ICD-10: C84.48

Peripheral T-cell lymphoma, not elsewhere classified, lymph nodes of multiple sites

Additional Information

Description

Peripheral T-cell lymphoma (PTCL) is a diverse group of hematologic malignancies that arise from mature T-cells. The ICD-10-CM code C84.48 specifically refers to "Peripheral T-cell lymphoma, not elsewhere classified, lymph nodes of multiple sites." This classification is crucial for accurate diagnosis, treatment planning, and billing purposes.

Clinical Description of C84.48

Definition and Classification

C84.48 is part of the broader category of mature T/NK-cell lymphomas, which are characterized by the proliferation of T-lymphocytes that have undergone malignant transformation. PTCL encompasses various subtypes, and the "not elsewhere classified" designation indicates that the specific subtype does not fit into the more defined categories of T-cell lymphomas, such as anaplastic large cell lymphoma or angioimmunoblastic T-cell lymphoma[1][2].

Epidemiology

PTCL is relatively rare compared to B-cell lymphomas, accounting for approximately 10-15% of all non-Hodgkin lymphomas. It is more prevalent in adults, with a higher incidence in older populations. Risk factors include immunosuppression, certain infections (like HIV), and exposure to chemicals or radiation[1].

Clinical Presentation

Patients with PTCL may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, which can occur in multiple sites throughout the body.
- B symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many lymphomas.
- Fatigue: A general sense of tiredness that does not improve with rest.
- Pruritus: Itching of the skin, which can be a symptom associated with lymphoproliferative disorders[1][2].

Diagnosis

Diagnosis of PTCL typically involves:
- Histopathological examination: A biopsy of affected lymph nodes is essential for confirming the diagnosis and determining the specific subtype.
- Immunophenotyping: This process helps identify the specific T-cell markers present on the malignant cells, aiding in classification.
- Imaging studies: CT or PET scans may be used to assess the extent of disease and involvement of lymph nodes and other organs[1][2].

Treatment

Treatment options for PTCL can vary based on the specific subtype, stage of the disease, and patient factors. Common approaches include:
- Chemotherapy: Combination chemotherapy regimens are often employed, with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) being a standard treatment.
- Targeted therapies: Newer agents, such as brentuximab vedotin and belinostat, may be used, particularly in relapsed or refractory cases.
- Stem cell transplantation: In eligible patients, autologous or allogeneic stem cell transplantation may be considered, especially for those with aggressive disease[1][2].

Conclusion

ICD-10 code C84.48 is essential for the classification and management of peripheral T-cell lymphoma affecting multiple lymph node sites. Understanding the clinical presentation, diagnostic criteria, and treatment options is crucial for healthcare providers in delivering effective care to patients with this complex and heterogeneous group of lymphomas. Accurate coding not only facilitates appropriate treatment but also ensures proper reimbursement and tracking of healthcare outcomes related to this malignancy.

For further information on billing and coding related to PTCL, healthcare providers may refer to specific guidelines and local coverage determinations that address the nuances of treatment and reimbursement for these patients[3][4].

Clinical Information

Peripheral T-cell lymphoma (PTCL), not elsewhere classified (ICD-10 code C84.48), is a heterogeneous group of aggressive lymphomas that arise from mature T-cells. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for timely diagnosis and management.

Clinical Presentation

Signs and Symptoms

Patients with PTCL often present with a variety of symptoms that can be nonspecific, making diagnosis challenging. Common signs and symptoms include:

  • Lymphadenopathy: The most prominent feature is the enlargement of lymph nodes, which may occur in multiple sites throughout the body. Patients may notice swollen lymph nodes in the neck, armpits, or groin[1].
  • Fever: Many patients experience unexplained fevers, which can be intermittent or persistent[1].
  • Night Sweats: Profuse sweating during the night is a common symptom, often leading to discomfort and sleep disturbances[1].
  • Weight Loss: Unintentional weight loss is frequently reported, which can be significant and is often associated with the disease's systemic effects[1].
  • Fatigue: Patients may experience extreme tiredness or lack of energy, which can be debilitating[1].
  • Pruritus: Some individuals report itching, which may be generalized or localized[1].

Additional Symptoms

In advanced cases, patients may also exhibit:

  • Abdominal Pain or Distension: This can occur if lymph nodes in the abdomen are involved, leading to organ compression[1].
  • Respiratory Symptoms: If lymph nodes in the thoracic region are affected, patients may experience cough or difficulty breathing[1].
  • Skin Lesions: Some subtypes of PTCL can present with skin manifestations, including rashes or nodules[1].

Patient Characteristics

Demographics

  • Age: PTCL typically affects adults, with a median age of diagnosis around 60 years. However, it can occur in younger individuals as well[2].
  • Gender: There is a slight male predominance in the incidence of PTCL, with men being more frequently diagnosed than women[2].

Risk Factors

Several factors may increase the risk of developing PTCL, including:

  • Immunosuppression: Patients with compromised immune systems, such as those with HIV/AIDS or those undergoing immunosuppressive therapy, are at higher risk[2].
  • Autoimmune Diseases: Conditions like rheumatoid arthritis or Sjögren's syndrome have been associated with an increased risk of lymphomas, including PTCL[2].
  • Environmental Exposures: Certain chemical exposures, such as pesticides or solvents, may also contribute to the risk of developing T-cell lymphomas[2].

Comorbidities

Patients with PTCL may have other health conditions that can complicate their clinical picture, including:

  • Infections: Due to immunosuppression, patients may be more susceptible to infections, which can complicate treatment and management[2].
  • Other Malignancies: There is an increased risk of secondary malignancies in patients with PTCL, particularly in those with a history of other lymphoproliferative disorders[2].

Conclusion

Peripheral T-cell lymphoma, not elsewhere classified (C84.48), presents with a range of symptoms primarily related to lymphadenopathy, systemic symptoms like fever and weight loss, and can significantly impact a patient's quality of life. Understanding the clinical presentation and patient characteristics is essential for healthcare providers to facilitate early diagnosis and appropriate management strategies. Given the aggressive nature of PTCL, timely intervention is critical to improve patient outcomes.

Approximate Synonyms

Peripheral T-cell lymphoma (PTCL), not elsewhere classified (NEC), is a type of non-Hodgkin lymphoma that primarily affects T-cells. The ICD-10 code C84.48 specifically refers to this condition when it involves lymph nodes of multiple sites. Here are some alternative names and related terms associated with this diagnosis:

Alternative Names

  1. Peripheral T-cell Lymphoma, NOS: This term is often used interchangeably with PTCL, NEC, indicating that the lymphoma does not fit into more specific subtypes.
  2. PTCL, Unspecified: Similar to NOS, this term highlights the lack of specific classification within the broader category of peripheral T-cell lymphomas.
  3. Peripheral T-cell Lymphoma, Multiple Lymph Nodes: This name emphasizes the involvement of multiple lymph node sites in the disease process.
  1. Non-Hodgkin Lymphoma (NHL): PTCL is a subtype of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers that include various types of lymphomas.
  2. T-cell Lymphoma: This broader category includes all lymphomas that originate from T-cells, including PTCL and its various subtypes.
  3. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with PTCL.
  4. Lymphoma: A general term for cancers that affect the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.
  5. Aggressive Lymphoma: PTCL is often classified as an aggressive lymphoma due to its rapid progression and the need for prompt treatment.

Clinical Context

Peripheral T-cell lymphoma, NEC, is characterized by a heterogeneous group of diseases with varying clinical presentations and outcomes. The involvement of multiple lymph nodes can complicate diagnosis and treatment, making it essential for healthcare providers to be familiar with these alternative names and related terms to ensure accurate communication and documentation in clinical settings.

In summary, understanding the alternative names and related terms for ICD-10 code C84.48 is crucial for healthcare professionals involved in the diagnosis and treatment of patients with peripheral T-cell lymphoma. This knowledge aids in accurate coding, effective communication, and comprehensive patient care.

Diagnostic Criteria

Peripheral T-cell lymphoma (PTCL) is a diverse group of hematologic malignancies characterized by the proliferation of mature T-cells. The ICD-10 code C84.48 specifically refers to PTCL that is not classified elsewhere and affects lymph nodes in multiple sites. The diagnosis of PTCL, including the specific subtype represented by C84.48, involves several criteria, which can be categorized into clinical, histopathological, and immunophenotypic assessments.

Clinical Criteria

  1. Symptoms: Patients often present with symptoms such as:
    - Lymphadenopathy (swollen lymph nodes)
    - Fever
    - Night sweats
    - Weight loss
    - Fatigue

  2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy in multiple regions, which is a hallmark of this condition.

  3. Medical History: A detailed medical history, including any previous lymphoproliferative disorders, autoimmune diseases, or infections, is crucial for context.

Histopathological Criteria

  1. Biopsy: A lymph node biopsy is necessary to confirm the diagnosis. The biopsy should show:
    - Atypical lymphoid cells that are predominantly T-cells.
    - A diffuse or infiltrative pattern of growth.

  2. Histological Subtypes: The pathologist will assess the histological features to classify the PTCL subtype, as there are several, including:
    - PTCL, not otherwise specified (PTCL-NOS)
    - Angioimmunoblastic T-cell lymphoma
    - Anaplastic large cell lymphoma

Immunophenotypic Criteria

  1. Flow Cytometry: This technique is used to analyze the surface markers on the lymphoid cells. The following markers are typically assessed:
    - Positive for T-cell markers (e.g., CD3, CD4, CD8)
    - Negative for B-cell markers (e.g., CD19, CD20)

  2. Genetic Studies: In some cases, genetic abnormalities may be identified, such as the presence of the ALK gene rearrangement in anaplastic large cell lymphoma, which can help in distinguishing between subtypes.

Additional Diagnostic Tools

  1. Imaging Studies: Imaging techniques such as CT scans or PET scans may be employed to assess the extent of lymphadenopathy and to identify any extranodal involvement.

  2. Bone Marrow Biopsy: This may be performed to evaluate for bone marrow involvement, which can influence treatment decisions and prognosis.

Conclusion

The diagnosis of Peripheral T-cell lymphoma, not elsewhere classified, affecting multiple lymph node sites (ICD-10 code C84.48) is a multifaceted process that requires a combination of clinical evaluation, histopathological examination, and immunophenotypic analysis. Accurate diagnosis is critical for determining the appropriate treatment strategy and for prognostic assessment. If you have further questions or need more specific information, feel free to ask!

Treatment Guidelines

Peripheral T-cell lymphoma (PTCL), particularly the subtype classified under ICD-10 code C84.48, refers to a group of aggressive hematological malignancies that primarily affect T-cells and can involve lymph nodes at multiple sites. The treatment approaches for PTCL are multifaceted and typically involve a combination of chemotherapy, targeted therapy, and sometimes stem cell transplantation. Below is a detailed overview of the standard treatment strategies for this condition.

Overview of Peripheral T-Cell Lymphoma

Peripheral T-cell lymphomas are a heterogeneous group of lymphoid malignancies that arise from mature T-cells. The prognosis and treatment can vary significantly based on the specific subtype, the extent of disease, and the patient's overall health. C84.48 specifically denotes PTCL that does not fit into other defined categories, indicating a need for tailored treatment approaches.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains the cornerstone of treatment for PTCL. The most commonly used regimens include:

  • CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. While effective for many lymphomas, its efficacy in PTCL can be limited, and it is often used in combination with other agents.

  • CHOP-like Regimens: Variations of the CHOP regimen, such as EPOCH (Etoposide, Prednisone, Oncovin, Cyclophosphamide, and Doxorubicin), may be employed, particularly in cases of more aggressive disease.

  • Targeted Chemotherapy: Agents like Pralatrexate and Belinostat (Beleodaq) have shown promise in treating PTCL and may be used in relapsed or refractory cases[3][7].

2. Targeted Therapy

Targeted therapies are increasingly being integrated into treatment protocols for PTCL:

  • Brentuximab Vedotin: This antibody-drug conjugate targets CD30 and is particularly effective in patients with anaplastic large cell lymphoma (ALCL), a subtype of PTCL. It may be used in combination with chemotherapy or as a single agent in relapsed cases[3].

  • Histone Deacetylase Inhibitors: Drugs like Belinostat have been approved for use in relapsed PTCL and can be part of the treatment regimen, especially for patients who are not candidates for intensive chemotherapy[3][7].

3. Stem Cell Transplantation

For eligible patients, especially those with aggressive disease or those who achieve remission after initial therapy, autologous stem cell transplantation (ASCT) may be considered. This approach can significantly improve outcomes and is often recommended after first-line therapy in younger patients or those with good performance status[5].

4. Radiation Therapy

Radiation therapy may be utilized in specific scenarios, particularly for localized disease or as a palliative measure to relieve symptoms associated with lymphadenopathy. It is not typically the primary treatment modality for systemic PTCL but can be effective in localized cases[6].

5. Clinical Trials

Given the aggressive nature of PTCL and the variability in response to standard treatments, participation in clinical trials is encouraged. These trials may offer access to novel therapies and combinations that are not yet widely available but show promise in improving outcomes for patients with PTCL[5].

Conclusion

The treatment of Peripheral T-cell lymphoma, particularly the subtype classified under ICD-10 code C84.48, involves a comprehensive approach that includes chemotherapy, targeted therapies, and potentially stem cell transplantation. The choice of treatment is influenced by various factors, including the specific subtype of PTCL, the patient's overall health, and the extent of the disease. As research continues to evolve, new therapies and combinations are being explored, making clinical trials a valuable option for many patients. For optimal management, a multidisciplinary approach involving hematologists, oncologists, and supportive care teams is essential.

Related Information

Description

  • Peripheral T-cell lymphoma (PTCL) is a diverse group
  • Malignancies that arise from mature T-cells
  • Lymph nodes of multiple sites are affected
  • Relatively rare compared to B-cell lymphomas
  • More prevalent in adults with higher incidence in older populations
  • Symptoms include lymphadenopathy, B symptoms and fatigue
  • Diagnosis involves histopathological examination and immunophenotyping

Clinical Information

  • Lymphadenopathy occurs in multiple sites
  • Fever is a common symptom
  • Night Sweats disrupt sleep patterns
  • Unintentional weight loss is frequent
  • Extreme fatigue affects daily life
  • Pruritus can be generalized or localized
  • Abdominal pain may occur with lymph node involvement
  • Respiratory symptoms arise from thoracic region
  • Skin lesions are seen in some subtypes
  • Median age of diagnosis is 60 years
  • Male gender has a higher incidence rate
  • Immunosuppression increases risk significantly
  • Autoimmune diseases raise lymphoma risk
  • Environmental exposures contribute to risk
  • Infections complicate treatment and management
  • Secondary malignancies occur in patients

Approximate Synonyms

  • Peripheral T-cell Lymphoma NOS
  • PTCL Unspecified
  • Peripheral T-cell Lymphoma Multiple Sites
  • Non-Hodgkin Lymphoma NHL
  • T-cell Lymphoma
  • Lymphadenopathy
  • Aggressive Lymphoma

Diagnostic Criteria

  • Lymphadenopathy in multiple regions
  • Fever and night sweats as symptoms
  • Atypical lymphoid cells with T-cell markers
  • Diffuse or infiltrative growth pattern
  • Positive for CD3, CD4, CD8; negative for CD19, CD20
  • Bone marrow involvement by biopsy
  • Genetic abnormalities like ALK gene rearrangement

Treatment Guidelines

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