ICD-10: C83.14

Mantle cell lymphoma (MCL), classified under ICD-10 code C83.14, is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. This aggressive form of lymphoma often presents with advanced disease at diagnosis, frequently involving lymph nodes, including those in the axilla and upper limb. The treatment approaches for MCL have evolved significantly over the years, focusing on both immediate management and long-term outcomes.

Standard Treatment Approaches

1. Chemotherapy Regimens

The cornerstone of treatment for MCL typically involves chemotherapy, often in combination with immunotherapy. Commonly used regimens include:

• R-CHOP: This regimen combines Rituximab (an anti-CD20 monoclonal antibody) with Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. R-CHOP is frequently used for initial treatment and is effective in many patients with MCL[1].

• R-HyperCVAD: This is a more intensive regimen that alternates between cycles of Rituximab, Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone (HyperCVAD) and high-dose Methotrexate and Cytarabine. This approach is often reserved for younger patients or those with more aggressive disease[2].

2. Targeted Therapies

Recent advancements have introduced targeted therapies that have changed the landscape of MCL treatment:

• Ibrutinib (Imbruvica): This Bruton's tyrosine kinase (BTK) inhibitor has shown significant efficacy in MCL, particularly in patients who are not candidates for intensive chemotherapy or those with relapsed disease. Ibrutinib is often used as a single agent or in combination with other therapies[3].

• Acalabrutinib (Calquence): Another BTK inhibitor, Acalabrutinib, has also been approved for MCL and may offer a different side effect profile compared to Ibrutinib[4].

3. Stem Cell Transplantation

For eligible patients, especially those with relapsed or refractory MCL, autologous stem cell transplantation (ASCT) may be considered. This approach is typically used after achieving a complete or partial response to initial therapy and can provide a chance for long-term remission[5].

4. Radiation Therapy

While not a primary treatment modality for MCL, radiation therapy may be utilized in specific scenarios, such as localized disease or for palliative care to relieve symptoms associated with lymphadenopathy. It can also be used in conjunction with other treatments to target specific areas of disease involvement[6].

5. Clinical Trials

Given the evolving nature of MCL treatment, participation in clinical trials may be an option for patients. These trials often explore new combinations of therapies, novel agents, or different treatment strategies that may offer improved outcomes compared to standard approaches[7].

Conclusion

The management of mantle cell lymphoma, particularly in cases involving lymph nodes of the axilla and upper limb, requires a multidisciplinary approach tailored to the individual patient's disease characteristics and overall health. Standard treatment typically involves a combination of chemotherapy and targeted therapies, with options for stem cell transplantation and radiation therapy as needed. As research continues to advance, new therapies and combinations are likely to emerge, offering hope for improved outcomes in patients with this challenging lymphoma subtype.

For patients diagnosed with MCL, it is crucial to discuss treatment options with a healthcare provider specializing in hematologic malignancies to determine the most appropriate and effective treatment plan.

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 C83.14 specifically refers to mantle cell lymphoma that affects the lymph nodes of the axilla (armpit area) and upper limb. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Mantle Cell Lymphoma

Pathophysiology

Mantle cell lymphoma arises from the transformation of naive B-cells, typically involving a chromosomal translocation between chromosomes 11 and 14, which leads to the overexpression of the cyclin D1 protein. This abnormality drives the uncontrolled proliferation of B-cells, contributing to the development of lymphoma.

Symptoms

Patients with mantle cell lymphoma may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the axillary region and upper limbs.
- B-symptoms: These include fever, night sweats, and unexplained weight loss.
- Fatigue: Generalized tiredness and weakness.
- Abdominal discomfort: If lymph nodes in the abdomen are involved, patients may experience pain or fullness.

Diagnosis

Diagnosis of mantle cell lymphoma typically involves:
- Histopathological Examination: A biopsy of affected lymph nodes is essential for diagnosis, where the presence of characteristic mantle zone patterns is observed.
- Immunophenotyping: Flow cytometry is used to identify specific surface markers (e.g., CD5, CD19, CD23, and cyclin D1) that are indicative of MCL.
- Genetic Testing: Detection of the t(11;14) translocation can confirm the diagnosis.

Staging

The staging of mantle cell lymphoma follows the Ann Arbor classification, which assesses the extent of disease based on the number of lymph node regions involved and the presence of systemic symptoms. The involvement of axillary and upper limb lymph nodes would typically indicate at least stage II disease.

Treatment Options

Treatment for mantle cell lymphoma may include:
- Chemotherapy: Common regimens include R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) or more intensive regimens like R-HyperCVAD.
- Targeted Therapy: Agents such as ibrutinib, a Bruton's tyrosine kinase inhibitor, have shown efficacy in treating MCL.
- Stem Cell Transplantation: In eligible patients, autologous or allogeneic stem cell transplantation may be considered, especially in relapsed cases.

Prognosis

The prognosis for mantle cell lymphoma can vary significantly based on factors such as the stage at diagnosis, patient age, and response to initial treatment. Historically, MCL has been associated with a poorer prognosis compared to other types of non-Hodgkin lymphoma, but advancements in treatment have improved outcomes.

Conclusion

ICD-10 code C83.14 designates mantle cell lymphoma affecting the lymph nodes of the axilla and upper limb, highlighting the need for accurate diagnosis and tailored treatment strategies. Understanding the clinical features, diagnostic criteria, and treatment options is crucial for managing this complex lymphoma subtype effectively. For further information or specific case management, consultation with a hematologist or oncologist is recommended.

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 C83.14 specifically refers to mantle cell lymphoma located in the lymph nodes of the axilla and upper limb. 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 signs and symptoms, which can vary based on the extent of the disease and the specific lymph nodes involved:

• Lymphadenopathy: The most common presentation is painless swelling of lymph nodes, particularly in the axillary region. Patients may notice enlarged lymph nodes in the upper limb as well[1].
• B Symptoms: These include systemic symptoms such as fever, night sweats, and unexplained weight loss. These symptoms are indicative of more aggressive disease and can significantly impact the patient's quality of life[2].
• Fatigue: Many patients report a general sense of fatigue or malaise, which can be attributed to the underlying malignancy and its metabolic demands[3].
• Splenomegaly and Hepatomegaly: In some cases, patients may also exhibit enlargement of the spleen (splenomegaly) or liver (hepatomegaly), although these are less common in early presentations[4].
• Cytopenias: Patients may experience low blood cell counts (anemia, leukopenia, or thrombocytopenia) due to bone marrow involvement, leading to symptoms such as easy bruising, bleeding, or increased susceptibility to infections[5].

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 rare in younger populations[6].
• Gender: There is a male predominance, with men being diagnosed approximately two to three times more frequently than women[7].
• Comorbidities: Patients may have other health conditions that can complicate treatment, such as cardiovascular disease or diabetes, which are more prevalent in older populations[8].
• Genetic Factors: Some patients may have specific genetic markers or chromosomal abnormalities, such as the t(11;14) translocation, which is commonly associated with MCL and can influence prognosis and treatment decisions[9].

Diagnosis and Management

Diagnosis of mantle cell lymphoma typically involves a combination of imaging studies, biopsy of affected lymph nodes, and histopathological examination to confirm the presence of characteristic mantle zone B-cells. Immunophenotyping and genetic studies may also be performed to establish the diagnosis and guide treatment options.

Management strategies for MCL may include:

• Chemotherapy: Combination chemotherapy regimens are commonly used, often in conjunction with immunotherapy agents such as rituximab[10].
• Targeted Therapy: Newer treatments, including Bruton's tyrosine kinase inhibitors (e.g., ibrutinib), have shown promise in managing MCL, particularly in relapsed or refractory cases[11].
• Stem Cell Transplantation: In select patients, especially those with aggressive disease, autologous stem cell transplantation may be considered as part of the treatment plan[12].

Conclusion

Mantle cell lymphoma, particularly when affecting the lymph nodes of the axilla and upper limb, presents with distinct clinical features and patient characteristics. Early recognition of symptoms such as lymphadenopathy and systemic B symptoms is essential for timely diagnosis and effective management. As research continues to evolve, treatment options are expanding, offering hope for improved outcomes in affected patients.

For further information or specific case management strategies, consulting with a hematologist or oncologist specializing in lymphomas is recommended.

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 C83.14 specifically refers to mantle cell lymphoma located in the lymph nodes of the axilla (armpit) and upper limb. Here are some alternative names and related terms associated with this condition:

Alternative Names for Mantle Cell Lymphoma

1. MCL: The abbreviation commonly used 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 the umbrella of non-Hodgkin lymphomas, which includes various types of lymphomas that do not fall under Hodgkin lymphoma.
4. C83.14: The specific ICD-10 code designation for mantle cell lymphoma affecting the lymph nodes of the axilla and upper limb.

Related Terms

1. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with mantle cell lymphoma.
2. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes various types, including MCL.
3. Follicular lymphoma: While distinct, this is another type of B-cell lymphoma that may be mentioned in discussions about MCL due to their shared characteristics.
4. Chronic lymphocytic leukemia (CLL): Although different, CLL can sometimes be confused with MCL due to overlapping features in B-cell malignancies.
5. Stage IV lymphoma: Referring to advanced stages of lymphoma, which may include mantle cell lymphoma if it has spread beyond the lymph nodes.

Clinical Context

Mantle cell lymphoma is often diagnosed through a combination of clinical evaluation, imaging studies, and biopsy. The presence of MCL in the axillary and upper limb lymph nodes can indicate a more advanced disease state, necessitating specific treatment approaches, including chemotherapy, targeted therapy, or stem cell transplantation.

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

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 diagnosis of MCL, particularly when it involves lymph nodes of the axilla and upper limb (ICD-10 code C83.14), follows specific clinical, histological, and immunophenotypic criteria. Below is a detailed overview of the criteria used for diagnosis.

Clinical Presentation

1. Symptoms: Patients may present with:
   - Painless lymphadenopathy, particularly in the axillary and upper limb regions.
   - Systemic symptoms such as fever, night sweats, and weight loss (B symptoms).
   - Fatigue and general malaise.

2. Physical Examination: A thorough examination may reveal:
   - Enlarged lymph nodes in the axilla and upper limb.
   - Splenomegaly or hepatomegaly in some cases.

Histological Criteria

1. Biopsy: A lymph node biopsy is essential for diagnosis. The histological examination typically shows:
   - A diffuse or nodular proliferation of small to medium-sized lymphoid cells.
   - A characteristic "mantle zone" pattern, where neoplastic cells surround residual follicles.

2. Cell Morphology: The neoplastic cells often exhibit:
   - Irregular nuclear contours.
   - Scant cytoplasm.
   - High nuclear-to-cytoplasmic ratio.

Immunophenotypic Analysis

1. Immunohistochemistry: The following markers are typically assessed:
   - CD5: Positive in most cases of MCL.
   - CD19: Positive.
   - CD23: Usually negative, which helps differentiate MCL from chronic lymphocytic leukemia (CLL).
   - Cyclin D1: Positive, indicating the presence of the t(11;14) translocation, which is a hallmark of MCL.

2. Genetic Testing: Detection of the t(11;14)(q13;q32) translocation is crucial, as it leads to overexpression of cyclin D1, a key feature of MCL.

Staging and Imaging

1. Staging: The Ann Arbor staging system is commonly used to determine the extent of the disease, which includes:
   - Stage I: Involvement of a single lymph node region.
   - Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm.
   - Stage III: Involvement of lymph node regions on both sides of the diaphragm.
   - Stage IV: Disseminated involvement of one or more extralymphatic organs.

2. Imaging Studies: Imaging techniques such as CT scans or PET scans may be employed to assess the extent of lymph node involvement and to rule out other conditions.

Conclusion

The diagnosis of mantle cell lymphoma, particularly in the axilla and upper limb, involves a combination of clinical evaluation, histological examination, immunophenotypic profiling, and genetic testing. Accurate diagnosis is crucial for determining the appropriate treatment strategy and prognosis for patients. If you have further questions or need more specific information, feel free to ask!