ICD-10: C83.1

Mantle cell lymphoma (MCL), classified under ICD-10 code C83.1, is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. 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. Below, we explore the standard treatment strategies currently employed for MCL.

Overview of Mantle Cell Lymphoma

MCL is known for its aggressive nature and typically presents in advanced stages. It is often associated with a poor prognosis, making effective treatment crucial. The management of MCL generally involves a combination of chemotherapy, targeted therapies, and sometimes stem cell transplantation.

Standard Treatment Approaches

1. Chemotherapy Regimens

Historically, chemotherapy has been the cornerstone of treatment for MCL. Commonly used regimens include:

• CHOP: This regimen combines Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. It has been a standard treatment but is often less effective in MCL compared to other lymphomas.
• R-CHOP: The addition of Rituximab (an anti-CD20 monoclonal antibody) to the CHOP regimen has improved outcomes significantly. R-CHOP is now a standard first-line treatment for many patients with MCL.
• Hyper-CVAD: This intensive regimen alternates between hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone, followed by high-dose methotrexate and cytarabine. It is often used in younger patients or those with more aggressive disease.

2. Targeted Therapies

Recent advancements have introduced targeted therapies that have transformed the treatment landscape for MCL:

• Ibrutinib: This Bruton's tyrosine kinase (BTK) inhibitor has shown significant efficacy in MCL, particularly in patients who are refractory to traditional chemotherapy. It is often used as a single agent or in combination with other therapies.
• Acalabrutinib: Another BTK inhibitor, Acalabrutinib, is also used in the treatment of MCL, offering a favorable side effect profile compared to Ibrutinib.
• CAR T-cell Therapy: Chimeric antigen receptor (CAR) T-cell therapy has emerged as a promising option for relapsed or refractory MCL, providing a potential curative approach for some patients.

3. Stem Cell Transplantation

For eligible patients, particularly those with younger age and good performance status, autologous stem cell transplantation (ASCT) may be considered after achieving remission with initial therapy. This approach can improve long-term outcomes but is typically reserved for patients with a favorable response to initial treatment.

4. Maintenance Therapy

Post-remission maintenance therapy with agents like Rituximab may be employed to prolong remission and delay disease progression. This strategy has been shown to improve overall survival in some patient populations.

Conclusion

The treatment of mantle cell lymphoma (ICD-10 code C83.1) has evolved to include a combination of traditional chemotherapy, targeted therapies, and innovative approaches like CAR T-cell therapy. The choice of treatment is influenced by various factors, including the patient's age, overall health, and specific disease characteristics. Ongoing research continues to refine these strategies, aiming to improve outcomes and quality of life for patients with MCL. As treatment options expand, personalized approaches are becoming increasingly important in managing this challenging lymphoma subtype.

Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-lymphocytes in the mantle zone of lymphoid follicles. It is classified under the ICD-10 code C83.1, which specifically denotes mantle cell lymphoma. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Mantle Cell Lymphoma

Definition and Pathophysiology

Mantle cell lymphoma is a type of B-cell non-Hodgkin lymphoma that arises from the mantle zone of lymphoid follicles. It is associated with a specific genetic alteration, most commonly the translocation t(11;14)(q13;q32), which leads to the overexpression of the cyclin D1 protein. This overexpression promotes cell cycle progression, contributing to the uncontrolled proliferation of B-cells[1][2].

Epidemiology

MCL accounts for approximately 6% to 7% of all non-Hodgkin lymphomas. It predominantly affects older adults, with a median age of diagnosis around 60 years. The condition is more common in men than in women, with a male-to-female ratio of about 3:1[3][4].

Clinical Presentation

Patients with mantle cell lymphoma may present with a variety of symptoms, including:

• Lymphadenopathy: Swelling of lymph nodes, often in the neck, armpits, or groin.
• Splenomegaly: Enlargement of the spleen, which may cause discomfort or a feeling of fullness.
• B-symptoms: These include fever, night sweats, and unexplained weight loss, which can indicate more aggressive disease.
• Gastrointestinal Symptoms: Some patients may experience abdominal pain or changes in bowel habits due to gastrointestinal involvement[5][6].

Diagnosis

Diagnosis of mantle cell lymphoma typically involves:

• Histopathological Examination: A biopsy of affected lymph nodes or tissues is essential for diagnosis. The histological features include a diffuse proliferation of small to medium-sized lymphoid cells with irregular nuclei.
• Immunophenotyping: Flow cytometry is used to identify specific surface markers. MCL cells typically express CD5, CD19, CD20, and cyclin D1, while lacking CD23[7].
• Cytogenetic Analysis: Detection of the t(11;14) translocation is a hallmark of MCL and can be confirmed through fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) techniques[8].

Staging and Prognosis

The staging of mantle cell lymphoma is crucial for determining treatment options and prognosis. The Ann Arbor staging system is commonly used, which classifies the disease based on the extent of lymph node involvement and the presence of systemic symptoms. The prognosis for MCL can vary significantly, with factors such as age, performance status, and the presence of specific genetic markers influencing outcomes. The International Prognostic Index (IPI) is often utilized to assess prognosis[9][10].

Treatment Options

Treatment for mantle cell lymphoma may include:

• Chemotherapy: Combination chemotherapy regimens, such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), are commonly used.
• Targeted Therapy: Agents like ibrutinib, a Bruton's tyrosine kinase inhibitor, have shown efficacy in treating MCL, particularly in relapsed or refractory cases.
• Stem Cell Transplantation: Autologous stem cell transplantation may be considered for eligible patients, especially those with aggressive disease or those who achieve remission after initial therapy[11][12].

ICD-10 Code and Classification

The ICD-10 code for mantle cell lymphoma is C83.1, which falls under the broader category of non-Hodgkin lymphomas. This classification is essential for accurate coding in medical records, billing, and epidemiological studies[13][14].

Conclusion

Mantle cell lymphoma is a complex and aggressive form of non-Hodgkin lymphoma that requires careful diagnosis and management. Understanding its clinical features, diagnostic criteria, and treatment options is crucial for healthcare providers involved in the care of affected patients. Ongoing research continues to explore new therapeutic strategies to improve outcomes for individuals diagnosed with this challenging condition.

Mantle cell lymphoma (MCL), classified under ICD-10 code C83.1, is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-cells in the mantle zone of lymphoid follicles. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for diagnosis and management.

Clinical Presentation

Signs and Symptoms

The clinical presentation of mantle cell lymphoma can vary significantly among patients, but common signs and symptoms include:

• Lymphadenopathy: Patients often present with painless swelling of lymph nodes, particularly in the neck, armpits, or groin. This is one of the most common initial findings[13].
• Splenomegaly: Enlargement of the spleen may occur, leading to discomfort or a feeling of fullness in the abdomen[15].
• B Symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement and can affect prognosis[13][15].
• Fatigue: Many patients report significant fatigue, which can be debilitating and is often related to the disease burden or anemia[15].
• Abdominal Symptoms: Some patients may experience abdominal pain or discomfort due to lymphadenopathy or splenomegaly[15].

Late Effects

Patients who have undergone treatment for mantle cell lymphoma may experience late effects, which can include secondary malignancies, cardiovascular issues, and other long-term health complications. Monitoring for these effects is essential for ongoing patient care[2].

Patient Characteristics

Demographics

• Age: MCL predominantly affects older adults, with a median age of diagnosis around 60 years. It is rare in individuals under 40[13][15].
• Gender: There is a male predominance, with men being diagnosed approximately two to three times more frequently than women[15].

Risk Factors

While the exact cause of mantle cell lymphoma is not fully understood, several risk factors have been identified:
- Genetic Factors: Certain genetic mutations and chromosomal translocations, particularly the t(11;14) translocation involving the BCL-1 gene, are commonly associated with MCL[15].
- Immune System Status: Patients with compromised immune systems, such as those with HIV/AIDS or those who have undergone organ transplantation, may have an increased risk of developing lymphomas, including MCL[15].

Comorbidities

Patients with mantle cell lymphoma often present with comorbid conditions, which can complicate treatment and management. Common comorbidities include cardiovascular diseases, diabetes, and other malignancies, particularly in older adults[5][6].

Conclusion

Mantle cell lymphoma (ICD-10 code C83.1) presents with a range of clinical signs and symptoms, primarily involving lymphadenopathy, splenomegaly, and systemic B symptoms. The patient demographic is typically older adults, with a notable male predominance. Understanding these characteristics is vital for healthcare providers in diagnosing and managing this complex disease effectively. Regular follow-up and monitoring for late effects post-treatment are essential components of comprehensive care for patients with MCL.

Mantle cell lymphoma (MCL), classified under ICD-10 code C83.1, is a type of non-Hodgkin lymphoma that arises from B-cells in the mantle zone of lymphoid follicles. Understanding the alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below are some of the key alternative names and related terms associated with mantle cell lymphoma.

Alternative Names for Mantle Cell Lymphoma

1. MCL: This is the commonly used abbreviation for mantle cell lymphoma.
2. Mantle Zone Lymphoma: This term emphasizes the origin of the lymphoma in the mantle zone of lymphoid follicles.
3. B-cell Mantle Cell Lymphoma: This name specifies the B-cell lineage of the lymphoma, distinguishing it from other types of lymphomas.
4. Non-Follicular Lymphoma: While this term is broader, mantle cell lymphoma is categorized under non-follicular lymphomas, which do not arise from follicle center cells[1][11].

Related Terms

1. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes various types such as Hodgkin and non-Hodgkin lymphomas.
2. Non-Hodgkin Lymphoma (NHL): Mantle cell lymphoma is a subtype of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers.
3. B-cell Lymphoma: This term refers to lymphomas that originate from B-cells, the type of white blood cell involved in mantle cell lymphoma.
4. C83: This is the broader ICD-10 code category for non-follicular lymphomas, under which mantle cell lymphoma falls[1][4][11].

Clinical Context

Mantle cell lymphoma is characterized by specific genetic alterations, such as the translocation t(11;14), which leads to the overexpression of the cyclin D1 protein. This genetic marker is often used in the diagnosis of MCL. The disease typically presents in older adults and can manifest with lymphadenopathy, splenomegaly, and gastrointestinal involvement.

Conclusion

Understanding the alternative names and related terms for mantle cell lymphoma is crucial for healthcare professionals involved in diagnosis, treatment, and coding. These terms not only facilitate better communication among medical teams but also enhance the accuracy of medical records and billing processes. If you need further information on the clinical aspects or treatment options for mantle cell lymphoma, feel free to ask!

Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma characterized by specific clinical, histological, and genetic features. The diagnosis of MCL, which corresponds to the ICD-10 code C83.1, involves a combination of clinical evaluation, imaging studies, laboratory tests, and histopathological examination. Below are the key criteria used for diagnosing mantle cell lymphoma:

Clinical Presentation

1. Symptoms: Patients may present with symptoms such as lymphadenopathy (swollen lymph nodes), splenomegaly (enlarged spleen), fatigue, weight loss, and night sweats. These symptoms are common in many lymphomas and necessitate further investigation[3].

2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy and organomegaly, which can indicate the presence of lymphoma[3].

Laboratory Tests

1. Blood Tests: Complete blood counts (CBC) may reveal anemia, thrombocytopenia, or leukocytosis. Additionally, serum lactate dehydrogenase (LDH) levels can be elevated in lymphoma cases, serving as a prognostic indicator[3].

2. Immunophenotyping: Flow cytometry is used to analyze the surface markers on lymphocytes. MCL typically expresses CD5, CD19, CD20, and CD23, along with the absence of FMC-7, which helps differentiate it from other types of B-cell lymphomas[3][4].

Imaging Studies

1. CT Scans: Computed tomography (CT) scans of the chest, abdomen, and pelvis are performed to assess the extent of lymphadenopathy and organ involvement. This imaging is crucial for staging the disease[3].

2. PET Scans: Positron emission tomography (PET) scans may be utilized to evaluate metabolic activity in lymph nodes and other tissues, helping to determine the aggressiveness of the lymphoma and guide treatment decisions[3].

Histopathological Examination

1. Biopsy: A definitive diagnosis of MCL requires a biopsy of an affected lymph node or other involved tissue. Histological examination typically reveals a diffuse proliferation of small to medium-sized lymphoid cells with irregular nuclei[4].

2. Genetic Testing: The presence of the t(11;14)(q13;q32) chromosomal translocation, which results in the overexpression of cyclin D1, is a hallmark of MCL. This genetic alteration can be confirmed through fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) techniques[4][5].

Diagnostic Criteria Summary

To summarize, the diagnosis of mantle cell lymphoma (ICD-10 code C83.1) is based on:

• Clinical symptoms and physical examination findings.
• Laboratory tests, including blood counts and immunophenotyping.
• Imaging studies to assess disease extent.
• Histopathological examination of biopsy samples.
• Genetic testing for specific chromosomal abnormalities.

These criteria collectively help clinicians confirm the diagnosis of mantle cell lymphoma and differentiate it from other types of lymphomas, ensuring appropriate management and treatment strategies are implemented[3][4][5].

In conclusion, the diagnosis of MCL is a multifaceted process that integrates clinical, laboratory, imaging, and histopathological data to ensure accurate identification and effective treatment planning.