ICD-10: C82.42

Follicular lymphoma is a type of non-Hodgkin lymphoma characterized by the proliferation of follicle center (germinal center) B-cells. It is generally considered an indolent (slow-growing) form of lymphoma, but it can transform into a more aggressive form over time. The ICD-10 code C82.42 specifically refers to follicular lymphoma grade IIIb that is localized in the intrathoracic lymph nodes.

Clinical Description of Follicular Lymphoma Grade IIIb

Definition and Classification

Follicular lymphoma is classified into different grades based on the histological features observed under a microscope. Grade IIIb indicates a higher degree of malignancy compared to lower grades (I and II). In grade IIIb, the lymphoma is characterized by a predominance of large cells, which can lead to a more aggressive clinical course. This grade is often associated with a higher risk of transformation to diffuse large B-cell lymphoma (DLBCL), which is a more aggressive form of lymphoma.

Symptoms

Patients with follicular lymphoma may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, which may be painless.
- B-symptoms: These include fever, night sweats, and unexplained weight loss.
- Fatigue: Generalized tiredness and lack of energy.
- Abdominal discomfort: If lymph nodes in the abdomen are involved.

Intrathoracic Involvement

The designation of intrathoracic lymph nodes indicates that the lymphoma has spread to lymph nodes located within the thoracic cavity, which may include mediastinal or hilar lymph nodes. This can lead to additional symptoms such as:
- Cough: Due to compression of the airways.
- Chest pain: Resulting from lymph node enlargement.
- Shortness of breath: If lymph nodes compress the lungs or major airways.

Diagnosis

Diagnosis of follicular lymphoma grade IIIb typically involves:
- Biopsy: A tissue sample from an affected lymph node is examined histologically to confirm the diagnosis and grade.
- Imaging Studies: CT scans or PET scans are often used to assess the extent of disease and involvement of lymph nodes, particularly in the thoracic region.
- Blood Tests: These may include complete blood counts and tests for lactate dehydrogenase (LDH), which can indicate tumor burden.

Treatment Options

Treatment for follicular lymphoma grade IIIb may vary based on the stage of the disease, symptoms, and overall health of the patient. Common treatment modalities include:
- Chemotherapy: Often combined with immunotherapy (e.g., rituximab) for more effective results.
- Radiation Therapy: May be used for localized disease or to alleviate symptoms.
- Targeted Therapy: Newer agents that specifically target cancer cells may be considered.
- Stem Cell Transplant: In some cases, particularly for relapsed or refractory disease, an allogeneic or autologous stem cell transplant may be indicated.

Prognosis

The prognosis for patients with follicular lymphoma grade IIIb can vary significantly based on several factors, including the patient's age, overall health, response to treatment, and the presence of any transformation to a more aggressive form of lymphoma. Generally, the prognosis is less favorable than for lower-grade follicular lymphomas, but advancements in treatment continue to improve outcomes.

In summary, ICD-10 code C82.42 identifies a specific and serious form of follicular lymphoma that requires careful diagnosis and management due to its potential for aggressive behavior and complications associated with intrathoracic lymph node involvement.

Follicular lymphoma grade IIIb, classified under ICD-10 code C82.42, is a subtype of non-Hodgkin lymphoma characterized by specific clinical presentations, signs, symptoms, and patient characteristics. Understanding these aspects is crucial for diagnosis and management.

Clinical Presentation

Follicular lymphoma is a type of indolent (slow-growing) lymphoma that originates from B-lymphocytes. Grade IIIb indicates a more aggressive form of follicular lymphoma, where the cancer cells exhibit a higher proliferation rate. The involvement of intrathoracic lymph nodes suggests that the disease has spread to lymph nodes located within the thoracic cavity, which can influence the clinical presentation.

Signs and Symptoms

Patients with follicular lymphoma grade IIIb may present with a variety of signs and symptoms, including:

• Lymphadenopathy: Swelling of lymph nodes is often the most noticeable sign. In grade IIIb, lymph nodes may be enlarged in the neck, axilla, or groin, and specifically in the thoracic region, which can lead to mediastinal lymphadenopathy.

• B Symptoms: These include systemic symptoms such as:

• Fever: Unexplained fevers that may be intermittent.
• Night Sweats: Profuse sweating during the night.

• Weight Loss: Unintentional weight loss exceeding 10% of body weight over six months.

• Respiratory Symptoms: If intrathoracic lymph nodes are involved, patients may experience:

• Cough: Persistent cough due to compression of the airways.
• Dyspnea: Shortness of breath, especially if lymph nodes compress the trachea or bronchi.

• Chest Pain: Discomfort or pain in the chest area, which may be related to lymph node enlargement.

• Fatigue: A common symptom in many cancer patients, often due to the disease itself or associated anemia.

Patient Characteristics

Certain demographic and clinical characteristics are often associated with patients diagnosed with follicular lymphoma grade IIIb:

• Age: Follicular lymphoma typically occurs in adults, with a median age of diagnosis around 60 years. However, it can occur in younger individuals as well.

• Gender: There is a slight male predominance in the incidence of follicular lymphoma.

• History of Autoimmune Disorders: Some patients may have a history of autoimmune diseases, which have been associated with an increased risk of developing lymphomas.

• Family History: A family history of lymphoproliferative disorders may also be noted in some patients.

• Comorbidities: Patients may present with other health conditions, which can complicate the management of lymphoma.

Conclusion

Follicular lymphoma grade IIIb, particularly with intrathoracic lymph node involvement, presents with a range of clinical signs and symptoms that can significantly impact a patient's quality of life. Recognizing these manifestations is essential for timely diagnosis and appropriate treatment. Clinicians should consider the patient's age, gender, and any relevant medical history when evaluating potential cases of this lymphoma subtype. Early intervention can lead to better management outcomes and improved patient prognosis.

When discussing the ICD-10 code C82.42, which specifically refers to Follicular lymphoma grade IIIb affecting the intrathoracic lymph nodes, it is helpful to understand the alternative names and related terms associated with this diagnosis. Below is a detailed overview of these terms.

Alternative Names for Follicular Lymphoma Grade IIIb

1. Follicular Lymphoma, Grade 3b: This is a direct alternative name that specifies the grade of the lymphoma, indicating a more aggressive form of follicular lymphoma.

2. Follicular Non-Hodgkin Lymphoma (NHL): Since follicular lymphoma is a subtype of non-Hodgkin lymphoma, this term is often used interchangeably, although it encompasses a broader category of lymphomas.

3. Follicular Lymphoma, High-Grade: This term emphasizes the aggressive nature of grade IIIb follicular lymphoma, which is characterized by a higher proliferation rate compared to lower grades.

4. Intrathoracic Follicular Lymphoma: This term highlights the specific location of the lymphoma, indicating that it is present in the lymph nodes located within the thoracic cavity.

Related Terms

1. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with follicular lymphoma.

2. B-cell Lymphoma: Follicular lymphoma is classified as a B-cell lymphoma, as it originates from B lymphocytes, a type of white blood cell.

3. Stage IV Lymphoma: Depending on the extent of the disease, follicular lymphoma can be classified as stage IV if it has spread to distant lymph nodes or organs, including intrathoracic locations.

4. Chronic Lymphocytic Leukemia (CLL): While distinct, CLL can sometimes be confused with follicular lymphoma due to overlapping characteristics, particularly in the context of B-cell malignancies.

5. Lymphoma, Grade III: This broader term can refer to any lymphoma classified as grade III, which includes both follicular and other types of lymphomas.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C82.42 is crucial for accurate diagnosis, treatment planning, and communication among healthcare professionals. These terms not only help in identifying the specific type of lymphoma but also in understanding its implications for patient care and management. If you have further questions or need more specific information, feel free to ask!

Follicular lymphoma, particularly classified under ICD-10 code C82.42, refers to a specific subtype of non-Hodgkin lymphoma characterized by its indolent nature and follicular growth pattern. The diagnosis of follicular lymphoma, especially grade IIIb, involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria used for diagnosis:

Clinical Evaluation

1. Symptoms: Patients may present with symptoms such as painless lymphadenopathy, fever, night sweats, and weight loss. These systemic symptoms, often referred to as "B symptoms," can indicate more advanced disease.

2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy, particularly in the cervical, axillary, and inguinal regions, as well as any splenomegaly or hepatomegaly.

Imaging Studies

1. CT Scans: Computed tomography (CT) scans of the chest, abdomen, and pelvis are commonly used to assess the extent of lymphadenopathy and to identify any involvement of intrathoracic lymph nodes.

2. PET Scans: Positron emission tomography (PET) scans may be utilized to evaluate metabolic activity in lymph nodes and to help differentiate between active disease and residual masses post-treatment.

Histopathological Examination

1. Biopsy: A definitive diagnosis of follicular lymphoma requires a biopsy of the affected lymph node or tissue. The biopsy can be performed via excisional, incisional, or fine-needle aspiration methods.

2. Microscopic Analysis: The histological examination will reveal a characteristic follicular pattern of neoplastic cells. In grade IIIb follicular lymphoma, there is a predominance of large cells, which can be identified through specific staining techniques.

3. Immunophenotyping: Flow cytometry or immunohistochemistry is used to assess the expression of specific markers. Follicular lymphoma typically expresses CD10, BCL2, and BCL6, while lacking CD5.

4. Genetic Studies: The presence of the t(14;18) chromosomal translocation, which results in the overexpression of the BCL2 gene, is a hallmark of follicular lymphoma and can be confirmed through cytogenetic analysis or molecular techniques.

Staging

The Ann Arbor staging system is often employed to determine the extent of the disease, which is crucial for treatment planning. In the case of C82.42, the involvement of intrathoracic lymph nodes indicates a more advanced stage of the disease.

Conclusion

The diagnosis of follicular lymphoma grade IIIb, particularly with involvement of intrathoracic lymph nodes (ICD-10 code C82.42), relies on a comprehensive approach that includes clinical assessment, imaging studies, and detailed histopathological evaluation. Accurate diagnosis is essential for determining the appropriate treatment strategy and prognosis for the patient.

Follicular lymphoma, particularly grade IIIb, is a subtype of non-Hodgkin lymphoma characterized by its aggressive nature and specific treatment requirements. The ICD-10 code C82.42 refers to follicular lymphoma grade IIIb located in the intrathoracic lymph nodes. Understanding the standard treatment approaches for this condition is crucial for effective management.

Overview of Follicular Lymphoma Grade IIIb

Follicular lymphoma is classified into different grades based on histological features, with grade IIIb being the most aggressive form. It typically presents with a higher proliferation index and may exhibit more aggressive clinical behavior compared to lower-grade follicular lymphomas. The involvement of intrathoracic lymph nodes can complicate the disease, necessitating a tailored treatment approach.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone of treatment for grade IIIb follicular lymphoma. Common 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 aggressive lymphomas, including grade IIIb follicular lymphoma[1].
• R-CVP: This regimen includes Rituximab, Cyclophosphamide, and Prednisone, and may be considered for patients who are not candidates for more intensive chemotherapy[2].

2. Targeted Therapy

Targeted therapies have emerged as significant options in the treatment of follicular lymphoma:

• Obinutuzumab (Gazyva): This is a monoclonal antibody that targets CD20 and is used in combination with chemotherapy or as a single agent in relapsed cases[3].
• Copanlisib (Aliqopa): An oral PI3K inhibitor that has shown efficacy in treating relapsed follicular lymphoma, particularly in patients who have received at least two prior therapies[4].

3. Radiation Therapy

Radiation therapy may be utilized in specific scenarios, particularly for localized disease or as a consolidation treatment after chemotherapy. Intensity-modulated radiation therapy (IMRT) can be particularly effective in targeting intrathoracic lymph nodes while minimizing exposure to surrounding healthy tissues[5].

4. Stem Cell Transplantation

For patients with relapsed or refractory follicular lymphoma, autologous stem cell transplantation (ASCT) may be considered, especially after achieving a complete response to initial therapy. This approach can provide a chance for long-term remission[6].

5. Clinical Trials

Participation in clinical trials can be an option for patients, offering access to novel therapies and treatment strategies that may not yet be widely available. Trials may focus on new combinations of chemotherapy, targeted therapies, or immunotherapies[7].

Conclusion

The treatment of follicular lymphoma grade IIIb, particularly when involving intrathoracic lymph nodes, requires a comprehensive approach that may include chemotherapy, targeted therapies, radiation, and potentially stem cell transplantation. The choice of treatment should be individualized based on the patient's overall health, disease characteristics, and preferences. Ongoing research and clinical trials continue to shape the landscape of treatment options, providing hope for improved outcomes in this challenging disease.

For patients and healthcare providers, staying informed about the latest advancements in treatment is essential for optimizing care and enhancing quality of life.