ICD-10: C83.78

Burkitt lymphoma, lymph nodes of multiple sites

Additional Information

Clinical Information

Burkitt lymphoma, classified under ICD-10 code C83.78, 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

General Overview

Burkitt lymphoma typically presents as a rapidly enlarging mass, often involving lymph nodes, but it can also affect extranodal sites such as the abdomen, jaw, or central nervous system. The disease is most commonly seen in children and young adults, although it can occur at any age.

Signs and Symptoms

  1. Lymphadenopathy: Patients often exhibit significant lymph node enlargement, which may be painless. In the case of C83.78, lymph nodes in multiple sites are affected, leading to generalized lymphadenopathy.

  2. Abdominal Symptoms: If the lymphoma involves abdominal organs, patients may experience abdominal pain, distension, or obstruction due to enlarged lymph nodes or masses. This can lead to symptoms such as nausea, vomiting, or changes in bowel habits.

  3. Facial Swelling: In cases where the jaw is involved, particularly in children, there may be noticeable facial swelling or deformity.

  4. Fever and Night Sweats: Systemic symptoms such as fever, drenching night sweats, and unexplained weight loss are common, indicating a more systemic involvement of the disease.

  5. Fatigue: Patients often report significant fatigue, which can be attributed to the disease itself or anemia resulting from bone marrow involvement.

  6. CNS Symptoms: If the central nervous system is involved, symptoms may include headaches, seizures, or neurological deficits, depending on the location of the lesions.

Patient Characteristics

  • Age: Burkitt lymphoma predominantly affects children and young adults, with a peak incidence in those aged 5 to 10 years. However, it can also occur in adults, particularly in immunocompromised individuals.

  • Gender: There is a slight male predominance in the incidence of Burkitt lymphoma.

  • Immunocompromised Status: Patients with compromised immune systems, such as those with HIV/AIDS, are at increased risk for developing Burkitt lymphoma.

  • Geographic Variability: The endemic form of Burkitt lymphoma is more common in Africa, often associated with Epstein-Barr virus (EBV) infection, particularly in children. The sporadic form is more prevalent in Western countries.

Conclusion

Burkitt lymphoma, particularly in its presentation as C83.78, is marked by rapid progression and a variety of clinical symptoms, primarily involving lymph nodes at multiple sites. Early recognition of the signs and symptoms, along with an understanding of patient demographics, is essential for effective management and treatment. Given its aggressive nature, prompt diagnosis and initiation of therapy are critical to improving patient outcomes.

Approximate Synonyms

Burkitt lymphoma, classified under ICD-10 code C83.78, is a type of non-Hodgkin lymphoma characterized by the rapid growth of tumors, often affecting lymph nodes. 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 when it involves lymph nodes of multiple sites.

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: Refers to the form of Burkitt lymphoma that is commonly found in equatorial Africa, often associated with Epstein-Barr virus (EBV) infection.
  3. Sporadic Burkitt Lymphoma: This term describes cases that occur outside of endemic regions and are not linked to EBV as frequently.
  4. AIDS-related Burkitt Lymphoma: This variant occurs in patients with HIV/AIDS and is characterized by a more aggressive clinical course.
  1. Non-Hodgkin Lymphoma (NHL): Burkitt lymphoma is a subtype of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers.
  2. Lymphoproliferative Disorders: This broader category includes various conditions where lymphocytes (a type of white blood cell) proliferate abnormally, including Burkitt lymphoma.
  3. B-cell Lymphoma: Since Burkitt lymphoma originates from B-cells, it is often classified under B-cell lymphomas.
  4. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with Burkitt lymphoma.
  5. C83.7: This is the more general ICD-10 code for Burkitt lymphoma, which may be used when specifying lymph nodes of multiple sites is not necessary.

Clinical Context

Burkitt lymphoma is notable for its aggressive nature and rapid progression, often requiring prompt diagnosis and treatment. The classification into endemic and sporadic forms helps in understanding the epidemiology and potential etiological factors associated with the disease. The use of specific ICD-10 codes, such as C83.78, aids healthcare providers in accurately documenting and billing for the condition, ensuring appropriate care and resource allocation.

In summary, recognizing the alternative names and related terms for ICD-10 code C83.78 can facilitate better communication among healthcare professionals and improve patient care strategies.

Diagnostic Criteria

Burkitt lymphoma, classified under ICD-10 code C83.78, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid cell proliferation. Diagnosing Burkitt lymphoma involves a combination of clinical evaluation, imaging studies, laboratory tests, and histopathological examination. Below are the key criteria and steps used in the diagnosis of this condition.

Clinical Presentation

Symptoms

Patients with Burkitt lymphoma may present with:
- Lymphadenopathy: Swollen lymph nodes, often in multiple sites, which is a hallmark of the disease.
- Abdominal Pain or Distension: Due to lymph node enlargement in the abdomen.
- Fever, Night Sweats, and Weight Loss: These systemic symptoms may also be present, indicating a more advanced disease state.

Physical Examination

A thorough physical examination is essential to assess the extent of lymphadenopathy and any other systemic signs of lymphoma.

Imaging Studies

Radiological Evaluation

  • CT Scans: Computed tomography scans of the chest, abdomen, and pelvis are commonly used to identify enlarged lymph nodes and assess the extent of disease.
  • PET Scans: Positron emission tomography can help evaluate metabolic activity in lymph nodes and other tissues, aiding in staging and treatment planning.

Laboratory Tests

Blood Tests

  • Complete Blood Count (CBC): May show anemia, thrombocytopenia, or leukopenia, although these findings can vary.
  • Lactate Dehydrogenase (LDH): Elevated levels can indicate a higher tumor burden and are often associated with aggressive lymphomas.

Cytogenetic and Molecular Studies

  • Cytogenetic Analysis: Detection of the characteristic chromosomal translocation t(8;14), which involves the MYC gene, is crucial for confirming the diagnosis of Burkitt lymphoma.
  • Flow Cytometry: This technique can be used to analyze the surface markers on lymphocytes, typically showing a B-cell phenotype with specific markers such as CD19, CD20, and CD10.

Histopathological Examination

Biopsy

  • Tissue Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph node or tissue. Histological examination typically reveals a high proliferation of atypical B-lymphoid cells with a "starry sky" appearance due to interspersed macrophages.
  • Immunohistochemistry: This is used to confirm the B-cell lineage and assess the expression of MYC protein, which is often overexpressed in Burkitt lymphoma.

Diagnostic Criteria Summary

To summarize, the diagnosis of Burkitt lymphoma (ICD-10 code C83.78) is based on:
- Clinical symptoms and physical examination findings.
- Imaging studies to assess lymph node involvement.
- Laboratory tests, including blood work and cytogenetic analysis.
- Histopathological confirmation through biopsy and immunohistochemical staining.

Conclusion

The diagnosis of Burkitt lymphoma requires a comprehensive approach that integrates clinical, radiological, laboratory, and histopathological data. Early and accurate diagnosis is critical for effective treatment and management of this aggressive lymphoma. If you have further questions or need more specific information, feel free to ask!

Treatment Guidelines

Burkitt lymphoma, classified under ICD-10 code C83.78, is a highly aggressive form of non-Hodgkin lymphoma characterized by rapid growth and a tendency to affect lymph nodes, particularly in multiple sites. The standard treatment approaches for Burkitt lymphoma typically involve a combination of chemotherapy, immunotherapy, and, in some cases, stem cell transplantation. Below is a detailed overview of these treatment modalities.

Chemotherapy

Intensive Chemotherapy Regimens

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 (Adriamycin), and Methotrexate (CODOX-M) with Ifosfamide, Etoposide, and Cytarabine (IVAC). This approach is designed to deliver high doses of chemotherapy to effectively target the rapidly dividing cancer cells[1].

  • Hyper-CVAD: Another widely used regimen is Hyper-CVAD, which alternates cycles of high-dose Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone with high-dose Methotrexate and Cytarabine. This regimen is particularly effective in younger patients and those with good performance status[2].

Supportive Care

Due to the intensity of these regimens, supportive care is crucial. This includes the management of side effects such as febrile neutropenia, infections, and tumor lysis syndrome, which can occur due to the rapid destruction of cancer cells[3].

Immunotherapy

Monoclonal Antibodies

Immunotherapy has become an integral part of the treatment landscape for Burkitt lymphoma. The use of monoclonal antibodies, such as Rituximab, can enhance the effectiveness of chemotherapy. Rituximab targets the CD20 antigen on B-cells, which is often expressed in Burkitt lymphoma, and can improve outcomes when used in combination with chemotherapy[4].

CAR T-Cell Therapy

Chimeric Antigen Receptor (CAR) T-cell therapy is an emerging treatment option for relapsed or refractory Burkitt lymphoma. This personalized therapy involves modifying a patient’s T-cells to better recognize and attack cancer cells. While still under investigation, CAR T-cell therapy has shown promising results in clinical trials[5].

Stem Cell Transplantation

For patients with high-risk Burkitt lymphoma or those who experience relapse after initial treatment, autologous stem cell transplantation (ASCT) may be considered. This procedure involves harvesting the patient’s own stem cells, administering high-dose chemotherapy to eradicate the lymphoma, and then reinfusing the stem cells to restore bone marrow function[6].

Conclusion

The treatment of Burkitt lymphoma (ICD-10 code C83.78) is multifaceted, primarily relying on intensive chemotherapy regimens, often supplemented with immunotherapy and, in select cases, stem cell transplantation. Given the aggressive nature of this lymphoma, timely diagnosis and initiation of treatment are critical for improving patient outcomes. Ongoing research continues to explore novel therapies and combinations to enhance efficacy and reduce toxicity, making it an active area of clinical investigation.

For patients diagnosed with Burkitt lymphoma, it is essential to work closely with a healthcare team specializing in hematologic malignancies to determine the most appropriate treatment plan tailored to individual needs and circumstances.

Description

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma characterized by the rapid proliferation of B-lymphoid cells. The ICD-10 code C83.78 specifically refers to Burkitt lymphoma affecting the lymph nodes of multiple sites. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Burkitt Lymphoma

Overview

Burkitt lymphoma is classified as a type of B-cell non-Hodgkin lymphoma and 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 a chromosomal translocation involving the MYC gene, which plays a crucial role in cell proliferation and growth.

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 with jaw lesions in children.
2. Sporadic Burkitt Lymphoma: More prevalent in non-endemic regions, this type often presents with abdominal masses and can involve the gastrointestinal tract.
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:
- Swollen lymph nodes, particularly in the neck, abdomen, or groin.
- Abdominal pain or swelling due to enlarged lymph nodes or masses.
- Fever, night sweats, and unexplained weight loss.
- Fatigue and weakness.

Diagnosis

Diagnosis typically involves:
- Physical Examination: Assessment of lymphadenopathy and organomegaly.
- Imaging Studies: CT scans or MRIs to evaluate the extent of lymph node involvement and any abdominal masses.
- Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph nodes, which reveals characteristic histological features, including a high proliferation index and the presence of starry sky appearance due to interspersed macrophages.

Treatment

The treatment for Burkitt lymphoma is aggressive and often includes:
- Chemotherapy: Intensive multi-agent chemotherapy regimens are the cornerstone of treatment.
- Immunotherapy: In some cases, monoclonal antibodies may be used to target specific cancer cells.
- Stem Cell Transplant: For relapsed or refractory cases, hematopoietic stem cell transplantation may be considered.

ICD-10 Code C83.78 Details

The ICD-10 code C83.78 specifically denotes Burkitt lymphoma affecting lymph nodes at multiple sites. This classification is crucial for accurate billing and coding in healthcare settings, ensuring that patients receive appropriate treatment and that healthcare providers are reimbursed correctly for their services.

  • C83.7: This code refers to Burkitt lymphoma affecting lymph nodes but does not specify multiple sites.
  • C83.79: This code is used for Burkitt lymphoma affecting other specified sites.

Conclusion

Burkitt lymphoma, particularly when coded as C83.78, indicates a serious condition 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. Accurate coding is vital for effective patient management and healthcare reimbursement processes.

Related Information

Clinical Information

  • Rapidly enlarging mass
  • Lymph node enlargement in multiple sites
  • Abdominal pain or distension
  • Facial swelling or deformity
  • Fever and night sweats
  • Significant fatigue
  • CNS symptoms such as headaches
  • Predominantly affects children and young adults
  • Slight male predominance in incidence
  • Increased risk for immunocompromised patients

Approximate Synonyms

  • Burkitt's Lymphoma
  • Endemic Burkitt Lymphoma
  • Sporadic Burkitt Lymphoma
  • AIDS-related Burkitt Lymphoma
  • Non-Hodgkin Lymphoma (NHL)
  • Lymphoproliferative Disorders
  • B-cell Lymphoma
  • Lymphadenopathy

Diagnostic Criteria

  • Lymphadenopathy in multiple sites
  • Abdominal pain or distension
  • Fever, night sweats, and weight loss
  • CT scans of chest, abdomen, and pelvis
  • PET scans for metabolic activity
  • Elevated LDH levels
  • t(8;14) chromosomal translocation
  • B-cell phenotype with CD19, CD20, and CD10 markers
  • High proliferation of atypical B-lymphoid cells
  • Starry sky appearance on histology

Treatment Guidelines

  • Intensive chemotherapy regimens first line
  • CODOX-M/IVAC regimen effective against Burkitt lymphoma
  • Hyper-CVAD effective in younger patients with good performance status
  • Supportive care crucial due to high toxicity
  • Immunotherapy enhances effectiveness of chemotherapy
  • Monoclonal antibodies like Rituximab target CD20 antigen
  • CAR T-cell therapy emerging treatment option for relapsed cases
  • Autologous stem cell transplantation for high-risk patients

Description

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