ICD-10: C82.21

Follicular lymphoma grade III, unspecified, lymph nodes of head, face, and neck

Additional Information

Clinical Information

Follicular lymphoma, particularly grade III, is a type of non-Hodgkin lymphoma characterized by the proliferation of follicle center (germinal center) B-cells. The ICD-10 code C82.21 specifically refers to follicular lymphoma grade III that is unspecified and localized to the lymph nodes of the head, face, and neck. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for diagnosis and management.

Clinical Presentation

Signs and Symptoms

Patients with follicular lymphoma grade III may present with a variety of signs and symptoms, which can include:

  • Lymphadenopathy: The most common presentation is painless swelling of lymph nodes, particularly in the cervical (neck) region, which may also involve the head and face. These lymph nodes can be firm and rubbery in texture.
  • B Symptoms: Some 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)
  • Fatigue: Patients often report a general sense of fatigue or malaise, which can be attributed to the disease process or anemia.
  • Pruritus: Itching without a rash may occur in some patients, although it is less common.
  • Abdominal Symptoms: In cases where the lymphoma has spread, patients may experience abdominal pain or discomfort due to lymphadenopathy in the abdominal region.

Patient Characteristics

Certain demographic and clinical characteristics are often associated with follicular lymphoma:

  • Age: Follicular lymphoma typically occurs in adults, with a median age of diagnosis around 60 years. It is relatively rare in younger individuals.
  • Gender: There is a slight male predominance, with men being more frequently diagnosed than women.
  • History of Autoimmune Disorders: Some patients may have a history of autoimmune diseases, which has been associated with an increased risk of developing lymphomas.
  • Family History: A family history of lymphoproliferative disorders may also be noted in some patients, suggesting a potential genetic predisposition.

Diagnosis

The diagnosis of follicular lymphoma grade III involves a combination of clinical evaluation, imaging studies, and histopathological examination:

  • Imaging: CT scans or PET scans may be utilized to assess the extent of lymphadenopathy and to rule out other conditions.
  • Biopsy: A definitive diagnosis is made through a lymph node biopsy, where histological examination reveals the characteristic follicular architecture and the presence of grade III features, such as a higher proliferation index (often assessed by Ki-67 staining).

Conclusion

Follicular lymphoma grade III, particularly when localized to the lymph nodes of the head, face, and neck, presents with specific clinical features that are essential for diagnosis and management. Recognizing the signs and symptoms, along with understanding patient demographics, can aid healthcare providers in identifying this condition early and implementing appropriate treatment strategies. Regular follow-up and monitoring are crucial, as follicular lymphoma can be indolent but may transform into a more aggressive form over time.

Treatment Guidelines

Follicular lymphoma, particularly grade III, is a subtype of non-Hodgkin lymphoma characterized by its aggressive nature and specific treatment requirements. The ICD-10 code C82.21 refers to follicular lymphoma grade III that is unspecified and located in the lymph nodes of the head, face, and neck. Understanding the standard treatment approaches for this condition is crucial for effective management.

Overview of Follicular Lymphoma Grade III

Follicular lymphoma is classified into different grades based on the histological characteristics of the cancer cells. Grade III follicular lymphoma is further divided into IIIA and IIIB, with IIIA being less aggressive than IIIB. The disease typically presents with painless lymphadenopathy, and in the case of grade III, it may involve more aggressive treatment strategies due to its higher proliferation index and potential for transformation into a more aggressive form of lymphoma.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone of treatment for grade III follicular 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 advanced-stage follicular lymphoma and is effective in inducing remission[1].
  • R-CVP: This regimen includes Rituximab, Cyclophosphamide, Vincristine, and Prednisone, and may be used in patients who are not candidates for more intensive chemotherapy[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 often used in combination with chemotherapy or as a maintenance therapy after initial treatment[3].
  • PI3K Inhibitors: Drugs like Copanlisib (Aliqopa) are used for relapsed follicular lymphoma and may be considered in cases where traditional chemotherapy is not effective or suitable[4].

3. Radiation Therapy

Radiation therapy can be an effective treatment for localized disease, particularly in cases where the lymphoma is confined to specific lymph nodes in the head, face, and neck. Intensity Modulated Radiation Therapy (IMRT) is often employed to minimize damage to surrounding healthy tissues while effectively targeting the lymphoma[5].

4. Stem Cell Transplantation

For patients with relapsed or refractory follicular lymphoma, autologous stem cell transplantation may be considered. This approach is typically reserved for younger patients or those with a good performance status, as it involves high-dose chemotherapy followed by the reinfusion of the patient’s own stem cells[6].

5. Clinical Trials

Participation in clinical trials may 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 it may offer additional benefits, especially in cases of aggressive or refractory disease[7].

Conclusion

The management of follicular lymphoma grade III, particularly in the lymph nodes of the head, face, and neck, involves a multifaceted approach that includes chemotherapy, targeted therapies, radiation, and potentially stem cell transplantation. The choice of treatment is influenced by various factors, including the patient's overall health, the extent of the disease, and the specific characteristics of the lymphoma. Ongoing research and clinical trials continue to shape the landscape of treatment options, providing hope for improved outcomes in patients with this challenging condition.

For personalized treatment plans, it is essential for patients to consult with a hematologist or oncologist who specializes in lymphoma.

Description

ICD-10 code C82.21 refers to Follicular lymphoma grade III, unspecified, specifically affecting the lymph nodes of the head, face, and neck. This classification falls under the broader category of non-Hodgkin lymphoma (NHL), which encompasses a diverse group of blood cancers that originate in the lymphatic system.

Clinical Description of Follicular Lymphoma

Overview

Follicular lymphoma is a type of indolent (slow-growing) non-Hodgkin lymphoma that arises from B-lymphocytes, a type of white blood cell. It is characterized by the proliferation of neoplastic follicle center (germinal center) cells. The disease is typically classified into different grades based on histological features, with grade III indicating a more aggressive form of the disease compared to grades I and II.

Grade III Characteristics

  • Histological Features: Follicular lymphoma grade III is further divided into two subtypes: grade IIIA and grade IIIB. Grade IIIA retains some follicular architecture, while grade IIIB is characterized by a diffuse pattern of growth, indicating a higher proliferation rate of the lymphoma cells.
  • Clinical Behavior: Grade III follicular lymphoma tends to have a more aggressive clinical course compared to lower-grade forms. Patients may experience more rapid disease progression and may require more intensive treatment strategies.

Symptoms

Patients with follicular lymphoma may present with:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the head, face, and neck regions, which is common in this diagnosis.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which may indicate more advanced disease.
- Fatigue: Generalized fatigue is a common complaint among patients.

Diagnosis

Diagnosis typically involves:
- Imaging Studies: CT scans or PET scans to assess the extent of lymphadenopathy and any involvement of other organs.
- Biopsy: A lymph node biopsy is essential for histological examination to confirm the diagnosis and grade of lymphoma.
- Immunophenotyping: Flow cytometry may be used to identify specific cell markers that characterize the lymphoma.

Treatment Options

Treatment for follicular lymphoma grade III may include:
- Chemotherapy: Often combined with immunotherapy (e.g., rituximab) to enhance treatment efficacy.
- Radiation Therapy: May be used in localized disease or as palliative care.
- Targeted Therapy: Newer agents targeting specific pathways involved in lymphoma cell survival may be considered.

Prognosis

The prognosis for patients with follicular lymphoma grade III can vary significantly based on several factors, including the extent of disease at diagnosis, response to initial treatment, and overall patient health. Generally, the prognosis is less favorable than for lower-grade follicular lymphomas, necessitating close monitoring and potentially more aggressive treatment approaches.

Conclusion

ICD-10 code C82.21 captures a specific and clinically significant diagnosis of follicular lymphoma grade III affecting the lymph nodes of the head, face, and neck. Understanding the characteristics, symptoms, diagnostic methods, and treatment options is crucial for effective management of this condition. As research continues, new therapies and treatment strategies are being developed to improve outcomes for patients diagnosed with this aggressive form of lymphoma.

Approximate Synonyms

ICD-10 code C82.21 refers specifically to "Follicular lymphoma grade III, unspecified, lymph nodes of head, face, and neck." This classification falls under the broader category of non-Hodgkin lymphoma, particularly follicular lymphoma, which is a type of cancer that originates in the lymphatic system.

  1. Follicular Lymphoma: This is the general term for a type of non-Hodgkin lymphoma that arises from follicle center (germinal center) B-cells. It is characterized by a slow growth rate and can transform into a more aggressive form.

  2. Grade III Follicular Lymphoma: This term specifies the aggressiveness of the lymphoma. Grade III indicates a higher proliferation of cancer cells compared to lower grades, which can affect treatment decisions and prognosis.

  3. Non-Hodgkin Lymphoma (NHL): Follicular lymphoma is a subtype of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers that include various types of lymphomas.

  4. Lymphoma of the Head and Neck: This term refers to lymphomas that specifically affect the lymph nodes in the head and neck region, which includes follicular lymphoma as a subtype.

  5. B-cell Lymphoma: Since follicular lymphoma originates from B-cells, it can also be referred to as a B-cell lymphoma, highlighting its cellular origin.

  6. Lymphadenopathy: This term describes the enlargement of lymph nodes, which is a common symptom in patients with follicular lymphoma, particularly in the head, face, and neck regions.

  7. Lymphoma, Follicular, Grade III: This is a more straightforward way to refer to the specific grade of follicular lymphoma without the additional detail of the location.

  • ICD-10 Code C82.20: This code refers to "Follicular lymphoma grade III, unspecified, lymph nodes of unspecified site," which is a broader classification without specifying the head, face, and neck.

  • ICD-10 Code C82.22: This code specifies "Follicular lymphoma grade III, unspecified, lymph nodes of axilla and upper limb," indicating a different anatomical location.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C82.21 is crucial for accurate diagnosis, treatment planning, and communication among healthcare professionals. These terms help in categorizing the disease and can influence clinical decisions and patient management strategies. If you need further details or specific information regarding treatment options or prognosis, feel free to ask!

Diagnostic Criteria

Follicular lymphoma, particularly grade III, is a subtype of non-Hodgkin lymphoma characterized by specific diagnostic criteria. The ICD-10 code C82.21 refers to follicular lymphoma grade III that is unspecified and located in the lymph nodes of the head, face, and neck. Here’s a detailed overview of the criteria used for diagnosis:

Diagnostic Criteria for Follicular Lymphoma Grade III

1. Histological Examination

  • Tissue Biopsy: A definitive diagnosis of follicular lymphoma requires a biopsy of the affected lymph node. The histological examination is crucial for identifying the characteristic follicular architecture.
  • Grading: Follicular lymphoma is graded based on the number of large cells (centroblasts) present. Grade III is further divided into:
    • Grade IIIA: Predominantly follicular architecture with a higher number of centroblasts.
    • Grade IIIB: Diffuse pattern with a predominance of centroblasts, often lacking the typical follicular structure.

2. Immunophenotyping

  • Immunohistochemistry: This technique is used to identify specific markers on the lymphoma cells. Follicular lymphoma typically expresses CD10, BCL2, and BCL6, which are essential for confirming the diagnosis.
  • Flow Cytometry: This may also be employed to analyze the surface markers of the lymphocytes, further supporting the diagnosis.

3. Cytogenetic Analysis

  • Chromosomal Abnormalities: The presence of the t(14;18) translocation, which involves the BCL2 gene, is a hallmark of follicular lymphoma. Cytogenetic studies can help identify this abnormality, aiding in diagnosis and prognosis.

4. Clinical Presentation

  • Symptoms: Patients may present with painless lymphadenopathy, particularly in the head, face, and neck regions. Systemic symptoms such as fever, night sweats, and weight loss may also be present but are less common in early-stage disease.
  • Physical Examination: A thorough examination of lymph nodes in the head and neck is essential, as this is the primary site of involvement for the specified ICD-10 code.

5. Imaging Studies

  • CT or PET Scans: Imaging studies may be utilized to assess the extent of lymphadenopathy and to rule out other conditions. These scans help in staging the disease and determining the involvement of other lymph node regions or organs.

6. Staging

  • Ann Arbor Staging System: This system is used to classify the extent of lymphoma. It considers the number of lymph node regions involved and whether the disease has spread to other organs.

Conclusion

The diagnosis of follicular lymphoma grade III, particularly in the lymph nodes of the head, face, and neck, involves a combination of histological, immunophenotypic, and cytogenetic evaluations, alongside clinical and imaging assessments. Accurate diagnosis is crucial for determining the appropriate treatment strategy and prognosis for the patient. If you have further questions or need more specific information regarding treatment options or management strategies, feel free to ask!

Related Information

Clinical Information

  • Painless swelling of lymph nodes
  • Firm and rubbery lymph node texture
  • Unexplained fever
  • Night sweats
  • Significant weight loss (10%+ body weight)
  • General sense of fatigue or malaise
  • Itching without a rash
  • Abdominal pain or discomfort due to lymphadenopathy
  • Typically occurs in adults over 60 years old
  • Male predominance, with men more frequently diagnosed than women
  • History of autoimmune diseases increases risk
  • Family history of lymphoproliferative disorders may be noted

Treatment Guidelines

  • Chemotherapy remains cornerstone of treatment
  • R-CHOP is first-line treatment for advanced-stage
  • Rituximab targets CD20 on B-cells
  • PI3K Inhibitors used in relapsed cases
  • Radiation Therapy effective for localized disease
  • Stem Cell Transplantation considered for relapsed/refractory
  • Clinical Trials offer access to novel therapies

Description

  • Follicular lymphoma type
  • Slow-growing cancer of B-lymphocytes
  • Proliferation of neoplastic follicle center cells
  • Histological features define grade IIIA and IIIB
  • Grade III has more aggressive clinical course
  • Lymphadenopathy is common symptom
  • B symptoms include fever, night sweats, weight loss

Approximate Synonyms

  • Follicular Lymphoma
  • Grade III Follicular Lymphoma
  • Non-Hodgkin Lymphoma (NHL)
  • Lymphoma of the Head and Neck
  • B-cell Lymphoma
  • Lymphadenopathy
  • Lymphoma, Follicular, Grade III

Diagnostic Criteria

  • Tissue biopsy required for definitive diagnosis
  • Histological examination identifies follicular architecture
  • Centroblasts counted to determine grade
  • Immunohistochemistry confirms CD10, BCL2, and BCL6 expression
  • Flow cytometry analyzes surface markers
  • Chromosomal abnormalities identified via cytogenetic analysis
  • t(14;18) translocation characteristic of follicular lymphoma
  • Painless lymphadenopathy common in head, face, and neck
  • Systemic symptoms may be present but less common
  • Imaging studies assess lymphadenopathy extent
  • CT or PET scans rule out other conditions
  • Ann Arbor staging system classifies disease extent

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