ICD-10: C82.57

Diffuse follicle center lymphoma, classified under ICD-10 code C82.57, is a subtype of non-Hodgkin lymphoma (NHL) that primarily affects the spleen. This condition is characterized by the proliferation of B-cells in the follicle centers of lymphoid tissue, leading to various clinical manifestations. The treatment approaches for this lymphoma subtype typically involve a combination of chemotherapy, targeted therapy, and sometimes radiation therapy, depending on the stage of the disease and the patient's overall health.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone of treatment for diffuse follicle center 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 the first-line treatment for many types of aggressive B-cell lymphomas, including diffuse follicle center lymphoma[1].
• R-CVP: For patients who may not tolerate more intensive regimens, R-CVP (Rituximab, Cyclophosphamide, Vincristine, and Prednisone) can be an alternative, particularly for those with less aggressive disease[2].

2. Targeted Therapy

Targeted therapies have emerged as effective options for treating diffuse follicle center lymphoma:

• Rituximab: This monoclonal antibody targets CD20 on B-cells and is used both as a standalone treatment and in combination with chemotherapy. It has been shown to improve outcomes significantly in patients with follicular lymphomas[3].
• Brentuximab vedotin: While primarily used for Hodgkin lymphoma, it may be considered in certain cases of B-cell lymphomas, depending on specific markers and patient characteristics[4].

3. Radiation Therapy

Radiation therapy may be utilized in specific scenarios, particularly for localized disease or as a consolidation treatment after chemotherapy. It can help reduce the risk of recurrence in patients with localized splenic involvement[5].

4. Stem Cell Transplantation

For patients with relapsed or refractory diffuse follicle center lymphoma, autologous stem cell transplantation (ASCT) may be considered. This approach is typically reserved for younger patients or those with a good performance status, as it can offer a chance for long-term remission[6].

5. Clinical Trials

Participation in clinical trials can provide access to novel therapies and treatment strategies that are not yet widely available. Patients are encouraged to discuss this option with their healthcare providers, as ongoing research may lead to improved outcomes[7].

Conclusion

The treatment of diffuse follicle center lymphoma, particularly when it involves the spleen, is multifaceted and tailored to the individual patient. Standard approaches typically include chemotherapy regimens like R-CHOP, targeted therapies such as Rituximab, and, in some cases, radiation therapy or stem cell transplantation. As research continues to evolve, new treatment modalities may emerge, offering hope for improved management of this lymphoma subtype. Patients should work closely with their healthcare team to determine the most appropriate treatment plan based on their specific circumstances and disease characteristics.

References

1. Billing and Coding: Off-label Use of Rituximab.
2. NON-HODGKIN LYMPHOMA Includes Follicular.
3. HCT for Non-Hodgkin Lymphoma.
4. CLINICAL MEDICATION POLICY.
5. Radioimmunotherapy in the Treatment of Non-Hodgkin.
6. Medical Necessity Tool for Flow Cytometry.
7. CMS Manual System.

Diffuse follicle center lymphoma, classified under ICD-10 code C82.57, is a subtype of non-Hodgkin lymphoma that primarily affects the spleen. Understanding alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below is a detailed overview of the alternative names and related terms associated with this diagnosis.

Alternative Names

1. Follicular Lymphoma, Diffuse Variant: This term emphasizes the diffuse nature of the lymphoma, distinguishing it from other follicular lymphomas that may present in a more localized manner.

2. Diffuse Large B-Cell Lymphoma (DLBCL): While not synonymous, DLBCL can sometimes be confused with diffuse follicle center lymphoma due to overlapping characteristics. It is important to note that DLBCL is a distinct entity but may share similar clinical features.

3. Spleen Involvement Follicular Lymphoma: This term highlights the specific involvement of the spleen, which is a critical aspect of the diagnosis.

4. B-Cell Lymphoma, Follicular Type: This broader term encompasses various types of follicular lymphomas, including diffuse follicle center lymphoma.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): This is the overarching category under which diffuse follicle center lymphoma falls. NHL includes a variety of lymphoid malignancies, and understanding this classification is essential for accurate diagnosis and treatment.

2. Lymphoid Tissue: Referring to the tissues involved in the immune response, including the spleen, lymph nodes, and bone marrow, this term is relevant when discussing the pathology of diffuse follicle center lymphoma.

3. Hematopoietic Neoplasms: This term encompasses all blood-related cancers, including lymphomas, leukemias, and myelomas, providing a broader context for understanding diffuse follicle center lymphoma.

4. Stage and Grade: These terms are often used in conjunction with C82.57 to describe the extent of the disease and its aggressiveness, which are critical for treatment planning.

5. Immunophenotyping: This term refers to the laboratory process used to identify specific markers on the lymphoma cells, which can help differentiate diffuse follicle center lymphoma from other types of lymphomas.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C82.57 is crucial for healthcare professionals involved in the diagnosis and treatment of diffuse follicle center lymphoma. This knowledge aids in accurate communication, documentation, and treatment planning, ensuring that patients receive the most appropriate care based on their specific condition. If you have further questions or need additional information on this topic, feel free to ask!

Diffuse follicle center lymphoma, classified under ICD-10 code C82.57, is a subtype of non-Hodgkin lymphoma (NHL) that primarily affects the spleen. The diagnosis of this condition involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below is a detailed overview of the criteria used for diagnosing diffuse follicle center lymphoma.

Clinical Presentation

Symptoms

Patients with diffuse follicle center lymphoma may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, which may be localized or generalized.
- Splenomegaly: Enlargement of the spleen, often a prominent feature in this subtype.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which can indicate more aggressive disease.

Medical History

A thorough medical history is essential, including any previous lymphoproliferative disorders, autoimmune diseases, or family history of lymphomas.

Diagnostic Imaging

Imaging Studies

Imaging techniques play a crucial role in the diagnosis and staging of diffuse follicle center lymphoma:
- Ultrasound: Can help assess splenomegaly and lymphadenopathy.
- CT Scans: Provide detailed images of the spleen and lymph nodes, helping to identify the extent of the disease.
- PET Scans: Useful for evaluating metabolic activity of the lymphoma and detecting any additional sites of disease.

Histopathological Examination

Biopsy

A definitive diagnosis of diffuse follicle center lymphoma requires a biopsy of the affected tissue. This can be performed through:
- Excisional Biopsy: Removal of an entire lymph node or a portion of the spleen.
- Core Needle Biopsy: Less invasive, but may not provide sufficient tissue for a definitive diagnosis.

Microscopic Analysis

The biopsy specimen is examined under a microscope by a pathologist. Key features include:
- Follicular Architecture: The presence of neoplastic follicles that disrupt normal lymphoid architecture.
- Cell Type: Predominantly composed of large B-cells, which are characteristic of follicle center lymphoma.
- Immunophenotyping: Immunohistochemical staining is performed to identify specific markers (e.g., CD10, BCL-6, and BCL-2) that confirm the diagnosis.

Molecular and Genetic Testing

Genetic Markers

Molecular testing may be conducted to identify genetic abnormalities associated with diffuse follicle center lymphoma, such as:
- BCL2 Gene Rearrangements: Common in follicular lymphomas and can be indicative of a more aggressive disease.
- Next-Generation Sequencing: May be used to identify mutations that could influence treatment decisions.

Staging

Ann Arbor Staging System

Once diagnosed, the lymphoma is staged using the Ann Arbor system, which considers the number of lymph node regions involved, the presence of splenic involvement, and any systemic symptoms.

Conclusion

The diagnosis of diffuse follicle center lymphoma (ICD-10 code C82.57) is a multifaceted process that combines clinical evaluation, imaging studies, histopathological examination, and molecular testing. Accurate diagnosis is crucial for determining the appropriate treatment strategy and prognosis. If you suspect lymphoma or have related symptoms, it is essential to consult a healthcare professional for a comprehensive evaluation and diagnosis.

Clinical Description of ICD-10 Code C82.57: Diffuse Follicle Center Lymphoma, Spleen

ICD-10 Code C82.57 refers specifically to Diffuse Follicle Center Lymphoma (DFCL) localized in the spleen. This subtype of non-Hodgkin lymphoma (NHL) is characterized by the proliferation of B-lymphocytes within the follicle centers of lymphoid tissue, leading to a diffuse pattern of growth. Below is a detailed overview of this condition, including its clinical features, diagnosis, and treatment options.

Overview of Diffuse Follicle Center Lymphoma

• Definition: Diffuse Follicle Center Lymphoma is a type of B-cell lymphoma that arises from the germinal centers of lymphoid follicles. It is classified under the broader category of follicular lymphomas but is distinguished by its diffuse growth pattern, which can lead to more aggressive behavior compared to other follicular lymphomas.

• Epidemiology: This lymphoma is more common in adults, particularly those over the age of 60. It accounts for a significant proportion of non-Hodgkin lymphomas, with varying incidence rates based on geographic and demographic factors.

Clinical Features

• Symptoms: Patients with DFCL may present with:
• Painless lymphadenopathy (swelling of lymph nodes)
• Splenomegaly (enlargement of the spleen), which is particularly relevant for C82.57
• Symptoms related to bone marrow involvement, such as fatigue, anemia, or thrombocytopenia

• B symptoms, including fever, night sweats, and weight loss, which may indicate more advanced disease.

• Physical Examination: Upon examination, clinicians may note:

• Enlarged spleen (splenomegaly) that can be palpated during a physical exam
• Possible lymphadenopathy in other regions, such as the neck, axilla, or groin.

Diagnosis

• Histopathological Examination: Diagnosis typically involves:
• Biopsy of affected lymphoid tissue, which reveals a diffuse infiltrate of neoplastic B-cells.

• Immunophenotyping to confirm the B-cell lineage and assess the expression of specific markers (e.g., CD10, BCL-6).

• Imaging Studies: Imaging techniques such as CT scans or PET scans may be utilized to assess the extent of disease, particularly to evaluate splenic involvement and any other lymphatic structures.

• Staging: The Ann Arbor staging system is commonly used to determine the extent of lymphoma, which is crucial for treatment planning.

Treatment Options

• Chemotherapy: The primary treatment for DFCL often involves chemotherapy regimens, which may include:
• CHOP (Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone)

• R-CHOP (adding Rituximab, a monoclonal antibody targeting CD20 on B-cells)

• Radiation Therapy: In some cases, localized radiation therapy may be considered, especially if the disease is confined to the spleen or specific lymph node regions.

• Targeted Therapy: Newer therapies, including targeted agents like Bruton’s tyrosine kinase inhibitors or CAR T-cell therapy, may be options for relapsed or refractory cases.

• Follow-Up Care: Regular follow-up is essential to monitor for disease recurrence and manage any long-term effects of treatment.

Prognosis

The prognosis for patients with Diffuse Follicle Center Lymphoma can vary widely based on several factors, including the stage at diagnosis, the presence of B symptoms, and the patient's overall health. Generally, the disease can be indolent but may transform into a more aggressive form, necessitating close monitoring and timely intervention.

Conclusion

ICD-10 code C82.57 encapsulates a specific and clinically significant subtype of non-Hodgkin lymphoma characterized by its diffuse growth pattern in the spleen. Understanding the clinical presentation, diagnostic criteria, and treatment options is crucial for effective management and improved patient outcomes. Regular follow-up and monitoring are essential components of care for individuals diagnosed with this condition.

Diffuse follicle center lymphoma (DFCL), classified under ICD-10 code C82.57, is a subtype of non-Hodgkin lymphoma characterized by the proliferation of B-lymphocytes in the follicular centers of lymphoid tissue. This condition primarily affects the spleen, among other sites, and presents with a range of clinical features, signs, and symptoms. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with this lymphoma subtype.

Clinical Presentation

General Overview

Diffuse follicle center lymphoma is a type of indolent (slow-growing) lymphoma that can transform into a more aggressive form. It is often diagnosed in adults, with a higher prevalence in individuals over the age of 60. The clinical presentation can vary significantly among patients, depending on the extent of the disease and the specific sites involved.

Common Symptoms

Patients with DFCL may present with a variety of symptoms, which can include:

• Lymphadenopathy: Swelling of lymph nodes, particularly in the cervical, axillary, or inguinal regions. This is often one of the first signs noticed by patients or during a physical examination.
• Splenomegaly: Enlargement of the spleen is a hallmark of DFCL, which may lead to abdominal discomfort or a feeling of fullness.
• Fatigue: Many patients report persistent fatigue, which can be attributed to the disease itself or associated anemia.
• Weight Loss: Unintentional weight loss is common and may be a result of increased metabolic demands or decreased appetite.
• Night Sweats: Patients may experience drenching night sweats, which are often associated with lymphoproliferative disorders.
• Fever: Low-grade fevers may occur, particularly in more advanced stages of the disease.

Signs on Physical Examination

During a physical examination, healthcare providers may observe:

• Palpable Lymph Nodes: Enlarged lymph nodes that are firm but not typically painful.
• Abdominal Distension: Due to splenomegaly or lymphadenopathy in the abdominal cavity.
• Hepatomegaly: In some cases, the liver may also be enlarged, indicating more extensive disease involvement.

Patient Characteristics

Demographics

• Age: DFCL is more commonly diagnosed in older adults, particularly those aged 60 and above.
• Gender: There is a slight male predominance in the incidence of non-Hodgkin lymphomas, including DFCL.

Risk Factors

Several risk factors may contribute to the development of diffuse follicle center lymphoma, including:

• Immunosuppression: Patients with compromised immune systems, such as those with HIV/AIDS or those undergoing immunosuppressive therapy, are at increased risk.
• Family History: A family history of lymphoproliferative disorders may suggest a genetic predisposition.
• Environmental Exposures: Certain environmental factors, such as exposure to pesticides or chemicals, have been implicated in the development of lymphomas.

Histological Characteristics

Histologically, DFCL is characterized by:

• Follicular Architecture: The presence of neoplastic follicles that disrupt normal lymphoid architecture.
• B-cell Markers: Tumor cells typically express B-cell markers such as CD19, CD20, and CD10, which are important for diagnosis and treatment planning.

Conclusion

Diffuse follicle center lymphoma, particularly when involving the spleen (ICD-10 code C82.57), presents with a range of clinical symptoms and signs that can significantly impact patient quality of life. Early recognition of symptoms such as lymphadenopathy, splenomegaly, and systemic symptoms like fatigue and night sweats is crucial for timely diagnosis and management. Understanding patient demographics and risk factors can aid healthcare providers in identifying at-risk individuals and implementing appropriate screening and treatment strategies.