ICD-10: C83.18

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 C83.18 specifically refers to mantle cell lymphoma affecting lymph nodes at multiple sites. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Mantle Cell Lymphoma

Definition and Characteristics

Mantle cell lymphoma is a type of B-cell lymphoma that arises from the mantle zone of lymphoid follicles. It is classified as an aggressive form of non-Hodgkin lymphoma and is often associated with a specific chromosomal translocation, t(11;14), which leads to the overexpression of the cyclin D1 protein. This overexpression contributes to the uncontrolled proliferation of B-cells, resulting in the formation of tumors in lymphoid tissues.

Symptoms

Patients with mantle cell lymphoma may present with a variety of symptoms, which can include:
- Lymphadenopathy: Swelling of lymph nodes, often in multiple regions such as the neck, armpits, and groin.
- B-symptoms: These include fever, night sweats, and unexplained weight loss.
- Fatigue: Generalized tiredness and weakness.
- Abdominal discomfort: Due to lymphadenopathy in the abdominal cavity or spleen.

Diagnosis

Diagnosis of mantle cell lymphoma typically involves:
- Histopathological examination: A biopsy of affected lymph nodes is essential for confirming the diagnosis through microscopic evaluation.
- Immunophenotyping: Flow cytometry or immunohistochemistry is used to identify specific markers (e.g., CD5, CD23, and cyclin D1) that are characteristic of MCL.
- Genetic testing: Detection of the t(11;14) translocation can further confirm the diagnosis.

Staging

The staging of mantle cell lymphoma is crucial for determining the appropriate treatment approach. The Ann Arbor staging system is commonly used, which assesses the extent of lymph node involvement and the presence of extranodal disease.

Treatment Options

Treatment for mantle cell lymphoma may include:
- Chemotherapy: Often a combination of drugs, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or more intensive regimens.
- Targeted therapy: Agents like 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 cases of relapsed disease.

Prognosis

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

Conclusion

ICD-10 code C83.18 denotes mantle cell lymphoma affecting lymph nodes at multiple sites, highlighting the need for comprehensive clinical evaluation and management. Understanding the characteristics, symptoms, and treatment options for this aggressive lymphoma subtype is essential for healthcare providers to deliver effective care and improve patient outcomes. For further information, healthcare professionals may refer to the latest clinical guidelines and research studies on mantle cell lymphoma 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 C83.18 specifically refers to mantle cell lymphoma affecting lymph nodes at multiple sites. 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 significantly among individuals. Common manifestations include:

• Lymphadenopathy: The most prominent feature is the enlargement of lymph nodes, which may be painless and can occur in multiple regions, including cervical, axillary, and inguinal areas[1].
• B Symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement and can be present in many lymphoma cases[2].
• Fatigue: Patients often report significant fatigue, which can be attributed to the disease itself or the body's response to the malignancy[3].
• Abdominal Symptoms: Some patients may experience abdominal discomfort or fullness due to splenomegaly or lymphadenopathy in the abdominal cavity[4].
• Cytopenias: Blood tests may reveal low levels of red blood cells (anemia), white blood cells (leukopenia), or platelets (thrombocytopenia), which can lead to increased susceptibility to infections or bleeding[5].

Patient Characteristics

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

• Age: MCL typically occurs in older adults, with a median age at diagnosis around 60 years. It is rare in individuals under 40[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, such as cardiovascular disease or diabetes, which can complicate treatment and management[8].
• Genetic Factors: The presence of specific genetic abnormalities, such as the t(11;14) translocation, is often associated with MCL and can influence prognosis and treatment decisions[9].

Diagnosis and Evaluation

The diagnosis of mantle cell lymphoma involves a combination of clinical evaluation, imaging studies, and histopathological examination:

• Imaging: CT scans or PET scans are commonly used to assess the extent of lymphadenopathy and to identify any extranodal involvement[10].
• Biopsy: A lymph node biopsy is essential for definitive diagnosis, allowing for histological examination and immunophenotyping to confirm the presence of mantle cell lymphoma[11].
• Laboratory Tests: Blood tests may be performed to evaluate blood counts and assess organ function, which can be affected by the disease[12].

Conclusion

Mantle cell lymphoma, classified under ICD-10 code C83.18, presents with a range of clinical features primarily characterized by lymphadenopathy and systemic symptoms. Understanding the typical signs, symptoms, and patient demographics is essential for timely diagnosis and effective management. Given the complexity of this lymphoma subtype, a multidisciplinary approach involving oncologists, pathologists, and radiologists is often necessary to optimize patient outcomes. Early recognition and treatment can significantly impact the prognosis and quality of life for affected individuals.

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 C83.18 specifically refers to mantle cell lymphoma affecting lymph nodes of multiple sites. Here are some 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. Mantle Zone Lymphoma: This term highlights the specific area of the lymphatic system where the lymphoma originates.
3. B-cell Lymphoma: Since mantle cell lymphoma is a type of B-cell lymphoma, this broader term is often used in clinical discussions.
4. 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.

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. C83.1: This is the more general ICD-10 code for mantle cell lymphoma, which may not specify the involvement of multiple lymph node sites.
3. ICD-10-CM: The International Classification of Diseases, 10th Revision, Clinical Modification, which includes codes for various diseases, including C83.18 for mantle cell lymphoma.
4. Follicular Lymphoma: While distinct, this term is often mentioned in discussions about B-cell lymphomas, as it shares some characteristics with mantle cell lymphoma.
5. Aggressive B-cell Lymphoma: MCL is considered an aggressive form of B-cell lymphoma, which may be referenced in treatment discussions.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.18 can enhance communication among healthcare professionals and improve patient education. Mantle cell lymphoma, while a specific diagnosis, is part of a broader category of lymphomas, and recognizing its synonyms and related terms can aid in better comprehension and management of the disease.

Mantle cell lymphoma (MCL), classified under ICD-10 code C83.18, is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. Diagnosing MCL involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria and methods used for diagnosis:

Clinical Presentation

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

2. Physical Examination: A thorough physical examination is essential to identify enlarged lymph nodes, spleen, or liver, which may indicate lymphatic involvement.

Laboratory Tests

1. Blood Tests: Routine blood tests may reveal:
   - Anemia
   - Thrombocytopenia (low platelet count)
   - Elevated lactate dehydrogenase (LDH) levels, which can indicate tumor burden.

2. Bone Marrow Biopsy: A bone marrow biopsy is often performed to assess the extent of disease involvement. In MCL, the bone marrow may show infiltration by neoplastic lymphoid cells.

Imaging Studies

1. CT Scans: Computed tomography (CT) scans of the chest, abdomen, and pelvis are used to evaluate lymphadenopathy and organ involvement.

2. PET Scans: Positron emission tomography (PET) scans can help assess metabolic activity of lymph nodes and detect any extranodal disease.

Histopathological Examination

1. Lymph Node Biopsy: A definitive diagnosis of MCL typically requires a biopsy of an affected lymph node. The histological examination will reveal:
   - A diffuse proliferation of small to medium-sized lymphoid cells.
   - A characteristic "mantle zone" pattern.

2. Immunophenotyping: Flow cytometry or immunohistochemistry is used to identify specific cell surface markers. MCL cells typically express:
   - CD5
   - CD19
   - CD23
   - Cyclin D1 (which is a hallmark of MCL due to the t(11;14) chromosomal translocation).

3. Cytogenetic Analysis: Detection of the t(11;14)(q13;q32) translocation, which leads to overexpression of cyclin D1, is a critical diagnostic criterion for MCL.

Conclusion

The diagnosis of mantle cell lymphoma (ICD-10 code C83.18) is multifaceted, relying on clinical symptoms, laboratory findings, imaging studies, and definitive histopathological analysis. The combination of these diagnostic criteria ensures accurate identification and appropriate management of this aggressive lymphoma subtype. If you have further questions or need more specific information, feel free to ask!

Mantle cell lymphoma (MCL), classified under ICD-10 code C83.18, 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, necessitating a comprehensive treatment approach. Below, we explore the standard treatment modalities for MCL, including chemotherapy, targeted therapies, and stem cell transplantation.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone in the treatment of mantle cell lymphoma. 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 initial treatment, especially in patients with less aggressive disease.
• 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. Targeted Therapies

Recent advancements in targeted therapies have significantly improved outcomes for patients with MCL:

• Ibrutinib (Imbruvica): This Bruton's tyrosine kinase (BTK) inhibitor has shown efficacy in treating MCL, particularly in relapsed or refractory cases. Ibrutinib is often used as a single agent or in combination with other therapies.
• Acalabrutinib (Calquence): Another BTK inhibitor, Acalabrutinib, is also approved for MCL and may have a more favorable side effect profile compared to Ibrutinib.

3. Immunotherapy

• 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 target and destroy lymphoma cells.

4. Stem Cell Transplantation

• Autologous Stem Cell Transplant (ASCT): For eligible patients, particularly those who achieve a complete response to initial therapy, ASCT can be considered. This approach involves harvesting the patient’s stem cells, administering high-dose chemotherapy, and then reinfusing the stem cells to restore bone marrow function.

5. Radiation Therapy

While not a primary treatment modality for MCL, radiation therapy may be used in specific scenarios, such as localized disease or to manage symptoms related to bulky lymphadenopathy.

Conclusion

The treatment of mantle cell lymphoma (ICD-10 code C83.18) is multifaceted, involving a combination of chemotherapy, targeted therapies, immunotherapy, and potentially stem cell transplantation. The choice of treatment is influenced by various factors, including the patient's age, overall health, and specific disease characteristics. Ongoing research and clinical trials continue to refine these approaches, aiming to improve outcomes and reduce treatment-related toxicity for patients with this challenging lymphoma subtype.