ICD-10: C83.12

Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. The ICD-10 code C83.12 specifically refers to mantle cell lymphoma that is primarily located in the intrathoracic lymph nodes. Below is a detailed overview of this condition, including its clinical description, symptoms, diagnosis, and treatment options.

Clinical Description

Definition

Mantle cell lymphoma is a rare and aggressive form of B-cell lymphoma that arises from the outer edge (mantle zone) of lymphoid follicles. It is classified as a type of non-Hodgkin lymphoma and is known for its unique genetic features, including the translocation t(11;14), which leads to the overexpression of the cyclin D1 protein. This overexpression contributes to the uncontrolled proliferation of lymphocytes.

Intrathoracic Involvement

The designation of C83.12 indicates that the lymphoma is specifically affecting the intrathoracic lymph nodes, which are located within the thoracic cavity, including the mediastinal and hilar lymph nodes. This localization can lead to various clinical manifestations due to the proximity to vital structures such as the lungs, heart, and major blood vessels.

Symptoms

Patients with mantle cell lymphoma involving intrathoracic lymph nodes may present with a range of symptoms, including:

• Respiratory Symptoms: Cough, dyspnea (shortness of breath), or chest pain due to lymph node enlargement compressing the airways or lungs.
• Systemic Symptoms: Fever, night sweats, and unexplained weight loss, which are common in many lymphomas.
• Lymphadenopathy: Swelling of lymph nodes, particularly in the neck, axilla, or groin, may also be observed.

Diagnosis

Diagnostic Procedures

The diagnosis of mantle cell lymphoma typically involves several steps:

1. Clinical Evaluation: A thorough history and physical examination to assess symptoms and lymphadenopathy.
2. Imaging Studies: CT scans or PET scans are often used to visualize lymph node involvement and assess the extent of the disease.
3. Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph nodes, followed by histopathological examination. Immunohistochemistry is used to identify the characteristic markers of mantle cell lymphoma, such as CD5, CD19, CD20, and cyclin D1.
4. Genetic Testing: Molecular studies may be performed to confirm the presence of the t(11;14) translocation.

Treatment Options

The treatment of mantle cell lymphoma, particularly when it involves intrathoracic lymph nodes, may include:

• Chemotherapy: Combination chemotherapy regimens are commonly used, often including agents such as cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or more intensive regimens.
• Targeted Therapy: The use of targeted agents like ibrutinib, a Bruton's tyrosine kinase inhibitor, has shown promise in treating mantle cell lymphoma.
• Immunotherapy: Monoclonal antibodies, such as rituximab, may be used in conjunction with chemotherapy.
• Stem Cell Transplantation: In eligible patients, autologous or allogeneic stem cell transplantation may be considered, especially in cases of relapsed or refractory disease.

Conclusion

Mantle cell lymphoma, particularly when affecting the intrathoracic lymph nodes, presents unique challenges in diagnosis and management. Understanding the clinical features, diagnostic criteria, and treatment options is crucial for healthcare providers to optimize patient outcomes. Continuous research and advancements in targeted therapies are improving the prognosis for patients diagnosed with this aggressive lymphoma subtype.

Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. The ICD-10 code C83.12 specifically refers to mantle cell lymphoma involving intrathoracic 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

Patients with mantle cell lymphoma may present with a variety of symptoms, which can vary based on the extent of the disease and the specific lymph nodes involved. Common signs and symptoms include:

• Lymphadenopathy: Swelling of lymph nodes, particularly in the neck, axilla, or groin. In the case of C83.12, there may be notable enlargement of intrathoracic lymph nodes, which can lead to respiratory symptoms.
• B Symptoms: These include fever, night sweats, and unexplained weight loss. These systemic symptoms are indicative of lymphoma and can significantly impact the patient's quality of life.
• Respiratory Symptoms: Due to the involvement of intrathoracic lymph nodes, patients may experience cough, dyspnea (shortness of breath), or chest pain, which can be attributed to compression of surrounding structures or pleural involvement.
• Fatigue: A common symptom in many cancer patients, fatigue can be profound and debilitating in those with MCL.

Patient Characteristics

Certain demographic and clinical characteristics are often observed in patients diagnosed with mantle cell lymphoma:

• Age: MCL typically affects older adults, with a median age at diagnosis around 60 years. It is relatively rare in younger populations.
• Gender: There is a male predominance, with men being diagnosed approximately three times more often than women.
• Comorbidities: Patients may have other health conditions, such as cardiovascular disease or diabetes, which can complicate treatment and management.
• Histological Features: MCL is characterized by the presence of cyclin D1 overexpression due to the t(11;14) chromosomal translocation, which is a key diagnostic marker.

Diagnosis and Evaluation

The diagnosis of mantle cell lymphoma, particularly with intrathoracic involvement, typically involves:

• Imaging Studies: CT scans of the chest, abdomen, and pelvis are essential for assessing lymph node involvement and staging the disease. PET scans may also be utilized to evaluate metabolic activity in lymph nodes.
• Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph nodes, which is examined histologically to confirm the presence of mantle cell lymphoma.
• Laboratory Tests: Blood tests may reveal anemia, elevated lactate dehydrogenase (LDH), and other abnormalities that can indicate lymphoma.

Conclusion

Mantle cell lymphoma, particularly when involving intrathoracic lymph nodes (ICD-10 code C83.12), presents with a range of symptoms including lymphadenopathy, respiratory issues, and systemic B symptoms. Understanding the clinical presentation and patient characteristics is essential for timely diagnosis and effective management. Given the complexity of this condition, a multidisciplinary approach involving oncologists, radiologists, and pathologists is often necessary to optimize patient outcomes.

Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. The ICD-10 code C83.12 specifically refers to mantle cell lymphoma that involves intrathoracic lymph nodes. Below are alternative names and related terms associated with this condition.

Alternative Names for Mantle Cell Lymphoma

1. MCL: This is the commonly used abbreviation for mantle cell lymphoma.
2. B-cell lymphoma: Since mantle cell lymphoma is a type of B-cell neoplasm, it may be referred to in this broader category.
3. Non-Hodgkin lymphoma (NHL): MCL is classified under non-Hodgkin lymphomas, which encompass a variety of lymphoid malignancies.
4. Cyclin D1-positive lymphoma: This term is used because mantle cell lymphoma is often characterized by the overexpression of cyclin D1 due to the t(11;14) chromosomal translocation.

Related Terms

1. Intrathoracic lymph nodes: This term refers to the lymph nodes located within the thoracic cavity, which can be involved in mantle cell lymphoma.
2. Lymphadenopathy: This is a general term for the enlargement of lymph nodes, which can occur in various types of lymphoma, including MCL.
3. Stage IV lymphoma: If mantle cell lymphoma has spread to intrathoracic lymph nodes, it may be classified as stage IV, indicating advanced disease.
4. Lymphoma staging: This refers to the process of determining the extent of lymphoma, which is crucial for treatment planning.

Clinical Context

Mantle cell lymphoma is known for its aggressive nature and can present with various symptoms, including lymphadenopathy, splenomegaly, and gastrointestinal involvement. The involvement of intrathoracic lymph nodes can complicate the clinical picture, necessitating specific diagnostic and therapeutic approaches.

Understanding these alternative names and related terms can aid healthcare professionals in accurately diagnosing and coding for mantle cell lymphoma, particularly when it involves intrathoracic lymph nodes. This specificity is essential for effective treatment planning and patient management.

Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. The ICD-10 code C83.12 specifically refers to mantle cell lymphoma with involvement of intrathoracic lymph nodes. The diagnosis of MCL, particularly when it involves intrathoracic lymph nodes, is based on a combination of clinical, histopathological, and immunophenotypic criteria.

Diagnostic Criteria for Mantle Cell Lymphoma

1. Clinical Presentation

Patients with mantle cell lymphoma may present with:
- Lymphadenopathy: Swollen lymph nodes, particularly in the neck, axilla, or groin, which may also include intrathoracic lymph nodes.
- B-symptoms: These include fever, night sweats, and unexplained weight loss, which can indicate systemic involvement.
- Splenomegaly: Enlargement of the spleen may also be observed.

2. Histopathological Examination

A definitive diagnosis of MCL requires a biopsy of the affected lymph node or tissue. The histopathological features include:
- Lymphoid Proliferation: A diffuse or nodular pattern of small to medium-sized lymphoid cells.
- Cytological Features: The presence of irregular nuclei and scant cytoplasm in the neoplastic cells.

3. Immunophenotyping

Immunophenotyping is crucial for distinguishing MCL from other types of lymphomas. Key markers include:
- CD5 Positive: MCL cells typically express CD5.
- CD23 Negative: Unlike chronic lymphocytic leukemia (CLL), MCL is usually CD23 negative.
- Cyclin D1 Positive: The overexpression of cyclin D1 is a hallmark of MCL, resulting from the t(11;14) chromosomal translocation that juxtaposes the cyclin D1 gene with the immunoglobulin heavy chain locus.

4. Genetic Testing

• Cytogenetic Analysis: The presence of the t(11;14)(q13;q32) translocation is a critical genetic marker for MCL. This translocation leads to the overexpression of cyclin D1, which is a key driver of the disease.

5. Imaging Studies

Imaging techniques such as CT scans or PET scans are utilized to assess the extent of the disease, particularly to evaluate the involvement of intrathoracic lymph nodes. These imaging studies help in staging the lymphoma and determining the appropriate treatment approach.

6. Staging

The Ann Arbor staging system is commonly used to classify the extent of lymphoma. For MCL, the involvement of intrathoracic lymph nodes would typically indicate at least stage II disease, where lymph nodes on both sides of the diaphragm are affected.

Conclusion

The diagnosis of mantle cell lymphoma, particularly with intrathoracic lymph node involvement (ICD-10 code C83.12), relies on a comprehensive evaluation that includes clinical assessment, histopathological examination, immunophenotyping, genetic testing, and imaging studies. Each of these components plays a vital role in confirming the diagnosis and guiding treatment decisions. If you have further questions or need more specific information, feel free to ask!

Mantle cell lymphoma (MCL), classified under ICD-10 code C83.12, is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. This type of lymphoma often presents with advanced disease at diagnosis, frequently involving lymph nodes, spleen, and bone marrow, and can also affect intrathoracic lymph nodes. The treatment approaches for MCL have evolved significantly over the years, focusing on both the management of the disease and the improvement of patient outcomes.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone of treatment for MCL. The most commonly used regimens include:

• R-CHOP: This regimen combines Rituximab (a monoclonal antibody) with Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. R-CHOP is often used for patients with newly diagnosed MCL and is effective in achieving remission[1].

• Hyper-CVAD: This intensive regimen alternates cycles of Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone with high-dose Methotrexate and Cytarabine. Hyper-CVAD is typically reserved for younger patients or those with more aggressive disease due to its intensity and potential side effects[2].

2. Targeted Therapy

Targeted therapies have transformed the treatment landscape for MCL:

• Ibrutinib (Imbruvica): This Bruton's tyrosine kinase (BTK) inhibitor has shown significant efficacy in MCL, particularly in relapsed or refractory cases. It is often used as a single agent or in combination with other therapies[3].

• Acalabrutinib (Calquence): Another BTK inhibitor, Acalabrutinib, is also approved for MCL and may have a more favorable side effect profile compared to Ibrutinib[4].

3. Immunotherapy

Immunotherapy options are increasingly being integrated into treatment regimens:

• Rituximab: As a monoclonal antibody targeting CD20, Rituximab is frequently used in combination with chemotherapy and can also be administered as maintenance therapy after initial treatment to prolong remission[5].

• CAR T-cell Therapy: Chimeric Antigen Receptor (CAR) T-cell therapy has emerged as a promising option for patients with relapsed or refractory MCL. This personalized treatment involves modifying a patient’s T-cells to better recognize and attack lymphoma cells[6].

4. Stem Cell Transplantation

For eligible patients, particularly those with high-risk features or relapsed disease, autologous stem cell transplantation (ASCT) may be considered. This approach typically follows initial chemotherapy and aims to consolidate remission[7].

5. Radiation Therapy

While not a primary treatment modality for MCL, radiation therapy may be utilized in specific scenarios, such as localized disease or to manage symptoms related to bulky lymphadenopathy, particularly in intrathoracic lymph nodes[8].

Conclusion

The management of mantle cell lymphoma, particularly with intrathoracic lymph node involvement, requires a multidisciplinary approach tailored to the individual patient’s disease characteristics and overall health. Advances in chemotherapy, targeted therapies, and immunotherapy have significantly improved outcomes for patients with MCL. Ongoing clinical trials continue to explore new treatment combinations and strategies, aiming to enhance efficacy and reduce toxicity. Regular follow-up and monitoring are essential to manage potential relapses and treatment-related complications effectively.

For patients diagnosed with MCL, it is crucial to discuss treatment options with a healthcare provider specializing in hematology or oncology to determine the most appropriate and personalized treatment plan.