenic involvement.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.77: Burkitt lymphoma, spleen, is essential for accurate diagnosis, treatment planning, and communication among healthcare professionals. These terms not only facilitate better understanding of the disease but also aid in the classification and management of patients with this aggressive form of lymphoma.

Burkitt lymphoma, classified under ICD-10 code C83.77, is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. Diagnosing this condition involves a combination of clinical evaluation, imaging studies, laboratory tests, and histopathological examination. Below, we outline the key criteria and methods used in the diagnosis of Burkitt lymphoma, particularly when it involves the spleen.

Clinical Presentation

Symptoms

Patients with Burkitt lymphoma may present with various symptoms, including:
- Abdominal pain or swelling: Often due to splenic involvement or lymphadenopathy.
- Fever: Commonly associated with malignancies.
- Night sweats: A typical symptom in lymphomas.
- Weight loss: Unintentional weight loss can occur.
- Fatigue: Generalized fatigue is frequently reported.

Physical Examination

During a physical examination, clinicians may find:
- Splenomegaly: Enlargement of the spleen is a significant indicator, especially in cases of C83.77.
- Lymphadenopathy: Swollen lymph nodes may be present in various regions.

Imaging Studies

Ultrasound and CT Scans

• Ultrasound: This imaging modality can help visualize splenomegaly and assess the spleen's structure.
• CT Scan: A computed tomography scan of the abdomen and pelvis is crucial for evaluating the extent of disease, including lymph node involvement and any abdominal masses.

Laboratory Tests

Blood Tests

• Complete Blood Count (CBC): May show anemia, thrombocytopenia, or leukopenia.
• Lactate Dehydrogenase (LDH): Elevated levels can indicate 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).
• PCR Testing: Polymerase chain reaction (PCR) can be used to detect specific genetic alterations associated with Burkitt lymphoma.

Histopathological Examination

Biopsy

• Tissue Biopsy: A definitive diagnosis is made through a biopsy of the affected tissue, which may include the spleen or lymph nodes. Histological examination typically reveals:
• High proliferation index: Characterized by a high Ki-67 labeling index.
• Starry sky appearance: This is due to the presence of macrophages engulfing apoptotic cells and necrotic debris.

Immunophenotyping

• Flow Cytometry: This technique is used to analyze the surface markers on the lymphoma cells. Burkitt lymphoma cells typically express:
• CD19, CD20, and CD10: These are common B-cell markers.
• IgM: Immunoglobulin M may also be expressed.

Conclusion

The diagnosis of Burkitt lymphoma, particularly when involving the spleen (ICD-10 code C83.77), relies on a comprehensive approach that includes clinical evaluation, imaging studies, laboratory tests, and histopathological analysis. The combination of these diagnostic criteria helps ensure accurate identification and appropriate management of this aggressive lymphoma. Early diagnosis is crucial for improving patient outcomes, given the rapid progression of the disease.

Burkitt lymphoma, classified under ICD-10 code C83.77, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid tumor growth. It primarily affects children and young adults but can occur in adults as well. The spleen is one of the common sites for Burkitt lymphoma involvement, and its treatment typically involves a combination of chemotherapy, immunotherapy, and sometimes radiation therapy. Below is a detailed overview of the standard treatment approaches for Burkitt lymphoma, particularly when the spleen is involved.

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 treatment is designed to achieve rapid tumor reduction and is often administered in cycles over several months[1].

• R-CHOP: While not the standard for Burkitt lymphoma, some patients may receive R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) if they have a less aggressive variant or if they are older and cannot tolerate more intensive regimens[2].

2. Immunotherapy

Rituximab, a monoclonal antibody targeting CD20, is often included in treatment regimens for Burkitt lymphoma, especially in cases where the lymphoma expresses this antigen. It can enhance the effectiveness of chemotherapy and improve overall outcomes[3].

3. Radiation Therapy

Radiation therapy may be used in specific situations, such as when there is localized disease or to manage symptoms. However, it is not typically the primary treatment modality for Burkitt lymphoma due to the disease's aggressive nature and the effectiveness of chemotherapy[4].

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[5].

5. Supportive Care

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

• Antibiotic prophylaxis to prevent infections due to immunosuppression.
• Growth factor support (e.g., G-CSF) to stimulate white blood cell production.
• Nutritional support to maintain strength and health during treatment[6].

Conclusion

The treatment of Burkitt lymphoma, particularly when involving the spleen, is aggressive and multifaceted, primarily relying on chemotherapy and immunotherapy. The choice of specific regimens may vary based on patient age, overall health, and the extent of the disease. Continuous monitoring and supportive care are essential to manage the side effects of treatment and improve patient outcomes. For patients diagnosed with Burkitt lymphoma, a multidisciplinary approach involving oncologists, hematologists, and supportive care teams is critical for effective management.

For further information or personalized treatment options, consulting with a healthcare provider specializing in hematologic malignancies is recommended.