ICD-10: C83.89

Other non-follicular lymphoma, extranodal and solid organ sites

Additional Information

Treatment Guidelines

When addressing the standard treatment approaches for ICD-10 code C83.89, which refers to "Other non-follicular lymphoma, extranodal and solid organ sites," it is essential to understand the nature of this condition and the various treatment modalities available. Non-follicular lymphomas encompass a diverse group of lymphoproliferative disorders that do not fall under the category of follicular lymphoma, and they can manifest in extranodal sites, including solid organs.

Overview of Non-Follicular Lymphoma

Non-follicular lymphomas are typically aggressive and can arise in various locations outside the lymphatic system, such as the gastrointestinal tract, skin, and other solid organs. The treatment approach often depends on several factors, including the specific subtype of lymphoma, the stage of the disease, the patient's overall health, and their preferences.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone of treatment for many types of non-follicular lymphoma. Common regimens may include:

  • CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. It is frequently used for aggressive lymphomas and can be adapted based on the patient's response and tolerance.
  • R-CHOP: The addition of Rituximab (a monoclonal antibody) to the CHOP regimen has been shown to improve outcomes in many patients with B-cell lymphomas, including some non-follicular types[1].

2. Targeted Therapy

Targeted therapies have emerged as effective options for certain subtypes of non-follicular lymphoma. For instance:

  • Ibrutinib (Imbruvica): This Bruton's tyrosine kinase inhibitor is used for specific types of B-cell lymphomas and may be considered for patients with relapsed or refractory disease[2].
  • Other targeted agents: Depending on the specific characteristics of the lymphoma, other agents may be utilized, such as Idelalisib or Venetoclax, particularly in cases with specific genetic mutations or markers[3].

3. Radiation Therapy

Radiation therapy can be an effective treatment for localized non-follicular lymphomas, especially when the disease is confined to a specific extranodal site. It may be used as:

  • Primary treatment: For localized disease, radiation can be curative.
  • Adjuvant therapy: Following chemotherapy to eliminate residual disease and reduce the risk of recurrence[4].

4. Stem Cell Transplantation

For patients with aggressive non-follicular lymphoma, particularly those who are younger and have a good performance status, high-dose chemotherapy followed by autologous stem cell transplantation may be considered. This approach is typically reserved for patients who have achieved remission after initial treatment but are at high risk for relapse[5].

5. Clinical Trials

Participation in clinical trials may also be an option for patients with non-follicular lymphoma. These trials often explore new therapies or combinations of existing treatments and can provide access to cutting-edge care[6].

Conclusion

The treatment of non-follicular lymphoma, particularly those classified under ICD-10 code C83.89, is multifaceted and tailored to the individual patient. Standard approaches typically involve a combination of chemotherapy, targeted therapies, radiation, and potentially stem cell transplantation, depending on the specific characteristics of the lymphoma and the patient's overall health. As research continues to evolve, new treatment modalities and clinical trials may offer additional options for patients facing this challenging diagnosis. It is crucial for patients to discuss their treatment options with a healthcare provider specializing in hematology or oncology to determine the best course of action tailored to their specific situation.


References

  1. Billing and Coding: Intensity Modulated Radiation Therapy.
  2. Subject: Ibrutinib (Imbruvica®) - Medical Coverage Guideline.
  3. Medical Drug Clinical Criteria.
  4. HCT for Non-Hodgkin Lymphoma.
  5. Medical Necessity Tool for Flow Cytometry.
  6. CMS Manual System.

Description

ICD-10 code C83.89 refers to "Other non-follicular lymphoma, extranodal and solid organ sites." This classification is part of the broader category of non-Hodgkin lymphomas (NHL), which are a diverse group of blood cancers that include various types of lymphomas that do not originate from follicular (germinal center) cells.

Clinical Description

Definition

Non-follicular lymphomas are characterized by their origin from lymphoid tissue that is not part of the follicular structure. The term "extranodal" indicates that the lymphoma occurs outside of the lymph nodes, affecting other organs or tissues. This can include a variety of sites such as the gastrointestinal tract, skin, central nervous system, and other solid organs.

Types of Non-Follicular Lymphomas

The category of non-follicular lymphomas encompasses several subtypes, including but not limited to:
- Diffuse Large B-Cell Lymphoma (DLBCL): The most common subtype of non-Hodgkin lymphoma, which can present in extranodal sites.
- Mantle Cell Lymphoma: Often presents with extranodal involvement and can affect solid organs.
- Peripheral T-Cell Lymphoma: A heterogeneous group of aggressive lymphomas that can also manifest in extranodal sites.

Symptoms

Symptoms of extranodal non-follicular lymphoma can vary widely depending on the site of involvement but may include:
- Localized swelling: In the affected organ or tissue.
- Systemic symptoms: Such as fever, night sweats, and unexplained weight loss.
- Organ-specific symptoms: For example, gastrointestinal symptoms if the lymphoma involves the digestive tract, or neurological symptoms if the central nervous system is affected.

Diagnosis

Diagnosis typically involves a combination of:
- Imaging studies: Such as CT scans, PET scans, or MRI to identify the extent of the disease.
- Biopsy: To obtain tissue samples for histological examination, confirming the type of lymphoma.
- Immunophenotyping: To determine the specific subtype of lymphoma based on cell surface markers.

Treatment

Treatment options for C83.89 may include:
- Chemotherapy: Often the first line of treatment, especially for aggressive forms of lymphoma.
- Radiation therapy: May be used in localized disease or as part of a combined modality approach.
- Targeted therapies: Such as monoclonal antibodies (e.g., Rituximab) that target specific markers on lymphoma cells.
- Stem cell transplant: In certain cases, particularly for relapsed or refractory disease.

Conclusion

ICD-10 code C83.89 captures a critical aspect of non-Hodgkin lymphoma, specifically focusing on cases that present in extranodal and solid organ sites. Understanding the clinical characteristics, diagnostic approaches, and treatment options for this category of lymphoma is essential for effective patient management. As with all cancers, early detection and tailored treatment strategies are vital for improving patient outcomes.

Approximate Synonyms

ICD-10 code C83.89 refers to "Other non-follicular lymphoma, extranodal and solid organ sites." This classification encompasses various types of non-Hodgkin lymphoma that do not fall under the follicular category and are primarily located in extranodal sites or solid organs. Below are alternative names and related terms associated with this code.

Alternative Names

  1. Extranodal Non-Hodgkin Lymphoma: This term emphasizes the lymphoma's occurrence outside of lymph nodes, which is a key characteristic of the conditions classified under C83.89.

  2. Non-Follicular Lymphoma: This broader term includes all types of non-follicular lymphomas, which can be either nodal or extranodal.

  3. Aggressive Non-Hodgkin Lymphoma: Some of the lymphomas classified under C83.89 may be aggressive in nature, although this term is more general and can apply to various types of non-Hodgkin lymphoma.

  4. Primary Extranodal Lymphoma: This term is often used to describe lymphomas that originate in extranodal sites, highlighting their primary location.

  5. Solid Organ Lymphoma: This term refers specifically to lymphomas that affect solid organs, which can include the liver, spleen, and other organs.

  1. Lymphoproliferative Disorders: This term encompasses a range of conditions, including lymphomas, where there is an abnormal proliferation of lymphocytes.

  2. B-cell Lymphoma: Many non-follicular lymphomas are of B-cell origin, and this term is often used in clinical settings to describe these types of lymphomas.

  3. T-cell Lymphoma: Similar to B-cell lymphoma, this term refers to lymphomas that arise from T-cells, which can also be classified under C83.89 if they are non-follicular and extranodal.

  4. Non-Hodgkin Lymphoma (NHL): This is a general term that includes all types of lymphomas that are not classified as Hodgkin lymphoma, including those under C83.89.

  5. Extranodal Marginal Zone Lymphoma: While this is a specific subtype, it is relevant as it can sometimes be included in discussions about non-follicular lymphomas affecting extranodal sites.

  6. Diffuse Large B-cell Lymphoma (DLBCL): This is one of the most common types of non-Hodgkin lymphoma and can be classified under C83.89 if it presents in an extranodal site.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.89 is crucial for accurate diagnosis, treatment planning, and coding in medical records. These terms help healthcare professionals communicate effectively about the various forms of non-follicular lymphoma that can occur in extranodal and solid organ sites. If you need further information on specific types of lymphomas or their treatment options, feel free to ask!

Clinical Information

When discussing ICD-10 code C83.89, which refers to "Other non-follicular lymphoma, extranodal and solid organ sites," it is essential to understand the clinical presentation, signs, symptoms, and patient characteristics associated with this condition. Non-follicular lymphomas are a diverse group of lymphoproliferative disorders that can manifest in various ways, particularly when they involve extranodal sites.

Clinical Presentation

Overview of Non-Follicular Lymphoma

Non-follicular lymphomas, including those classified under C83.89, are characterized by their origin outside the lymph nodes, often affecting solid organs such as the liver, spleen, and gastrointestinal tract. These lymphomas can arise from different types of lymphoid tissue and may present with a variety of clinical features depending on the site of involvement.

Common Signs and Symptoms

Patients with C83.89 may exhibit a range of symptoms, which can vary significantly based on the specific extranodal site affected. Common signs and symptoms include:

  • Lymphadenopathy: Swelling of lymph nodes, although less prominent in extranodal presentations.
  • Organ-specific symptoms: Depending on the organ involved, symptoms may include:
  • Abdominal pain or discomfort: If the gastrointestinal tract is affected.
  • Hepatomegaly or splenomegaly: Enlargement of the liver or spleen, often leading to discomfort or fullness.
  • Respiratory symptoms: Such as cough or difficulty breathing if the lungs are involved.
  • Neurological symptoms: Including headaches, seizures, or changes in mental status if the central nervous system is affected.
  • B symptoms: These systemic symptoms may include:
  • Fever: Often low-grade and intermittent.
  • Night sweats: Profuse sweating during the night.
  • Weight loss: Unintentional weight loss over a short period.

Patient Characteristics

The demographic and clinical characteristics of patients diagnosed with C83.89 can vary, but several trends are observed:

  • Age: Non-follicular lymphomas can occur in adults of any age, but they are more common in older adults, typically those over 60 years.
  • Gender: There may be a slight male predominance in certain types of non-follicular lymphomas.
  • Comorbidities: Patients may have underlying conditions such as autoimmune diseases or previous infections (e.g., HIV) that can predispose them to lymphoproliferative disorders.
  • Histological Variants: The specific subtype of non-follicular lymphoma (e.g., diffuse large B-cell lymphoma, peripheral T-cell lymphoma) can influence the clinical presentation and prognosis.

Diagnostic Considerations

Diagnosis of C83.89 typically involves a combination of clinical evaluation, imaging studies, and histopathological examination. Key diagnostic tools include:

  • Imaging: CT scans, PET scans, or MRI to assess the extent of disease and identify extranodal involvement.
  • Biopsy: Tissue samples from affected organs or lymph nodes are crucial for histological diagnosis and classification.
  • Laboratory Tests: Blood tests may reveal abnormalities such as elevated lactate dehydrogenase (LDH) levels, which can indicate tumor burden.

Conclusion

ICD-10 code C83.89 encompasses a range of clinical presentations associated with other non-follicular lymphomas affecting extranodal and solid organ sites. The symptoms can be diverse and are often influenced by the specific organs involved. Early recognition and accurate diagnosis are critical for effective management and treatment planning. Understanding the patient characteristics and potential signs and symptoms can aid healthcare providers in identifying and addressing this complex group of lymphoproliferative disorders.

Diagnostic Criteria

The diagnosis of ICD-10 code C83.89, which pertains to "Other non-follicular lymphoma, extranodal and solid organ sites," involves a comprehensive evaluation that includes clinical, laboratory, and imaging assessments. Below is a detailed overview of the criteria typically used for diagnosing this specific type of lymphoma.

Clinical Presentation

Symptoms

Patients may present with a variety of symptoms, which can include:
- Lymphadenopathy: Swelling of lymph nodes, which may be localized or generalized.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in lymphomas.
- Extranodal Involvement: Symptoms may also arise from the involvement of extranodal sites, such as the gastrointestinal tract, skin, or other organs, leading to specific symptoms related to those areas.

Medical History

A thorough medical history is essential, including:
- Previous history of lymphoproliferative disorders.
- Family history of lymphomas or other malignancies.
- Exposure to risk factors such as certain infections (e.g., Epstein-Barr virus) or environmental toxins.

Laboratory Tests

Blood Tests

  • Complete Blood Count (CBC): To check for anemia, leukopenia, or thrombocytopenia, which may indicate bone marrow involvement.
  • Lactate Dehydrogenase (LDH): Elevated levels can indicate a higher tumor burden and are often associated with aggressive disease.

Biopsy

  • Tissue Biopsy: A definitive diagnosis typically requires a biopsy of the affected tissue. This can be done through:
  • Excisional biopsy: Removal of an entire lymph node or mass.
  • Core needle biopsy: A less invasive option that removes a small cylinder of tissue.
  • Histopathological Examination: The biopsy sample is examined microscopically to identify the type of lymphoma. Immunohistochemistry is often used to differentiate between various types of lymphomas.

Imaging Studies

Radiological Imaging

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

Staging

The staging of non-follicular lymphoma is crucial for determining the prognosis and treatment approach. The Ann Arbor staging system is commonly used, which considers:
- The number of affected lymph node regions.
- The presence of extranodal disease.
- The presence of B symptoms.

Differential Diagnosis

It is important to differentiate non-follicular lymphoma from other types of lymphomas and malignancies. This may involve:
- Flow Cytometry: To analyze the surface markers on lymphocytes, helping to classify the lymphoma.
- Cytogenetic Studies: To identify specific chromosomal abnormalities associated with certain types of lymphoma.

Conclusion

The diagnosis of ICD-10 code C83.89 involves a multifaceted approach that includes clinical evaluation, laboratory tests, imaging studies, and histopathological analysis. Accurate diagnosis is essential for effective treatment planning and management of the disease. If you have further questions or need more specific information, feel free to ask!

Related Information

Treatment Guidelines

  • Chemotherapy is cornerstone treatment
  • R-CHOP improves outcomes in B-cell lymphomas
  • Ibrutinib used for relapsed or refractory disease
  • Radiation therapy effective for localized disease
  • Stem cell transplantation reserved for high-risk patients
  • Clinical trials offer access to new therapies
  • Treatment approach depends on subtype and stage

Description

  • Non-follicular lymphomas occur outside lymph nodes
  • Lymphoma affects other organs or tissues
  • Includes gastrointestinal tract, skin, CNS, solid organs
  • Symptoms vary depending on site of involvement
  • Localized swelling and systemic symptoms common
  • Diagnosis involves imaging studies and biopsy
  • Treatment includes chemotherapy, radiation therapy

Approximate Synonyms

  • Extranodal Non-Hodgkin Lymphoma
  • Non-Follicular Lymphoma
  • Aggressive Non-Hodgkin Lymphoma
  • Primary Extranodal Lymphoma
  • Solid Organ Lymphoma
  • Lymphoproliferative Disorders
  • B-cell Lymphoma
  • T-cell Lymphoma
  • Non-Hodgkin Lymphoma (NHL)
  • Extranodal Marginal Zone Lymphoma
  • Diffuse Large B-cell Lymphoma (DLBCL)

Clinical Information

  • Non-follicular lymphoma affects extranodal sites
  • Swelling of lymph nodes less common
  • Organ-specific symptoms vary by site
  • Abdominal pain or discomfort common in GI tract
  • Hepatomegaly and splenomegaly common in liver and spleen
  • Respiratory symptoms occur in lung involvement
  • Neurological symptoms occur in CNS involvement
  • B symptoms include fever, night sweats, weight loss
  • Common in adults over 60 years old
  • Male predominance in some subtypes

Diagnostic Criteria

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