ICD-10: C82.37

Follicular lymphoma grade IIIa, classified under ICD-10 code C82.37, 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 IIIa indicates a more aggressive form of follicular lymphoma, where there is a higher proliferation of neoplastic cells. When the spleen is involved, it can lead to various systemic and localized symptoms.

Signs and Symptoms

1. Lymphadenopathy:
   - Patients often present with painless swelling of lymph nodes, particularly in the neck, axilla, or groin. In grade IIIa, lymphadenopathy may be more pronounced 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 when the spleen is involved. 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:

   • Fever
   • Night sweats
   • Unexplained weight loss
     These symptoms can indicate a more advanced disease state and are often associated with higher-grade lymphomas[2].

4. Fatigue:
   - A common complaint among patients, fatigue can be due to the disease itself or related to anemia, which may occur as the lymphoma progresses[1].

5. Abdominal Pain or Discomfort:
   - As the spleen enlarges, it can cause abdominal pain or discomfort, particularly if it compresses surrounding organs[2].

6. Cytopenias:
   - Patients may present with blood count abnormalities, such as anemia, thrombocytopenia, or leukopenia, due to bone marrow infiltration by lymphoma cells[1].

Patient Characteristics

1. Age:
   - Follicular lymphoma typically affects adults, with a median age of diagnosis around 60 years. However, it can occur in younger individuals as well[2].

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 the management of lymphoma, such as cardiovascular disease or diabetes, which are more common in older adults[2].

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

5. Ethnicity:
   - Epidemiological studies indicate variations in incidence based on ethnicity, with higher rates observed in Caucasian populations compared to African American populations[2].

Conclusion

Follicular lymphoma grade IIIa, particularly when involving the spleen, presents with a range of clinical signs and symptoms that reflect its aggressive nature. Key features include lymphadenopathy, splenomegaly, systemic B symptoms, and fatigue. Understanding these characteristics is essential for timely diagnosis and effective management of the disease. Regular monitoring and comprehensive evaluation are crucial for patients diagnosed with this condition, given its potential for progression and the need for tailored therapeutic approaches.

Follicular lymphoma, particularly grade IIIa, is a subtype of non-Hodgkin lymphoma characterized by specific histological features and clinical presentations. The diagnosis of follicular lymphoma, including grade IIIa, 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 C82.37, which specifies follicular lymphoma grade IIIa 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 used to assess metabolic activity of the lymphoma and to evaluate for any other sites of disease.

Histopathological Examination

1. Biopsy: A definitive diagnosis requires a biopsy of the affected lymph node or spleen. The biopsy sample is examined microscopically.

2. Histological Features: For grade IIIa follicular lymphoma, the following features are typically observed:
   - Follicular Architecture: The presence of neoplastic follicles, which may be disrupted in grade IIIa.
   - Cellularity: Increased cellularity with a predominance of large cells (centroblasts).
   - Grading: Grade IIIa is characterized by 6-15 centroblasts per high-power field (HPF) in the biopsy sample.

3. Immunophenotyping: Immunohistochemical staining is performed to confirm the diagnosis. Key markers include:
   - CD10: Positive
   - BCL2: Positive
   - CD19: Positive
   - CD20: Positive
   - CD5: Typically negative

4. Cytogenetic Studies: Detection of the t(14;18) translocation, which involves the BCL2 gene, is often performed to support the diagnosis.

Laboratory Tests

1. Blood Tests: Complete blood count (CBC) may show anemia or thrombocytopenia, and lactate dehydrogenase (LDH) levels may be elevated, indicating tumor burden.

2. Bone Marrow Biopsy: In some cases, a bone marrow biopsy may be performed to assess for marrow involvement by lymphoma.

Conclusion

The diagnosis of follicular lymphoma grade IIIa, particularly in the spleen, relies on a combination of clinical findings, imaging studies, and detailed histopathological analysis. The integration of these diagnostic criteria is crucial for accurate classification and subsequent management of the disease. If you have further questions or need more specific information, feel free to ask!

ICD-10 code C82.37 specifically refers to Follicular lymphoma grade IIIa located in the spleen. This classification is part of the broader category of non-Hodgkin lymphomas, which are a diverse group of blood cancers that include various subtypes. Below are alternative names and related terms associated with this specific diagnosis.

Alternative Names for Follicular Lymphoma Grade IIIa

1. Follicular Lymphoma, Grade 3A: This is a direct alternative name that maintains the same meaning as C82.37.
2. Follicular Lymphoma, Spleen Involvement: This term emphasizes the specific location of the lymphoma within the spleen.
3. Follicular Lymphoma, Grade III: While this term is broader, it can refer to the same grade of lymphoma without specifying the spleen.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): Follicular lymphoma is a subtype of non-Hodgkin lymphoma, which encompasses a wide range of lymphatic cancers.
2. Lymphoproliferative Disorders: This term refers to conditions where lymphocytes are produced in excessive quantities, including various types of lymphomas.
3. B-cell Lymphoma: Follicular lymphoma is classified as a B-cell lymphoma, indicating that it originates from B lymphocytes, a type of white blood cell.
4. Spleen Lymphoma: This term can be used to describe lymphomas that specifically involve the spleen, including follicular lymphoma.
5. Grade III Lymphoma: This term refers to the aggressiveness of the lymphoma, indicating a higher grade of malignancy.

Clinical Context

Follicular lymphoma is characterized by its indolent nature, but grade IIIa indicates a more aggressive form of the disease. The involvement of the spleen can affect treatment decisions and prognosis. Understanding these alternative names and related terms is crucial for accurate communication among healthcare providers and for coding purposes in medical billing and documentation.

In summary, while C82.37 specifically denotes Follicular lymphoma grade IIIa in the spleen, it is important to recognize the broader context of non-Hodgkin lymphomas and the various terminologies that may be used interchangeably or in related discussions.

ICD-10 code C82.37 refers specifically to Follicular lymphoma grade IIIa, which is a subtype of non-Hodgkin lymphoma (NHL) primarily affecting the spleen. Below is a detailed clinical description and relevant information regarding this condition.

Overview of Follicular Lymphoma

Follicular lymphoma is a type of indolent (slow-growing) non-Hodgkin lymphoma that originates from B-lymphocytes, which are a type of white blood cell. It is characterized by the formation of follicle-like structures in lymphoid tissue. The disease is classified into several grades based on the histological features observed under a microscope, with grade IIIa indicating a more aggressive form compared to lower grades.

Characteristics of Follicular Lymphoma Grade IIIa

• Histological Features: Follicular lymphoma grade IIIa is characterized by a predominance of large cells (centroblasts) in the follicles, with a higher number of these cells compared to grades I and II. The presence of at least 15 centroblasts per high-power field is a defining feature of grade IIIa[1].
• Clinical Presentation: Patients may present with lymphadenopathy (swollen lymph nodes), splenomegaly (enlarged spleen), and sometimes bone marrow involvement. Symptoms can include fatigue, weight loss, fever, and night sweats, although some patients may be asymptomatic at diagnosis[2].
• Prognosis: Grade IIIa follicular lymphoma is considered more aggressive than lower-grade forms, with a higher risk of transformation to a more aggressive lymphoma, such as diffuse large B-cell lymphoma (DLBCL). The prognosis can vary significantly based on individual factors, including age, overall health, and response to treatment[3].

Diagnosis and Staging

Diagnosis typically involves a combination of clinical evaluation, imaging studies (such as CT scans), and biopsy of affected lymph nodes or spleen tissue. The biopsy is crucial for determining the grade of the lymphoma and guiding treatment decisions. Staging is performed according to the Ann Arbor classification, which assesses the extent of disease spread in the body[4].

Treatment Options

Treatment for follicular lymphoma grade IIIa may include:

• Chemotherapy: Often combined with immunotherapy (e.g., rituximab) to enhance effectiveness.
• Radiation Therapy: May be used in localized disease or as palliative care.
• Targeted Therapy: Newer agents targeting specific pathways involved in lymphoma growth are being explored.
• Stem Cell Transplantation: In some cases, especially for relapsed or refractory disease, autologous stem cell transplantation may be considered[5].

Conclusion

ICD-10 code C82.37 identifies follicular lymphoma grade IIIa, a more aggressive form of this type of lymphoma affecting the spleen. Understanding its clinical features, diagnostic criteria, and treatment options is essential for effective management. As research continues, new therapies and approaches are being developed to improve outcomes for patients with this condition.

For further information or specific case management, consulting with a hematologist or oncologist specializing in lymphomas is recommended.

References

1. Local Coverage Determination (LCD) for Hematologic Malignancies.
2. Non-Hodgkin Lymphoma Overview.
3. Prognostic Factors in Follicular Lymphoma.
4. Ann Arbor Staging System for Lymphoma.
5. Treatment Guidelines for Follicular Lymphoma.

Follicular lymphoma, particularly grade IIIa, is a subtype of non-Hodgkin lymphoma characterized by its indolent nature and specific histological features. The ICD-10 code C82.37 refers to follicular lymphoma localized to the spleen. Treatment approaches for this condition can vary based on several factors, including the patient's overall health, the extent of the disease, and specific clinical characteristics. Below is a detailed overview of standard treatment approaches for follicular lymphoma grade IIIa.

Overview of Follicular Lymphoma Grade IIIa

Follicular lymphoma is classified into different grades based on the number of large cells present in the tumor. Grade IIIa indicates a higher proportion of large cells compared to lower grades, which can influence treatment decisions. This subtype is often more aggressive than lower-grade follicular lymphomas but still tends to respond well to treatment.

Standard Treatment Approaches

1. Watchful Waiting

In cases where the lymphoma is asymptomatic and not causing significant health issues, a strategy of "watchful waiting" may be employed. This involves regular monitoring without immediate treatment, as many patients with follicular lymphoma can live for years without requiring intervention.

2. Chemotherapy

For symptomatic patients or those with rapidly progressing disease, chemotherapy is a common treatment option. The most frequently used regimens include:

• CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. It is a standard treatment for aggressive lymphomas and may be used in combination with other therapies.
• R-CHOP: This is a variation of the CHOP regimen that includes Rituximab, a monoclonal antibody that targets CD20 on B-cells, enhancing the effectiveness of chemotherapy.

3. Immunotherapy

Rituximab is a cornerstone of treatment for follicular lymphoma. It can be used alone or in combination with chemotherapy. In cases of relapsed or refractory disease, newer agents such as:

• Obinutuzumab: Another anti-CD20 monoclonal antibody that may be used in combination with chemotherapy.
• Lenalidomide: An oral immunomodulatory drug that can be effective in relapsed cases.

4. Radiation Therapy

Localized radiation therapy may be considered for patients with limited-stage disease or for those with bulky disease in the spleen. It can be particularly effective in controlling localized symptoms and reducing tumor burden.

5. Stem Cell Transplantation

For patients with relapsed or refractory follicular lymphoma, especially those with high-risk features, autologous stem cell transplantation may be an option. This approach involves harvesting the patient's stem cells, administering high-dose chemotherapy, and then reinfusing the stem cells to restore bone marrow function.

6. Targeted Therapies

Recent advancements have led to the development of targeted therapies that focus on specific pathways involved in lymphoma growth. These include:

• PI3K Inhibitors: Such as copanlisib (Aliqopa), which has shown efficacy in treating relapsed follicular lymphoma.
• BCL-2 Inhibitors: Like venetoclax, which can be effective in certain patient populations.

Conclusion

The treatment of follicular lymphoma grade IIIa, particularly when localized to the spleen, involves a multifaceted approach tailored to the individual patient's needs and disease characteristics. While watchful waiting may be appropriate for some, others may require chemotherapy, immunotherapy, or targeted treatments. Ongoing research continues to refine these approaches, aiming to improve outcomes and quality of life for patients with this complex disease. Regular follow-up and monitoring are essential to adapt treatment plans as needed and to manage any potential side effects effectively.