ICD-10: C83.7A

Burkitt lymphoma, in remission

Additional Information

Description

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-cells. The ICD-10-CM code C83.7A specifically refers to Burkitt lymphoma that is currently in remission. Understanding this diagnosis involves several key aspects, including clinical features, treatment options, and the implications of remission.

Clinical Features of Burkitt Lymphoma

Burkitt lymphoma is known for its distinct clinical presentation, which may include:

  • Rapid Tumor Growth: Patients often present with a rapidly enlarging mass, typically in the abdomen, lymph nodes, or jaw.
  • B Symptoms: These may include fever, night sweats, and weight loss, which are common in many lymphomas.
  • Cytogenetic Abnormalities: The hallmark of Burkitt lymphoma is the translocation involving the MYC gene, often t(8;14), which plays a crucial role in its pathogenesis.

Diagnosis

Diagnosis of Burkitt lymphoma involves a combination of clinical evaluation, imaging studies, and histopathological examination. Key diagnostic tools include:

  • Biopsy: A tissue sample is essential for confirming the diagnosis through microscopic examination.
  • Immunophenotyping: This helps in identifying the specific type of lymphoma based on the surface markers present on the cells.
  • Cytogenetic Analysis: Detecting the MYC translocation is critical for confirming Burkitt lymphoma.

Treatment Options

The treatment of Burkitt lymphoma is aggressive due to its rapid progression. Common treatment modalities include:

  • Chemotherapy: Intensive multi-agent chemotherapy regimens, such as the CODOX-M/IVAC protocol, are standard.
  • Immunotherapy: Rituximab, a monoclonal antibody targeting CD20, may be used, especially in cases with CD20-positive cells.
  • Stem Cell Transplantation: In some cases, particularly for relapsed or refractory disease, autologous stem cell transplantation may be considered.

Remission and Its Implications

The term "in remission" (C83.7A) indicates that the signs and symptoms of Burkitt lymphoma have significantly decreased or are no longer detectable following treatment. This status is crucial for several reasons:

  • Monitoring: Patients in remission require regular follow-up to monitor for potential relapse, which can occur even after successful initial treatment.
  • Quality of Life: Achieving remission often leads to an improvement in the patient's quality of life, as symptoms resolve and normal activities can be resumed.
  • Long-term Outcomes: While many patients achieve remission, ongoing research is focused on understanding long-term outcomes and potential late effects of treatment.

Conclusion

ICD-10 code C83.7A for Burkitt lymphoma in remission reflects a critical phase in the management of this aggressive lymphoma. Understanding the clinical features, diagnostic criteria, treatment options, and implications of remission is essential for healthcare providers managing patients with this condition. Continuous monitoring and supportive care are vital to ensure the best possible outcomes for patients who have achieved remission.

Clinical Information

Burkitt lymphoma, classified under ICD-10 code C83.7A, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for effective diagnosis and management.

Clinical Presentation

Overview

Burkitt lymphoma typically presents with a rapidly enlarging mass, often in the abdomen, lymph nodes, or jaw. The aggressive nature of this lymphoma necessitates prompt recognition and treatment to improve patient outcomes.

Common Sites of Involvement

  • Abdominal Mass: Often presents as an abdominal tumor, which may lead to symptoms of bowel obstruction or abdominal pain.
  • Lymphadenopathy: Enlarged lymph nodes, particularly in the cervical, axillary, or inguinal regions.
  • Jaw Lesions: Particularly in endemic forms, jaw lesions are common, especially in children.
  • Central Nervous System (CNS): Involvement can lead to neurological symptoms, including headaches, seizures, or altered mental status.

Signs and Symptoms

General Symptoms

  • Fever: Often low-grade but can be persistent.
  • Night Sweats: Commonly reported by patients.
  • Weight Loss: Unintentional weight loss is frequently observed.
  • Fatigue: Generalized fatigue and malaise are common complaints.

Specific Symptoms

  • Abdominal Pain: Due to mass effect or organ involvement.
  • Nausea and Vomiting: May occur if the gastrointestinal tract is involved.
  • Respiratory Symptoms: If there is mediastinal involvement, patients may experience cough or difficulty breathing.
  • Neurological Symptoms: Headaches, visual disturbances, or other CNS-related symptoms if the lymphoma has spread to the brain.

Patient Characteristics

Demographics

  • Age: Burkitt lymphoma predominantly affects children and young adults, although it can occur at any age.
  • 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.
  • Geographic Variability: Endemic Burkitt lymphoma is more common in certain regions, particularly in Africa, often associated with malaria and Epstein-Barr virus (EBV) infection.

Prognosis and Remission

Patients diagnosed with Burkitt lymphoma can achieve remission with appropriate treatment, which typically includes intensive chemotherapy. The ICD-10 code C83.7A specifically denotes cases where the patient is in remission, indicating a favorable response to treatment and a reduction in disease activity.

Conclusion

Burkitt lymphoma, particularly in its aggressive form, presents with a range of clinical signs and symptoms that require immediate attention. Understanding the typical clinical presentation, including common sites of involvement and patient demographics, is essential for healthcare providers. Early diagnosis and treatment are critical for achieving remission and improving patient outcomes. Regular follow-up is necessary to monitor for any signs of recurrence, especially in patients previously diagnosed with this aggressive lymphoma.

Approximate Synonyms

Burkitt lymphoma, classified under the ICD-10 code C83.7A, is a type of aggressive B-cell non-Hodgkin lymphoma. When discussing this condition, several alternative names and related terms can be useful for understanding its context and implications in medical coding and treatment. Below are some of the key terms associated with Burkitt lymphoma, particularly in the context of remission.

Alternative Names for Burkitt Lymphoma

  1. Burkitt's Lymphoma: This is a common variation in naming, often used interchangeably with Burkitt lymphoma.
  2. Burkitt's Disease: This term is sometimes used to refer to the same condition, emphasizing its classification as a disease.
  3. Mature B-cell Leukemia, Burkitt-type: This term may be used in specific contexts, particularly when discussing the leukemic form of Burkitt lymphoma.
  1. Non-Hodgkin Lymphoma (NHL): Burkitt lymphoma is a subtype of non-Hodgkin lymphoma, which encompasses a wide range of lymphoid malignancies.
  2. B-cell Lymphoma: Since Burkitt lymphoma originates from B-cells, this term is relevant in describing its cellular origin.
  3. Aggressive Lymphoma: Burkitt lymphoma is known for its rapid progression, making it an aggressive form of lymphoma.
  4. Lymphoproliferative Disorders: This broader category includes various conditions characterized by the proliferation of lymphocytes, including Burkitt lymphoma.
  5. Minimal Residual Disease (MRD): In the context of remission, MRD refers to the small number of cancer cells that may remain after treatment, which is crucial for monitoring the disease's status.

Clinical Context

In clinical practice, the term "in remission" indicates that the signs and symptoms of Burkitt lymphoma have significantly decreased or are no longer detectable. This status is critical for treatment planning and ongoing patient management. The ICD-10 code C83.7A specifically denotes Burkitt lymphoma in remission, which is essential for accurate medical billing and coding.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.7A is vital for healthcare professionals involved in the diagnosis, treatment, and coding of Burkitt lymphoma. These terms not only facilitate communication among medical practitioners but also enhance the clarity of patient records and billing processes. If you have further questions or need additional information on this topic, feel free to ask!

Diagnostic Criteria

Burkitt lymphoma, classified under ICD-10 code C83.7A, is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-cells. The diagnosis of Burkitt lymphoma, particularly when indicating remission, involves a combination of clinical evaluation, laboratory tests, and imaging studies. Below are the key criteria used for diagnosis:

Clinical Presentation

  1. Symptoms: Patients may present with symptoms such as:
    - Swollen lymph nodes (often in the abdomen, neck, or groin)
    - Abdominal pain or swelling
    - Fever
    - Night sweats
    - Unexplained weight loss
    - Fatigue

  2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy or splenomegaly, which are common in Burkitt lymphoma cases.

Laboratory Tests

  1. 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.

  2. Bone Marrow Biopsy: This procedure is performed to assess the involvement of the bone marrow by lymphoma cells. In Burkitt lymphoma, the bone marrow may show a high percentage of atypical lymphoid cells.

  3. 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).

Imaging Studies

  1. Computed Tomography (CT) Scan: A CT scan of the chest, abdomen, and pelvis is often performed to identify the extent of lymphadenopathy and any organ involvement.

  2. Positron Emission Tomography (PET) Scan: This imaging modality can help assess metabolic activity and determine the response to treatment, particularly in evaluating remission status.

Histopathological Examination

  1. Tissue Biopsy: A definitive diagnosis of Burkitt lymphoma is made through a biopsy of an affected lymph node or mass. Histological examination typically reveals:
    - A high proliferation index (often >90% Ki-67 positivity)
    - A characteristic "starry sky" appearance due to interspersed macrophages among the neoplastic cells.

  2. Immunophenotyping: Flow cytometry or immunohistochemistry is used to confirm the B-cell lineage of the lymphoma cells, typically showing positivity for CD19, CD20, and CD10, while being negative for CD5 and CD23.

Criteria for Remission

To classify Burkitt lymphoma as being in remission, the following criteria are generally considered:

  1. Clinical Evaluation: Absence of symptoms and physical signs of disease.
  2. Imaging Studies: No evidence of residual disease on CT or PET scans.
  3. Laboratory Tests: Normalization of blood counts and LDH levels.
  4. Bone Marrow Biopsy: No evidence of lymphoma cells in the bone marrow.

In summary, the diagnosis of Burkitt lymphoma (ICD-10 code C83.7A) and the determination of remission status involve a comprehensive approach that includes clinical assessment, laboratory tests, imaging studies, and histopathological evaluation. Each of these components plays a crucial role in ensuring accurate diagnosis and effective management of the disease.

Treatment Guidelines

Burkitt lymphoma, classified under ICD-10 code C83.7A, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. The treatment approaches for Burkitt lymphoma, particularly when the patient is in remission, focus on maintaining that remission and preventing relapse. Here’s a detailed overview of the standard treatment strategies:

Initial Treatment of Burkitt Lymphoma

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) with Ifosfamide, Etoposide, and Cytarabine (IVAC). This approach is designed to achieve rapid tumor reduction and is typically administered in cycles over several months[1].

  • Hyper-CVAD: Another effective regimen is Hyper-CVAD, which includes high doses of Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone, followed by high-dose Methotrexate and Cytarabine. This regimen is also delivered in alternating cycles and is known for its efficacy in treating aggressive lymphomas[2].

Targeted Therapy

In some cases, targeted therapies may be considered, especially if the lymphoma is associated with specific genetic markers. For instance, the use of monoclonal antibodies like Rituximab can be beneficial, although its role in Burkitt lymphoma is less established compared to other types of non-Hodgkin lymphoma[3].

Maintenance Therapy

Once a patient achieves remission, maintenance therapy may be employed to prolong the duration of remission. This can include:

  • Low-Dose Chemotherapy: Some regimens may involve lower doses of chemotherapy agents to reduce the risk of relapse while minimizing toxicity[4].

  • Immunotherapy: Emerging treatments, including CAR T-cell therapy, are being explored for patients in remission to enhance the immune response against any residual lymphoma cells[5].

Monitoring and Follow-Up

Regular follow-up is crucial for patients in remission from Burkitt lymphoma. This typically involves:

  • Imaging Studies: Periodic PET scans or CT scans to monitor for any signs of relapse.
  • Blood Tests: Regular complete blood counts and other relevant tests to assess overall health and detect any abnormalities early[6].

Conclusion

The management of Burkitt lymphoma, particularly in remission, requires a comprehensive approach that includes intensive initial chemotherapy, potential maintenance therapy, and vigilant monitoring. The goal is to maintain remission and prevent relapse, utilizing both traditional chemotherapy and emerging therapies as appropriate. As research continues, treatment protocols may evolve, emphasizing the importance of personalized medicine in the management of this aggressive lymphoma.

For further information or specific treatment plans, consulting with a hematologist or oncologist specializing in lymphomas is recommended.

Related Information

Description

  • Highly aggressive non-Hodgkin lymphoma
  • Rapid proliferation of B-cells
  • Typically presents with abdominal mass
  • Fever, night sweats, weight loss common
  • Translocation involving MYC gene critical
  • Intensive chemotherapy and immunotherapy used
  • Regular monitoring for potential relapse

Clinical Information

  • Rapidly enlarging abdominal mass
  • Enlarged lymph nodes
  • Jaw lesions common in endemic forms
  • Central Nervous System involvement common
  • Fever, night sweats and weight loss symptoms
  • Abdominal pain and nausea vomiting specific symptoms
  • Respiratory symptoms if mediastinal involved
  • Neurological symptoms if CNS affected
  • Predominantly affects children and young adults
  • Male predominance in some cases
  • Increased risk with immunocompromised state
  • Geographic variability in endemic forms

Approximate Synonyms

Diagnostic Criteria

  • Swollen lymph nodes in abdomen, neck, or groin
  • Abdominal pain or swelling
  • Fever
  • Night sweats
  • Unexplained weight loss
  • Fatigue
  • Elevated LDH levels
  • Bone marrow involvement by lymphoma cells
  • MYC gene rearrangement on cytogenetic analysis
  • High proliferation index (Ki-67 >90%)
  • Starry sky appearance on histological examination
  • B-cell lineage confirmed by immunophenotyping

Treatment Guidelines

  • Intensive chemotherapy cornerstone
  • CODOX-M/IVAC regimen used for rapid tumor reduction
  • Hyper-CVAD effective against aggressive lymphomas
  • Targeted therapy with monoclonal antibodies considered
  • Low-dose chemotherapy to reduce relapse risk
  • Immunotherapy explored for CAR T-cell therapy
  • Regular imaging and blood tests for monitoring

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