ICD-10: C84.46

Peripheral T-cell lymphoma, not elsewhere classified, intrapelvic lymph nodes

Additional Information

Approximate Synonyms

ICD-10 code C84.46 refers specifically to "Peripheral T-cell lymphoma, not elsewhere classified, intrapelvic lymph nodes." This classification falls under the broader category of peripheral T-cell lymphomas, which are a diverse group of hematologic malignancies. Here are some alternative names and related terms associated with this specific code:

Alternative Names

  1. Peripheral T-cell Lymphoma (PTCL): This is a general term for a group of aggressive lymphomas that originate from T-cells, which are a type of white blood cell involved in the immune response.
  2. Non-Hodgkin Lymphoma (NHL): While this term encompasses a wide range of lymphomas, peripheral T-cell lymphomas are a subtype of NHL.
  3. T-cell Lymphoma: A broader term that includes various types of lymphomas arising from T-cells, including PTCL.
  1. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which can be a symptom of peripheral T-cell lymphoma.
  2. Lymphoma: A general term for cancers that affect the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.
  3. Intrapelvic Lymph Nodes: This term specifies the location of the lymph nodes affected by the lymphoma, indicating that the disease is localized to the pelvic region.
  4. Aggressive Lymphoma: Peripheral T-cell lymphomas are often classified as aggressive due to their rapid progression and poor prognosis compared to other types of lymphomas.

Clinical Context

Peripheral T-cell lymphomas, including those classified under C84.46, are characterized by their origin from mature T-cells and can present with various clinical features, including lymphadenopathy, systemic symptoms, and involvement of extranodal sites. The specific mention of "intrapelvic lymph nodes" indicates a localized manifestation of the disease, which can influence treatment decisions and prognosis.

Understanding these alternative names and related terms can aid healthcare professionals in accurately diagnosing and coding for this condition, ensuring appropriate treatment and management strategies are employed.

Description

Peripheral T-cell lymphoma (PTCL) is a diverse group of hematologic malignancies that arise from mature T-cells. The ICD-10 code C84.46 specifically refers to "Peripheral T-cell lymphoma, not elsewhere classified, intrapelvic lymph nodes." This classification is crucial for accurate diagnosis, treatment planning, and billing purposes.

Clinical Description of C84.46

Definition and Classification

C84.46 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, with C84.46 indicating a specific localization of the disease in the intrapelvic lymph nodes, which are situated in the pelvic region, including the iliac and inguinal lymph nodes.

Symptoms

Patients with PTCL may present with a range of symptoms, which can include:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the pelvic area.
- B-symptoms: These may include fever, night sweats, and unexplained weight loss.
- Abdominal discomfort: Due to lymph node enlargement or involvement of surrounding structures.
- Fatigue: Generalized tiredness is common in lymphoma patients.

Diagnosis

Diagnosis of PTCL, including C84.46, typically involves:
- Histopathological examination: A biopsy of the affected lymph nodes is essential for confirming the diagnosis.
- Immunophenotyping: This process helps to identify the specific type of T-cell lymphoma by analyzing the surface markers on the cells.
- Imaging studies: CT scans or PET scans may be utilized to assess the extent of lymph node involvement and to check for any additional sites of disease.

Treatment Options

Treatment for PTCL, including cases classified under C84.46, may involve:
- Chemotherapy: Combination chemotherapy regimens are commonly used, often including agents such as CHOP (Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) or more intensive regimens depending on the subtype and stage.
- Radiation therapy: This may be used in localized disease or as part of a combined modality approach.
- Stem cell transplantation: In eligible patients, autologous or allogeneic stem cell transplantation may be considered, especially in relapsed or refractory cases.

Prognosis

The prognosis for patients with PTCL can vary significantly based on several factors, including the specific subtype, stage at diagnosis, and response to initial treatment. Generally, PTCL tends to have a poorer prognosis compared to other types of non-Hodgkin lymphoma, with overall survival rates influenced by the aforementioned factors.

Conclusion

ICD-10 code C84.46 is essential for the classification of peripheral T-cell lymphoma localized to the intrapelvic lymph nodes. Understanding the clinical presentation, diagnostic criteria, treatment options, and prognosis associated with this condition is vital for healthcare providers in managing patients effectively. Accurate coding not only aids in treatment planning but also ensures appropriate reimbursement and resource allocation in clinical settings.

Clinical Information

Peripheral T-cell lymphoma (PTCL) is a diverse group of hematological malignancies characterized by the proliferation of mature T-cells. The specific subtype coded as ICD-10 code C84.46 refers to PTCL that is not classified elsewhere and involves the intrapelvic lymph nodes. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for diagnosis and management.

Clinical Presentation

Signs and Symptoms

  1. Lymphadenopathy:
    - Patients often present with enlarged lymph nodes, particularly in the pelvic region. This can lead to noticeable swelling or masses in the lower abdomen or groin area[11][12].

  2. B Symptoms:
    - Common systemic symptoms include:

    • Fever: Unexplained fevers that may be intermittent.
    • Night Sweats: Profuse sweating during the night.
    • Weight Loss: Significant and unexplained weight loss over a short period[11][12].
  3. Abdominal Symptoms:
    - Due to the involvement of intrapelvic lymph nodes, patients may experience abdominal discomfort, pain, or a feeling of fullness. This can also lead to gastrointestinal symptoms such as nausea or changes in bowel habits[12][13].

  4. Fatigue:
    - A common complaint among patients, often related to the disease process and systemic effects of the lymphoma[11][12].

  5. Skin Manifestations:
    - Some patients may exhibit skin lesions or rashes, particularly in certain subtypes of PTCL, although this is less common in intrapelvic presentations[12][13].

Patient Characteristics

  1. Demographics:
    - PTCL can occur in adults of any age, but it is more commonly diagnosed in middle-aged and older adults, typically between the ages of 50 and 70[11][12].

  2. Gender:
    - There is a slight male predominance in the incidence of PTCL, including the subtype associated with intrapelvic lymph nodes[12][13].

  3. Comorbidities:
    - Patients may have a history of autoimmune diseases or prior infections, which can influence the development of lymphomas. Additionally, some may have a history of exposure to certain chemicals or prior chemotherapy, which can increase the risk of developing PTCL[11][12].

  4. Histological Features:
    - The diagnosis of PTCL is confirmed through histological examination, which may reveal atypical lymphoid cells and a varied immunophenotype. The presence of specific markers can help differentiate PTCL from other lymphomas[12][13].

Conclusion

Peripheral T-cell lymphoma, not elsewhere classified, involving intrapelvic lymph nodes (ICD-10 code C84.46) presents with a range of clinical features, including lymphadenopathy, systemic B symptoms, and abdominal discomfort. Understanding these signs and symptoms, along with patient demographics and characteristics, is essential for timely diagnosis and effective management. Early recognition and treatment can significantly impact patient outcomes, making awareness of this condition critical for healthcare providers.

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 C84.46 specifically refers to PTCL that is not classified elsewhere and is localized in the intrapelvic lymph nodes. The diagnosis of PTCL, including the specific subtype represented by C84.46, involves several criteria, which can be categorized into clinical, histopathological, and immunophenotypic assessments.

Clinical Criteria

  1. Symptoms: Patients may present with nonspecific symptoms such as fever, night sweats, weight loss, and fatigue, often referred to as "B symptoms." Lymphadenopathy, particularly in the pelvic region, may also be noted.

  2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy or splenomegaly, which can indicate lymphatic involvement.

  3. Imaging Studies: Imaging techniques such as CT scans or PET scans are utilized to assess the extent of lymphadenopathy and to confirm the involvement of intrapelvic lymph nodes.

Histopathological Criteria

  1. Biopsy: A definitive diagnosis of PTCL requires a biopsy of the affected lymph nodes. Histological examination is crucial to identify the characteristic features of PTCL.

  2. Morphological Features: Pathologists look for atypical lymphoid cells, which may vary in size and shape. The presence of a mixed infiltrate of small and large cells is often noted.

  3. Necrosis: Areas of necrosis within the lymph node may also be observed, which can be indicative of aggressive disease.

Immunophenotypic Criteria

  1. Immunohistochemistry: The use of immunohistochemical staining is critical for characterizing the T-cell lineage. Markers such as CD3, CD4, CD5, and CD8 are typically expressed in T-cell lymphomas.

  2. T-cell Receptor Gene Rearrangement: Molecular studies may be performed to detect T-cell receptor gene rearrangements, which support the diagnosis of a clonal T-cell population.

  3. Exclusion of Other Lymphomas: It is essential to rule out other types of lymphomas, particularly B-cell lymphomas and other subtypes of T-cell lymphomas, through comprehensive immunophenotyping and genetic studies.

Conclusion

The diagnosis of Peripheral T-cell lymphoma, not elsewhere classified, intrapelvic lymph nodes (ICD-10 code C84.46) is a multifaceted process that requires a combination of clinical evaluation, histopathological examination, and immunophenotypic characterization. Accurate diagnosis is crucial for determining the appropriate treatment strategy and prognosis for patients with this aggressive form of lymphoma. 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 C84.46, refers to a group of aggressive hematological malignancies that primarily affect T-cells. This specific code denotes PTCL that is localized in the intrapelvic lymph nodes and is not classified elsewhere. The treatment approaches for this condition typically involve a combination of chemotherapy, targeted therapy, and sometimes stem cell transplantation. Below is a detailed overview of the standard treatment strategies for PTCL, particularly focusing on C84.46.

Standard Treatment Approaches

1. Chemotherapy Regimens

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

  • CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. While CHOP is a standard treatment for many lymphomas, its efficacy in PTCL can be variable, and it may not be sufficient for all patients.

  • CHOP-like Regimens: Variations of the CHOP regimen, such as EPOCH (Etoposide, Prednisone, Oncovin, Cyclophosphamide, and Doxorubicin), may be employed, especially in cases where a more aggressive approach is warranted.

  • Targeted Chemotherapy: Agents like brentuximab vedotin (Adcetris®) may be used, particularly in cases where the lymphoma expresses CD30, which is common in some PTCL subtypes. This drug is an antibody-drug conjugate that targets CD30-positive cells, delivering cytotoxic agents directly to the cancer cells[1][6].

2. Targeted Therapy

In addition to traditional chemotherapy, targeted therapies are increasingly being integrated into treatment plans:

  • Belinostat (Beleodaq): This histone deacetylase inhibitor is approved for the treatment of relapsed or refractory PTCL. It works by altering the expression of genes involved in cell cycle regulation and apoptosis, leading to cancer cell death[3][4].

  • Newer Agents: Other investigational agents and combinations are being studied in clinical trials, which may offer additional options for patients with PTCL.

3. Stem Cell Transplantation

For eligible patients, particularly those with aggressive disease or those who have not responded adequately to initial therapy, stem cell transplantation may be considered:

  • Autologous Stem Cell Transplant (ASCT): This is often performed after achieving a complete or partial remission with chemotherapy. It involves harvesting the patient’s own stem cells, administering high-dose chemotherapy, and then reinfusing the stem cells to restore bone marrow function.

  • Allogeneic Stem Cell Transplant: This may be an option for patients with high-risk disease or those who relapse after ASCT. It involves using stem cells from a donor and carries a higher risk of complications but can offer a potential cure for some patients.

4. Radiation Therapy

While not a primary treatment modality for systemic PTCL, radiation therapy may be used in certain situations:

  • Localized Disease: If the lymphoma is confined to a specific area, such as the intrapelvic lymph nodes, radiation therapy may be employed either as a primary treatment or as an adjunct to chemotherapy to help control localized disease.

5. Clinical Trials

Given the aggressive nature of PTCL and the variability in response to standard treatments, participation in clinical trials is often encouraged. These trials may offer access to novel therapies and combinations that are not yet widely available.

Conclusion

The treatment of Peripheral T-cell lymphoma, particularly the subtype coded as C84.46, involves a multifaceted approach that includes chemotherapy, targeted therapies, and potentially stem cell transplantation. The choice of treatment is influenced by various factors, including the patient's overall health, the specific characteristics of the lymphoma, and the presence of any comorbid conditions. As research continues to evolve, new therapies and combinations are likely to emerge, providing hope for improved outcomes in patients with this challenging disease. For the most current treatment options, consultation with a hematologist or oncologist specializing in lymphomas is essential.

Related Information

Approximate Synonyms

  • Peripheral T-cell Lymphoma (PTCL)
  • Non-Hodgkin Lymphoma (NHL)
  • T-cell Lymphoma
  • Lymphadenopathy
  • Lymphoma
  • Intrapelvic Lymph Nodes
  • Aggressive Lymphoma

Description

Clinical Information

Diagnostic Criteria

  • Nonspecific symptoms such as fever
  • Lymphadenopathy in pelvic region
  • Thorough physical examination
  • Imaging studies with CT scans/PET scans
  • Biopsy of affected lymph nodes
  • Atypical lymphoid cells on histology
  • Presence of necrosis in lymph node
  • CD3, CD4, CD5, and CD8 markers expression
  • T-cell receptor gene rearrangement detection

Treatment Guidelines

  • Chemotherapy cornerstone of PTCL treatment
  • CHOP regimen primary treatment approach
  • Targeted therapy for relapsed or refractory cases
  • Brentuximab vedotin for CD30-positive lymphomas
  • Belinostat for histone deacetylase inhibition
  • Stem cell transplantation for eligible patients
  • Autologous stem cell transplant after chemotherapy
  • Allogeneic stem cell transplant for high-risk disease
  • Radiation therapy for localized disease
  • Clinical trials for novel therapies and combinations

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