ICD-10: C83.39

Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin lymphoma characterized by the rapid proliferation of large B-cells. The ICD-10 code C83.39 specifically refers to DLBCL that occurs in extranodal sites and solid organs, which can present with a variety of clinical features. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for diagnosis and management.

Clinical Presentation

Extranodal Involvement

DLBCL can manifest in various extranodal sites, including:
- Gastrointestinal Tract: Often presents with abdominal pain, weight loss, and gastrointestinal bleeding.
- Central Nervous System (CNS): Symptoms may include headaches, neurological deficits, or altered mental status.
- Skin: May appear as nodules or plaques, often with associated itching.
- Lymphoid Tissue: Involvement of the tonsils or other lymphoid tissues can lead to swelling and discomfort.

Solid Organ Involvement

When DLBCL affects solid organs, it can lead to:
- Liver: Symptoms may include hepatomegaly, jaundice, and abdominal discomfort.
- Spleen: Splenomegaly can occur, leading to early satiety and discomfort.
- Kidneys: Renal involvement may present with flank pain or hematuria.

Signs and Symptoms

Common Symptoms

Patients with DLBCL may experience a range of symptoms, including:
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement.
- Lymphadenopathy: Swelling of lymph nodes, which may be localized or generalized.
- Fatigue: A common complaint due to the systemic nature of the disease.
- Pain: Depending on the site of involvement, patients may experience localized pain.

Physical Examination Findings

During a physical examination, clinicians may note:
- Enlarged Lymph Nodes: Palpable lymphadenopathy in the neck, axilla, or groin.
- Organomegaly: Enlargement of the liver or spleen upon palpation.
- Skin Lesions: Any cutaneous manifestations should be documented.

Patient Characteristics

Demographics

• Age: DLBCL is more common in adults, particularly those aged 60 and older, although it can occur at any age.
• Gender: There is a slight male predominance in the incidence of DLBCL.

Risk Factors

Several risk factors have been associated with DLBCL, including:
- Immunosuppression: Patients with HIV/AIDS or those on immunosuppressive therapy are at increased risk.
- Autoimmune Diseases: Conditions such as rheumatoid arthritis or Sjögren's syndrome may predispose individuals to lymphoma.
- Family History: A family history of lymphoma or other hematologic malignancies can increase risk.

Comorbidities

Patients may present with various comorbid conditions that can complicate the management of DLBCL, such as:
- Cardiovascular Disease: Pre-existing heart conditions may affect treatment options.
- Diabetes: Can influence overall health and treatment response.

Conclusion

Diffuse large B-cell lymphoma, particularly in extranodal and solid organ sites, presents with a diverse array of clinical features, symptoms, and patient characteristics. Recognizing these aspects is essential for timely diagnosis and effective management. Clinicians should maintain a high index of suspicion for DLBCL in patients presenting with unexplained systemic symptoms, lymphadenopathy, or organomegaly, especially in those with known risk factors. Early intervention can significantly impact patient outcomes and quality of life.

Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin lymphoma characterized by the rapid growth of large B-cells. The ICD-10 code C83.39 specifically refers to DLBCL that occurs in extranodal and solid organ sites. Understanding alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below are some of the key alternative names and related terms associated with ICD-10 code C83.39.

Alternative Names for Diffuse Large B-Cell Lymphoma

1. Extranodal Diffuse Large B-Cell Lymphoma: This term emphasizes the extranodal nature of the lymphoma, indicating that it arises outside of the lymph nodes.

2. DLBCL: An abbreviation commonly used in clinical settings to refer to diffuse large B-cell lymphoma.

3. High-Grade B-Cell Lymphoma: This term may be used interchangeably in some contexts, particularly when discussing aggressive forms of B-cell lymphomas.

4. B-Cell Lymphoma, Diffuse Large Cell Type: A more descriptive term that specifies the cellular characteristics of the lymphoma.

5. Non-Hodgkin Lymphoma, Diffuse Large B-Cell Type: This term places DLBCL within the broader category of non-Hodgkin lymphomas.

Related Terms

1. Extranodal Lymphoma: Refers to lymphomas that occur outside the lymphatic system, which includes DLBCL when it manifests in solid organs.

2. Solid Organ Lymphoma: This term can be used to describe lymphomas that specifically affect solid organs, such as the liver, spleen, or kidneys.

3. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes various types of lymphomas, including DLBCL.

4. Aggressive B-Cell Lymphoma: This term highlights the aggressive nature of DLBCL, which is known for its rapid progression.

5. Primary Extranodal Lymphoma: This term is used when the lymphoma originates in an extranodal site rather than spreading from lymph nodes.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.39 is crucial for healthcare professionals involved in the diagnosis, treatment, and documentation of diffuse large B-cell lymphoma. These terms not only facilitate clearer communication among medical staff but also enhance patient understanding of their condition. For accurate coding and billing, it is essential to use the correct terminology that aligns with clinical findings and treatment plans.

Diffuse large B-cell lymphoma (DLBCL), particularly when classified under ICD-10 code C83.39, refers to a type of non-Hodgkin lymphoma that primarily affects extranodal sites and solid organs. The diagnosis of DLBCL involves a combination of clinical evaluation, imaging studies, laboratory tests, and histopathological examination. Below are the key criteria and steps typically used in the diagnosis of this condition.

Clinical Presentation

Symptoms

Patients with DLBCL 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.
- Extranodal involvement: Symptoms may vary depending on the organ affected, such as abdominal pain for gastrointestinal involvement or respiratory symptoms for lung involvement.

Medical History

A thorough medical history is essential, including:
- Previous history of lymphoma or other malignancies.
- Family history of lymphoproliferative disorders.
- Exposure to risk factors such as certain infections (e.g., Epstein-Barr virus) or immunosuppressive conditions.

Diagnostic Imaging

Imaging Studies

Imaging techniques are crucial for assessing the extent of the disease:
- CT scans: Often used to identify lymphadenopathy and organ involvement.
- PET scans: Help in evaluating metabolic activity of the lymphoma and staging the disease.

Laboratory Tests

Blood Tests

Routine blood tests may include:
- Complete blood count (CBC): To check for anemia, thrombocytopenia, or leukopenia.
- Lactate dehydrogenase (LDH): Elevated levels can indicate a more aggressive disease.

Bone Marrow Biopsy

A bone marrow biopsy may be performed to assess for involvement of the bone marrow, which is common in aggressive lymphomas.

Histopathological Examination

Biopsy

The definitive diagnosis of DLBCL is made through a biopsy of the affected tissue:
- Excisional biopsy: Preferred for lymph nodes to obtain a representative sample.
- Core needle biopsy: May be used for solid organ involvement.

Immunophenotyping

The biopsy sample is analyzed using immunohistochemistry to determine the cell type:
- CD19, CD20, CD22, and CD79a: Typically positive in DLBCL.
- BCL2 and BCL6: May also be assessed to help classify the lymphoma subtype.

Genetic Studies

Molecular studies, including fluorescence in situ hybridization (FISH), may be performed to identify specific genetic abnormalities, such as the presence of the MYC gene rearrangement, which can influence prognosis and treatment decisions.

Staging

Ann Arbor Staging System

The Ann Arbor staging system is commonly used to classify the extent of lymphoma:
- Stage I: Involvement of a single lymph node region or extranodal site.
- Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm.
- Stage III: Involvement of lymph node regions on both sides of the diaphragm.
- Stage IV: Disseminated involvement of one or more extranodal organs.

Conclusion

The diagnosis of diffuse large B-cell lymphoma, particularly in extranodal and solid organ sites, is a multifaceted process that requires careful clinical assessment, imaging studies, laboratory tests, and histopathological evaluation. The integration of these diagnostic criteria is essential for accurate diagnosis and effective treatment planning. If you have further questions or need more specific information, feel free to ask!

Diffuse large B-cell lymphoma (DLBCL), particularly when it presents in extranodal and solid organ sites, is a complex condition that requires a multifaceted treatment approach. The ICD-10 code C83.39 specifically refers to this subtype of lymphoma, which is characterized by aggressive behavior and a tendency to involve various organs outside of the lymphatic system. Below is a detailed overview of the standard treatment approaches for this condition.

Overview of Diffuse Large B-Cell Lymphoma

DLBCL is the most common subtype of non-Hodgkin lymphoma (NHL) and is known for its rapid progression. It can arise in lymph nodes or extranodal sites, including the gastrointestinal tract, skin, and central nervous system. The treatment strategy often depends on several factors, including the stage of the disease, the patient's overall health, and specific characteristics of the lymphoma.

Standard Treatment Approaches

1. Chemotherapy

The cornerstone of treatment for DLBCL is chemotherapy, typically administered in combination regimens. The most common regimen is R-CHOP, which includes:

• Rituximab: A monoclonal antibody targeting CD20 on B-cells.
• Cyclophosphamide: An alkylating agent.
• Doxorubicin (Adriamycin): An anthracycline antibiotic.
• Vincristine: A vinca alkaloid.
• Prednisone: A corticosteroid.

This regimen is usually given in cycles over several months, with the total duration often spanning six to eight cycles depending on the response to treatment and the specific clinical scenario[1][2].

2. Radiation Therapy

For patients with localized disease or those who have residual disease after chemotherapy, radiation therapy may be employed. This is particularly relevant for extranodal sites where the lymphoma is confined to a specific area. Radiation can help to eliminate remaining cancer cells and reduce the risk of recurrence[3].

3. Stem Cell Transplantation

In cases where DLBCL is refractory to initial treatment or relapses after first-line therapy, high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be considered. This approach is typically reserved for younger patients or those with good performance status, as it involves significant risks and requires recovery time[4].

4. Targeted Therapies and Immunotherapy

Recent advancements in the treatment of DLBCL have introduced targeted therapies and immunotherapies. These include:

• CAR T-cell therapy: This involves modifying a patient’s T-cells to better recognize and attack lymphoma cells. It has shown promise in treating relapsed or refractory DLBCL.
• Novel agents: Drugs such as lenalidomide and ibrutinib are being explored in clinical trials for their efficacy in treating DLBCL, particularly in specific subtypes or in combination with other therapies[5].

5. Clinical Trials

Participation in clinical trials is encouraged for patients with DLBCL, especially those with advanced disease or those who have not responded to standard treatments. These trials may offer access to cutting-edge therapies and contribute to the advancement of treatment options for future patients[6].

Conclusion

The treatment of diffuse large B-cell lymphoma, particularly in extranodal and solid organ sites, is evolving with ongoing research and clinical trials. The standard approach typically involves a combination of chemotherapy, radiation therapy, and potentially stem cell transplantation or novel therapies for relapsed cases. As treatment strategies continue to improve, personalized approaches based on the specific characteristics of the lymphoma and the patient’s overall health will likely become more prevalent. For patients diagnosed with C83.39, it is crucial to work closely with a healthcare team to determine the most appropriate treatment plan tailored to their individual needs.

References

1. Article - Billing and Coding: Radiation Therapies (A59350).
2. NON-HODGKIN LYMPHOMA Includes Follicular.
3. Medical Drug Clinical Criteria.
4. HCT for Non-Hodgkin Lymphoma.
5. Medical Policy - MMM-pr.com.
6. CMS Manual System.

Clinical Description of ICD-10 Code C83.39

ICD-10 Code C83.39 refers to Diffuse Large B-Cell Lymphoma (DLBCL) that occurs in extranodal sites and solid organ locations. This classification is part of the broader category of non-Hodgkin lymphomas, which are malignancies originating from B-lymphocytes. DLBCL is characterized by the rapid proliferation of large B-cells and is one of the most common types of non-Hodgkin lymphoma.

Key Characteristics of Diffuse Large B-Cell Lymphoma

1. Pathophysiology:
   - DLBCL arises from B-cells, which are a type of white blood cell involved in the immune response. The "diffuse" designation indicates that the lymphoma cells are spread throughout the lymphatic tissue rather than forming a localized tumor.
   - The extranodal variant signifies that the lymphoma has spread beyond the lymph nodes to other organs, which can include the gastrointestinal tract, liver, spleen, and central nervous system.

2. Clinical Presentation:
   - Patients may present with symptoms such as lymphadenopathy (swollen lymph nodes), fever, night sweats, weight loss, and fatigue.
   - Extranodal involvement can lead to specific symptoms depending on the organ affected. For instance, gastrointestinal involvement may cause abdominal pain or obstruction, while central nervous system involvement can lead to neurological symptoms.

3. Diagnosis:
   - Diagnosis typically involves a combination of imaging studies (such as CT or PET scans), biopsy of affected tissues, and histopathological examination to confirm the presence of large B-cells.
   - Immunophenotyping and genetic studies may also be performed to determine the specific subtype of DLBCL and to guide treatment.

4. Staging:
   - The staging of DLBCL is crucial for treatment planning and prognosis. The Ann Arbor staging system is commonly used, which considers the number of affected lymph node regions and the presence of extranodal disease.

5. Treatment:
   - Treatment often involves a combination of chemotherapy and immunotherapy. The standard regimen for DLBCL is R-CHOP, which includes rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.
   - In cases of localized disease, radiation therapy may also be considered.

6. Prognosis:
   - The prognosis for patients with DLBCL can vary widely based on factors such as age, overall health, stage at diagnosis, and response to initial treatment. The International Prognostic Index (IPI) is often used to assess risk and guide treatment decisions.

Extranodal Involvement

Extranodal DLBCL can manifest in various solid organ sites, including:

• Gastrointestinal Tract: Often presents with abdominal pain, nausea, or gastrointestinal bleeding.
• Liver and Spleen: May lead to hepatomegaly or splenomegaly, causing discomfort or fullness.
• Central Nervous System: Symptoms can include headaches, seizures, or neurological deficits, indicating a more aggressive disease course.

Conclusion

ICD-10 code C83.39 encapsulates a critical aspect of diffuse large B-cell lymphoma, highlighting its extranodal and solid organ involvement. Understanding the clinical features, diagnostic approaches, and treatment options is essential for healthcare providers managing patients with this aggressive lymphoma subtype. Early diagnosis and appropriate treatment are vital for improving patient outcomes in DLBCL.