ICD-10: C82.47

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 C82.47 refers specifically to follicular lymphoma grade IIIb located in the spleen. Here’s a detailed overview of the standard treatment approaches for this condition.

Overview of Follicular Lymphoma Grade IIIb

Follicular lymphoma is classified into different grades based on the histological features observed under a microscope. Grade IIIb is the most aggressive form, often presenting with a higher proliferation index and a tendency to transform into more aggressive forms of lymphoma. The involvement of the spleen can complicate the disease, necessitating a tailored treatment approach.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone of treatment for follicular lymphoma grade IIIb. 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 advanced-stage follicular lymphoma and is effective in inducing remission[1].

• R-CVP: This regimen includes Rituximab, Cyclophosphamide, Vincristine, and Prednisone. It is typically used for patients who may not tolerate the more intensive R-CHOP regimen[2].

2. Targeted Therapy

Targeted therapies have emerged as significant options for treating follicular lymphoma:

• Rituximab: This monoclonal antibody targets CD20 on B-cells and is used both as a single agent and in combination with chemotherapy. It has been shown to improve outcomes in patients with follicular lymphoma[3].

• Idelalisib: An oral inhibitor of PI3K, Idelalisib is used in combination with Rituximab for patients who have relapsed or refractory disease[4].

• Copanlisib (Aliqopa): This is another PI3K inhibitor that has shown efficacy in treating follicular lymphoma, particularly in patients who have received at least two prior therapies[5].

3. Immunotherapy

Immunotherapy options are increasingly being explored:

• CAR T-cell Therapy: Chimeric Antigen Receptor (CAR) T-cell therapy has shown promise in treating relapsed or refractory follicular lymphoma. This approach involves modifying a patient’s T-cells to better target and destroy lymphoma cells[6].

• Lunsumio (mosunetuzumab-axgb): This bispecific antibody targets both CD20 and CD3, facilitating T-cell activation against B-cell malignancies, including follicular lymphoma[7].

4. Radiation Therapy

Radiation therapy may be considered in specific scenarios, particularly for localized disease or for palliative care to relieve symptoms associated with splenic involvement. It is not typically the primary treatment for advanced-stage follicular lymphoma but can be effective in certain cases[8].

5. Stem Cell Transplantation

For patients with high-risk or relapsed follicular lymphoma, autologous stem cell transplantation may be considered. This approach is generally reserved for younger patients or those with significant disease burden after initial therapy[9].

Conclusion

The treatment of follicular lymphoma grade IIIb, particularly when involving the spleen, requires a multifaceted approach that may include chemotherapy, targeted therapies, immunotherapy, and possibly radiation or stem cell transplantation. The choice of treatment is influenced by various factors, including the patient's overall health, disease stage, and prior treatment history. Ongoing clinical trials continue to explore new therapies and combinations to improve outcomes for patients with this challenging form of lymphoma.

For personalized treatment plans, it is essential for patients to consult with a hematologist or oncologist specializing in lymphomas.

Follicular lymphoma is a type of non-Hodgkin lymphoma characterized by the proliferation of B-lymphocytes. The ICD-10-CM code C82.47 specifically refers to Follicular lymphoma grade IIIb located in the spleen. Below is a detailed clinical description and relevant information regarding this condition.

Overview of Follicular Lymphoma

Definition

Follicular lymphoma is a slow-growing (indolent) form of non-Hodgkin lymphoma that arises from the follicle center (germinal center) of lymphoid tissue. It is classified as a B-cell neoplasm and is one of the most common types of lymphoma, accounting for approximately 20% of all non-Hodgkin lymphoma cases[2].

Grading

Follicular lymphoma is graded based on the number of large cells (centroblasts) present in the tissue sample:
- Grade I: 0-5 centroblasts per high-power field (HPF)
- Grade II: 6-15 centroblasts per HPF
- Grade III: More than 15 centroblasts per HPF, which is further divided into:
- Grade IIIa: Predominantly small cells with some large cells
- Grade IIIb: Predominantly large cells, often resembling diffuse large B-cell lymphoma (DLBCL) in behavior and treatment response[1][4].

Clinical Features of Follicular Lymphoma Grade IIIb

Symptoms

Patients with follicular lymphoma may present with a variety of symptoms, including:
- Painless lymphadenopathy (swelling of lymph nodes)
- Splenomegaly (enlargement of the spleen)
- Fever, night sweats, and weight loss (B symptoms)
- Fatigue and weakness

Diagnosis

Diagnosis typically involves:
- Histopathological examination: A biopsy of the affected lymphoid tissue (e.g., spleen) is essential for confirming the diagnosis and grading the lymphoma.
- Immunophenotyping: This process helps identify the specific type of lymphoma by analyzing the surface markers on the cells.
- Imaging studies: CT scans, PET scans, or MRI may be used to assess the extent of the disease and involvement of other organs.

Treatment

Treatment options for follicular lymphoma grade IIIb may include:
- Chemotherapy: Common regimens include combinations of agents such as bendamustine, rituximab, or CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone).
- Immunotherapy: Monoclonal antibodies like rituximab are often used in conjunction with chemotherapy.
- Radiation therapy: May be considered for localized disease or as palliative care.
- Stem cell transplant: In some cases, especially for relapsed or refractory disease, an allogeneic stem cell transplant may be indicated[3][5].

Prognosis

The prognosis for patients with follicular lymphoma grade IIIb can vary significantly based on several factors, including the patient's age, overall health, and response to treatment. Generally, grade IIIb has a more aggressive course compared to lower grades, and patients may require more intensive treatment strategies.

Conclusion

ICD-10 code C82.47 identifies follicular lymphoma grade IIIb specifically affecting the spleen. This condition is characterized by a predominance of large B-cells and requires careful diagnosis and management due to its aggressive nature. Understanding the clinical features, diagnostic methods, and treatment options is crucial for effective patient care and improving outcomes in individuals diagnosed with this type of lymphoma.

Follicular lymphoma grade IIIb, classified under ICD-10 code C82.47, 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 can transform into a more aggressive form. Grade IIIb indicates a higher proliferation of malignant cells, which can lead to more pronounced symptoms and a different clinical course compared to lower grades. The involvement of the spleen in this subtype can further influence the clinical presentation.

Signs and Symptoms

1. Lymphadenopathy:
   - Patients often present with painless swelling of lymph nodes, particularly in the neck, axilla, or groin. In grade IIIb, lymphadenopathy may be more pronounced and widespread due to the aggressive nature of the disease[1].

2. Splenomegaly:
   - Enlargement of the spleen is a common finding in patients with follicular lymphoma, especially in those with grade IIIb. This can lead to discomfort or a feeling of fullness in the abdomen[1][2].

3. B Symptoms:
   - Patients may experience systemic symptoms known as "B symptoms," which include:

   • Unexplained fever
   • Night sweats
   • Significant weight loss (more than 10% of body weight over six months) [2].

4. Fatigue:
   - A common complaint among patients, often due to the disease's impact on overall health and the body's response to cancer[1].

5. Abdominal Pain or Discomfort:
   - This may occur due to splenomegaly or lymphadenopathy affecting abdominal organs[2].

6. Cytopenias:
   - Patients may present with anemia, thrombocytopenia, or leukopenia due to bone marrow involvement, which can lead to increased fatigue, bleeding tendencies, or increased susceptibility to infections[1][2].

Patient Characteristics

1. 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[1].

2. Gender:
   - There is a slight male predominance in the incidence of follicular lymphoma, although it affects both genders[1].

3. Comorbidities:
   - Patients may have other health conditions that can complicate treatment and management, such as cardiovascular disease or diabetes, which are more common in older populations[2].

4. Family History:
   - A family history of lymphoproliferative disorders may be noted in some patients, suggesting a potential genetic predisposition[1].

5. Ethnicity:
   - While follicular lymphoma can affect individuals of any ethnicity, some studies suggest variations in incidence and outcomes among different ethnic groups, although the reasons for these differences are not fully understood[2].

Conclusion

Follicular lymphoma grade IIIb, particularly with splenic involvement, presents with a range of clinical signs and symptoms that reflect its aggressive nature. Key features include lymphadenopathy, splenomegaly, systemic B symptoms, and potential cytopenias. Understanding these characteristics is essential for timely diagnosis and effective management of the disease. Regular monitoring and a comprehensive approach to treatment can significantly impact patient outcomes.

Follicular lymphoma grade IIIb, classified under ICD-10 code C82.47, is a specific type of non-Hodgkin lymphoma characterized by the proliferation of follicle center (germinal center) B-cells. This condition is part of a broader category of lymphomas and has several alternative names and related terms that can be useful for understanding its classification and implications in medical coding and billing.

Alternative Names for Follicular Lymphoma Grade IIIb

1. Follicular Lymphoma, High-Grade: This term emphasizes the aggressive nature of grade IIIb compared to lower grades of follicular lymphoma.
2. Follicular Lymphoma, Grade 3b: A simplified version of the full name, often used in clinical settings.
3. Follicular Lymphoma, Diffuse: In some contexts, grade IIIb may be referred to as diffuse follicular lymphoma due to its more aggressive behavior and the presence of diffuse patterns in histological examination.
4. B-cell Follicular Lymphoma: This term highlights the B-cell origin of the lymphoma, which is crucial for understanding its pathophysiology.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): Follicular lymphoma is a subtype of non-Hodgkin lymphoma, which encompasses a wide range of lymphoid malignancies.
2. Lymphoproliferative Disorders: This broader category includes various conditions characterized by the excessive proliferation of lymphocytes, including follicular lymphoma.
3. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.
4. Spleen Involvement: Since C82.47 specifies involvement of the spleen, terms like "splenic lymphoma" or "spleen involvement in lymphoma" may also be relevant in clinical discussions.

Clinical Context

Understanding these alternative names and related terms is essential for healthcare professionals involved in diagnosis, treatment, and coding of follicular lymphoma grade IIIb. Accurate coding is crucial for appropriate billing and insurance reimbursement, as well as for clinical research and epidemiological studies.

In summary, the ICD-10 code C82.47 for follicular lymphoma grade IIIb encompasses various alternative names and related terms that reflect its clinical characteristics and classification within the broader category of lymphomas. Familiarity with these terms can enhance communication among healthcare providers and improve patient care outcomes.

Follicular lymphoma, particularly grade IIIb, is a subtype of non-Hodgkin lymphoma characterized by specific histological features and clinical presentations. The diagnosis of follicular lymphoma, including grade IIIb, involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria used for diagnosis, particularly relevant to the ICD-10 code C82.47, which specifies follicular lymphoma grade IIIb located in the spleen.

Clinical Evaluation

1. Symptoms: Patients may present with symptoms such as:
   - Painless lymphadenopathy (swelling of lymph nodes)
   - Splenomegaly (enlargement of the spleen)
   - B symptoms (fever, night sweats, weight loss)

2. Physical Examination: A thorough physical examination is essential to assess for lymphadenopathy and splenomegaly.

Imaging Studies

1. CT Scans: Computed tomography (CT) scans of the abdomen and pelvis can help visualize lymphadenopathy and splenic involvement.

2. PET Scans: Positron emission tomography (PET) scans may be utilized to assess metabolic activity of the lymphoma and to evaluate for any additional sites of disease.

Histopathological Examination

1. Biopsy: A definitive diagnosis requires a biopsy of the affected tissue, which can be obtained from lymph nodes, spleen, or other involved sites. The biopsy specimen is then examined microscopically.

2. Histological Features: For follicular lymphoma grade IIIb, the following histological criteria are critical:
   - Follicular Architecture: The presence of neoplastic follicles, but with a predominance of large cells.
   - Cell Type: The neoplastic cells are typically large B-cells, and the presence of a high number of centroblasts (large B-cells) is characteristic of grade IIIb.
   - Immunophenotyping: Immunohistochemical staining is performed to confirm the B-cell lineage (positive for CD19, CD20, and CD10) and to assess the proliferation index (Ki-67), which is usually high in grade IIIb.

3. Cytogenetic Studies: Genetic testing may be performed to identify chromosomal abnormalities, such as the t(14;18) translocation, which is commonly associated with follicular lymphoma.

Staging

1. Ann Arbor Staging System: The disease is staged using the Ann Arbor classification, which considers the number of lymph node regions involved, the presence of splenic involvement, and any systemic symptoms.

2. Spleen Involvement: The diagnosis of C82.47 specifically indicates that the lymphoma is located in the spleen, which is an important factor in staging and treatment planning.

Conclusion

The diagnosis of follicular lymphoma grade IIIb, particularly with splenic involvement (ICD-10 code C82.47), relies on a combination of clinical assessment, imaging studies, and detailed histopathological evaluation. The presence of large B-cells, a high proliferation index, and specific immunophenotypic markers are critical for confirming the diagnosis. Accurate staging is essential for determining the appropriate treatment strategy and prognosis for the patient.