ICD-10: C83.75

Burkitt lymphoma, lymph nodes of inguinal region and lower limb

Additional Information

Description

Burkitt lymphoma, classified under ICD-10 code C83.75, is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. This specific code pertains to cases where the lymphoma is localized in the lymph nodes of the inguinal region and lower limb, indicating a specific anatomical involvement that can influence treatment and prognosis.

Clinical Description of Burkitt Lymphoma

Overview

Burkitt lymphoma is a type of B-cell lymphoma that 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.

Symptoms

Patients with Burkitt lymphoma may present with a variety of symptoms, including:
- Swollen lymph nodes: The most common presentation is the enlargement of lymph nodes, particularly in the abdomen, neck, or inguinal region.
- Abdominal pain or swelling: This can occur if the lymphoma affects abdominal organs or lymph nodes.
- Fever and night sweats: These systemic symptoms are common in many lymphomas.
- Weight loss: Unintentional weight loss can occur due to the disease's aggressive nature and metabolic demands.
- Fatigue: Generalized fatigue is often reported by patients.

Diagnosis

Diagnosis of Burkitt lymphoma typically involves:
- Histopathological examination: A biopsy of the affected lymph node is essential for confirming the diagnosis. The presence of a high proliferation index (often assessed by Ki-67 staining) is indicative of Burkitt lymphoma.
- Imaging studies: CT scans or PET scans may be used to assess the extent of the disease and to identify any additional sites of involvement.
- Cytogenetic analysis: Testing for MYC gene translocations can help confirm the diagnosis.

Specifics of ICD-10 Code C83.75

Code Definition

ICD-10 code C83.75 specifically refers to Burkitt lymphoma that is localized to the lymph nodes of the inguinal region and lower limb. This localization is significant for treatment planning and prognosis.

Treatment Options

The treatment for Burkitt lymphoma typically involves:
- Chemotherapy: Intensive chemotherapy regimens are the cornerstone of treatment. Common regimens include the use of cyclophosphamide, doxorubicin, vincristine, and methotrexate.
- Immunotherapy: In some cases, monoclonal antibodies may be used to target specific markers on the lymphoma cells.
- Stem cell transplant: For patients with high-risk disease or those who do not respond to initial therapy, 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 response to initial treatment. Generally, with appropriate and timely treatment, many patients can achieve remission.

Conclusion

ICD-10 code C83.75 identifies Burkitt lymphoma localized to the lymph nodes of the inguinal region and lower limb, highlighting the importance of precise coding in clinical practice. Understanding the clinical presentation, diagnostic criteria, and treatment options for this aggressive lymphoma is crucial for effective management and improved patient outcomes. Early diagnosis and intervention are key to enhancing survival rates in affected individuals.

Clinical Information

Burkitt lymphoma, classified under ICD-10 code C83.75, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid tumor growth. This lymphoma primarily affects lymphoid tissues and can present in various anatomical locations, including the lymph nodes of the inguinal region and lower limbs. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for timely diagnosis and management.

Clinical Presentation

General Characteristics

Burkitt lymphoma is known for its fast proliferation and is often seen in children and young adults, although it can occur at any age. The clinical presentation can vary significantly based on the site of involvement and the extent of the disease.

Lymph Node Involvement

When Burkitt lymphoma affects the lymph nodes, particularly in the inguinal region and lower limbs, patients may present with:

  • Swollen Lymph Nodes: Patients typically exhibit enlarged lymph nodes, which may be palpable in the groin area. These nodes can be firm and non-tender, and their size can vary from a few centimeters to larger masses.
  • Localized Pain or Discomfort: Some patients may experience pain or discomfort in the affected area, particularly if the lymph nodes are compressing surrounding structures.

Systemic Symptoms

In addition to localized signs, patients may also present with systemic symptoms, which can include:

  • Fever: Often low-grade but can be persistent.
  • Night Sweats: Patients may experience drenching night sweats.
  • Weight Loss: Unintentional weight loss is common, often due to the metabolic demands of the rapidly growing tumor.
  • Fatigue: Generalized fatigue and malaise are frequently reported.

Signs and Symptoms

Localized Signs

  • Palpable Masses: Enlarged lymph nodes in the inguinal region may be noted during physical examination.
  • Skin Changes: In some cases, skin lesions may develop if the lymphoma infiltrates the skin.

Systemic Symptoms

  • B Symptoms: The presence of fever, night sweats, and weight loss, collectively known as "B symptoms," is significant in the diagnosis of lymphomas, including Burkitt lymphoma.

Laboratory Findings

  • Lactate Dehydrogenase (LDH): Elevated levels of LDH can indicate tumor burden and are often seen in aggressive lymphomas.
  • Cytogenetic Abnormalities: The presence of the MYC gene rearrangement is a hallmark of Burkitt lymphoma, which can be identified through cytogenetic studies.

Patient Characteristics

Demographics

  • Age: Burkitt lymphoma predominantly affects children and young adults, particularly those aged 5 to 18 years, but it can also occur in adults.
  • Gender: There is a slight male predominance in the incidence 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 forms of Burkitt lymphoma are more common in certain regions, particularly in Africa, where it is associated with malaria and Epstein-Barr virus (EBV) infection.

Clinical Course

Burkitt lymphoma is characterized by a rapid clinical course, necessitating prompt diagnosis and treatment. The aggressive nature of the disease often leads to a high tumor burden at presentation, which can complicate management.

Conclusion

Burkitt lymphoma, particularly when involving the lymph nodes of the inguinal region and lower limbs, presents with a combination of localized and systemic symptoms. Early recognition of the clinical signs, such as swollen lymph nodes and systemic "B symptoms," is essential for effective management. Given its aggressive nature, understanding the patient demographics and risk factors can aid healthcare providers in identifying at-risk individuals and facilitating timely intervention.

Approximate Synonyms

Burkitt lymphoma, specifically coded as ICD-10 code C83.75, refers to a type of non-Hodgkin lymphoma characterized by rapid growth and a high proliferation index. This particular code denotes Burkitt lymphoma that affects the lymph nodes in the inguinal region and lower limb. 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 is used to describe the form of Burkitt lymphoma that is prevalent in certain regions, particularly in Africa, often associated with Epstein-Barr virus (EBV) infection.
  3. Sporadic Burkitt Lymphoma: This variant occurs less frequently and is not geographically restricted, typically seen in Western countries.
  4. AIDS-related Burkitt Lymphoma: This term refers to cases of Burkitt lymphoma that occur in patients with HIV/AIDS, where the immune system is compromised.
  1. Non-Hodgkin Lymphoma (NHL): Burkitt lymphoma is classified under the broader category of non-Hodgkin lymphomas, which encompasses various types of lymphoid malignancies.
  2. Lymphoproliferative Disorders: This term refers to a group of conditions, including Burkitt lymphoma, characterized by the excessive production of lymphocytes.
  3. Lymphadenopathy: This term describes the enlargement of lymph nodes, which is a common symptom in patients with Burkitt lymphoma.
  4. B-cell Lymphoma: Burkitt lymphoma is a type of B-cell lymphoma, indicating that it originates from B lymphocytes, a type of white blood cell.
  5. C83.7: This is a related ICD-10 code for Burkitt lymphoma that does not specify the site, indicating a more general classification.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.75 is crucial for accurate diagnosis, treatment planning, and communication among healthcare professionals. Burkitt lymphoma, with its various forms and classifications, highlights the complexity of lymphoid malignancies and the importance of precise coding in medical records. If you need further information on treatment options or clinical guidelines related to Burkitt lymphoma, feel free to ask!

Diagnostic Criteria

Burkitt lymphoma, classified under ICD-10 code C83.75, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. The diagnosis of Burkitt lymphoma, particularly when it involves the lymph nodes of the inguinal region and lower limb, follows specific clinical, histological, and cytogenetic criteria. Below is a detailed overview of the diagnostic criteria used for this condition.

Clinical Presentation

  1. Symptoms: Patients may present with:
    - Swollen lymph nodes, particularly in the inguinal region.
    - Abdominal pain or swelling if the abdomen is involved.
    - Systemic symptoms such as fever, night sweats, and weight loss (B symptoms).

  2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy, hepatosplenomegaly, or any other signs of systemic involvement.

Histological Criteria

  1. Biopsy: A definitive diagnosis requires a biopsy of the affected lymph node. The histological examination typically reveals:
    - A diffuse infiltrate of medium-sized lymphoid cells.
    - High mitotic activity, often with a "starry sky" appearance due to interspersed macrophages.

  2. Immunophenotyping: Flow cytometry or immunohistochemistry is used to characterize the lymphoma cells. Key markers include:
    - Positive for CD19, CD20, and CD10.
    - Negative for CD5 and CD23, which helps differentiate Burkitt lymphoma from other types of B-cell lymphomas.

Cytogenetic Criteria

  1. Genetic Testing: The presence of specific chromosomal abnormalities is crucial for diagnosis:
    - The most common genetic alteration is the translocation involving the MYC gene, typically t(8;14), which leads to overexpression of the MYC oncogene.

  2. Molecular Studies: PCR or FISH (Fluorescence In Situ Hybridization) can be employed to detect MYC rearrangements, confirming the diagnosis of Burkitt lymphoma.

Staging and Imaging

  1. Imaging Studies: Imaging techniques such as CT scans or PET scans are utilized to assess the extent of the disease, particularly to evaluate lymph node involvement and any potential extranodal sites.

  2. Staging: The Ann Arbor staging system is commonly used to determine the extent of the disease, which is critical for treatment planning.

Conclusion

The diagnosis of Burkitt lymphoma, particularly in the inguinal region and lower limb, involves a combination of clinical evaluation, histological examination, immunophenotyping, and cytogenetic analysis. Early and accurate diagnosis is essential due to the aggressive nature of this lymphoma and the need for prompt treatment. If you have further questions or need additional information on treatment options or prognosis, feel free to ask!

Treatment Guidelines

Burkitt lymphoma, classified under ICD-10 code C83.75, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. It primarily affects lymphoid tissues, including lymph nodes, and can manifest in various anatomical locations, including the inguinal region and lower limbs. The treatment for Burkitt lymphoma typically involves a combination of chemotherapy, immunotherapy, and, in some cases, radiation therapy. Below is a detailed overview of the standard treatment approaches for this condition.

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.

  • R-CHOP: While not the first-line treatment for classic 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 not candidates for more intensive regimens.

2. Immunotherapy

Immunotherapy plays a significant role in the management of Burkitt lymphoma, particularly with the use of monoclonal antibodies:

  • Rituximab: This anti-CD20 monoclonal antibody is often included in treatment regimens to target B-cell malignancies. It can enhance the effectiveness of chemotherapy and improve overall outcomes.

3. Radiation Therapy

Radiation therapy may be utilized in specific scenarios, particularly for localized disease or to manage symptoms. However, it is not typically the primary treatment modality for Burkitt lymphoma due to the aggressive nature of the disease and the need for systemic therapy.

4. Stem Cell Transplantation

For patients with high-risk or relapsed Burkitt lymphoma, autologous stem cell transplantation may be considered. This approach involves harvesting the patient's stem cells, administering high-dose chemotherapy to eradicate the disease, 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 treatment regimens. This includes:

  • Management of Tumor Lysis Syndrome: Due to the rapid cell turnover, patients are at risk for tumor lysis syndrome, which requires careful monitoring and management of electrolytes.

  • Infection Prophylaxis: Given the immunocompromised state from chemotherapy, prophylactic antibiotics and antifungals may be necessary.

Conclusion

The treatment of Burkitt lymphoma, particularly when it involves the lymph nodes of the inguinal region and lower limbs, is aggressive and multifaceted. The standard approach primarily revolves around intensive chemotherapy regimens, often supplemented with immunotherapy and, in select cases, radiation therapy or stem cell transplantation. Close monitoring and supportive care are essential to manage the side effects and complications associated with treatment. As with any cancer treatment, individual patient factors and disease characteristics will guide the specific therapeutic strategy employed.

Related Information

Description

  • Aggressive form of non-Hodgkin lymphoma
  • Rapid proliferation of B-lymphoid cells
  • Localized in inguinal region and lower limb
  • Enlarged lymph nodes in abdomen, neck or inguinal region
  • Abdominal pain or swelling due to lymph node involvement
  • Fever, night sweats, weight loss, fatigue common symptoms

Clinical Information

  • Fast proliferation of cancer cells
  • Primarily affects lymphoid tissues
  • Rapid tumor growth and progression
  • Often seen in children and young adults
  • Swollen lymph nodes in groin area
  • Fever, night sweats, and weight loss are common symptoms
  • Elevated lactate dehydrogenase (LDH) levels
  • Presence of MYC gene rearrangement is diagnostic

Approximate Synonyms

  • Burkitt's Lymphoma
  • Endemic Burkitt Lymphoma
  • Sporadic Burkitt Lymphoma
  • AIDS-related Burkitt Lymphoma
  • B-cell Lymphoma

Diagnostic Criteria

  • Symptoms include swollen lymph nodes.
  • Abdominal pain or swelling may be present.
  • Systemic symptoms like fever occur.
  • Histological examination shows diffuse infiltrate.
  • High mitotic activity with starry sky appearance.
  • Positive for CD19, CD20, and CD10 markers.
  • Negative for CD5 and CD23 markers.
  • t(8;14) translocation is common.
  • MYC rearrangements detected by PCR or FISH.

Treatment Guidelines

  • Chemotherapy is cornerstone of Burkitt lymphoma treatment
  • CODOX-M/IVAC is intensive chemotherapy regimen
  • R-CHOP used for less aggressive variants
  • Immunotherapy with Rituximab enhances effectiveness
  • Radiation therapy not primary modality due to aggression
  • Stem cell transplantation considered for high-risk patients
  • Supportive care manages treatment side effects
  • Tumor lysis syndrome requires careful monitoring
  • Infection prophylaxis necessary due to immunocompromised state

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