ICD-10: C83.398
Diffuse large B-cell lymphoma of other extranodal and solid organ sites
Additional Information
Description
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 C83.398 specifically refers to DLBCL that occurs in other extranodal and solid organ sites, which distinguishes it from other forms of DLBCL that may be more localized or involve lymph nodes.
Clinical Description
Definition and Characteristics
Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma, accounting for approximately 30% of all cases. It is known for its aggressive nature and can arise in various locations throughout the body, including lymph nodes, extranodal sites, and solid organs. The term "extranodal" refers to lymphomas that occur outside of the lymphatic system, which can include organs such as the spleen, liver, and gastrointestinal tract.
Presentation
Patients with DLBCL may present with a variety of symptoms, which can include:
- 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. For instance, if the gastrointestinal tract is involved, patients may experience abdominal pain or changes in bowel habits.
Diagnosis
Diagnosis of DLBCL typically involves:
- Histopathological examination: A biopsy of the affected tissue is essential for confirming the diagnosis. The tissue is examined microscopically to identify the presence of large B-cells.
- Immunophenotyping: This process helps to determine the specific type of lymphoma by analyzing the proteins expressed on the surface of the cells.
- Imaging studies: CT scans, PET scans, or MRIs may be used to assess the extent of the disease and to identify extranodal involvement.
Staging
The staging of DLBCL is crucial for determining the appropriate treatment plan. The Ann Arbor staging system is commonly used, which classifies the disease based on the number of affected lymph node regions and the presence of extranodal disease.
Treatment Options
Treatment for DLBCL typically involves a combination of therapies, including:
- Chemotherapy: The most common regimen is R-CHOP, which includes rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.
- Radiation therapy: This may be used in localized cases or as a consolidation treatment after chemotherapy.
- Targeted therapies: Newer treatments, such as CAR T-cell therapy, may be considered for relapsed or refractory cases.
Prognosis
The prognosis for patients with DLBCL can vary widely based on several factors, including the stage of the disease at diagnosis, the patient's age, overall health, and response to treatment. The International Prognostic Index (IPI) is often used to predict outcomes based on clinical features.
Conclusion
ICD-10 code C83.398 encompasses a critical aspect of diffuse large B-cell lymphoma, specifically focusing on cases that arise in extranodal and solid organ sites. Understanding the clinical presentation, diagnostic criteria, treatment options, and prognosis is essential for healthcare providers managing patients with this aggressive lymphoma subtype. Early diagnosis and appropriate treatment are key to improving outcomes for affected individuals.
Clinical Information
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 C83.398 specifically refers to DLBCL occurring in extranodal and solid organ sites other than the lymph nodes. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for accurate diagnosis and treatment.
Clinical Presentation
Signs and Symptoms
Patients with DLBCL of extranodal sites may present with a variety of symptoms, which can vary depending on the location of the lymphoma. Common signs and symptoms include:
- Lymphadenopathy: While DLBCL primarily affects lymph nodes, extranodal involvement may lead to swelling in areas where the lymphoma is present, such as the abdomen or mediastinum.
- B Symptoms: These systemic symptoms include fever, night sweats, and unexplained weight loss, which are indicative of lymphoma and can occur in DLBCL patients regardless of the site of involvement[1].
- Localized Symptoms: Depending on the extranodal site affected, patients may experience:
- Abdominal Pain: If the lymphoma involves the gastrointestinal tract or solid organs like the liver or spleen.
- Respiratory Symptoms: Such as cough or dyspnea if the lymphoma is located in the thoracic cavity.
- Neurological Symptoms: Including headaches, seizures, or changes in mental status if the central nervous system is involved[2].
Patient Characteristics
Certain demographic and clinical characteristics are often associated with DLBCL:
- Age: DLBCL can occur at any age but is more common in older adults, typically those over 60 years old[3].
- Gender: There is a slight male predominance in DLBCL cases, with men being more frequently diagnosed than women[4].
- Comorbidities: Patients may have underlying conditions such as autoimmune diseases or infections (e.g., HIV) that can predispose them to lymphoproliferative disorders[5].
- Immunosuppression: Individuals with compromised immune systems, whether due to medical conditions or treatments (like chemotherapy), are at higher risk for developing DLBCL[6].
Diagnosis and Evaluation
Diagnosis of DLBCL typically involves a combination of clinical evaluation, imaging studies, and histopathological examination:
- Imaging Studies: CT scans, PET scans, or MRI may be utilized to assess the extent of disease and identify extranodal involvement[7].
- Biopsy: A tissue biopsy is essential for confirming the diagnosis, with histological examination revealing the characteristic large B-cells[8].
- Laboratory Tests: Blood tests may be performed to evaluate overall health and organ function, as well as to check for markers associated with lymphoma.
Conclusion
Diffuse large B-cell lymphoma of extranodal and solid organ sites (ICD-10 code C83.398) presents with a range of symptoms that can vary significantly based on the affected area. Recognizing the clinical signs, understanding patient demographics, and employing appropriate diagnostic methods are essential for effective management of this aggressive lymphoma. Early diagnosis and treatment are critical to improving patient outcomes, emphasizing the importance of awareness among healthcare providers regarding the diverse presentations of DLBCL.
For further information on treatment options and management strategies, healthcare professionals should refer to the latest clinical guidelines and research studies related to DLBCL.
Approximate Synonyms
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 C83.398 specifically refers to DLBCL that occurs in other extranodal and solid organ sites. Understanding alternative names and related terms can enhance clarity in medical documentation and communication.
Alternative Names for DLBCL
- Large Cell Lymphoma: This term is often used interchangeably with DLBCL, emphasizing the size of the malignant cells.
- B-cell Lymphoma: A broader category that includes various types of lymphomas originating from B-cells, of which DLBCL is a prominent subtype.
- Non-Hodgkin Lymphoma (NHL): DLBCL falls under the umbrella of non-Hodgkin lymphomas, which are diverse and include many different types of lymphoid malignancies.
Related Terms
- Extranodal Lymphoma: This term refers to lymphomas that occur outside of the lymph nodes, which is relevant for DLBCL cases coded under C83.398.
- Solid Organ Lymphoma: This term describes lymphomas that manifest in solid organs, such as the liver, spleen, or kidneys, which can be sites for DLBCL.
- Aggressive B-cell Lymphoma: This term highlights the aggressive nature of DLBCL, which typically requires prompt treatment due to its rapid progression.
- Primary Extranodal Lymphoma: This term is used when the lymphoma originates in an extranodal site rather than spreading from lymph nodes.
Clinical Context
In clinical practice, the use of these alternative names and related terms can help in accurately describing the condition, facilitating better communication among healthcare providers, and ensuring appropriate coding for billing and insurance purposes. The specificity of the ICD-10 code C83.398 is crucial for identifying the exact nature of the lymphoma, which can influence treatment decisions and patient management strategies.
In summary, understanding the alternative names and related terms for ICD-10 code C83.398 can enhance clarity in medical discussions and documentation, ensuring that healthcare professionals are aligned in their understanding of this complex condition.
Treatment Guidelines
Diffuse large B-cell lymphoma (DLBCL) is a common and aggressive form of non-Hodgkin lymphoma (NHL) characterized by the rapid proliferation of B-cells. The ICD-10 code C83.398 specifically refers to DLBCL that occurs in extranodal and solid organ sites, which can complicate treatment approaches due to the involvement of various anatomical locations.
Standard Treatment Approaches for DLBCL
1. Chemotherapy
The cornerstone of treatment for DLBCL is chemotherapy, typically using a regimen known as R-CHOP, which includes:
- Rituximab: A monoclonal antibody targeting CD20 on B-cells.
- Cyclophosphamide: An alkylating agent that interferes with DNA replication.
- Doxorubicin (Adriamycin): An anthracycline that disrupts DNA and RNA synthesis.
- Vincristine: A vinca alkaloid that inhibits cell division.
- Prednisone: A corticosteroid that helps reduce inflammation and suppress the immune response.
This combination is administered in cycles, usually every 21 days, and is effective in treating DLBCL, including cases with extranodal involvement[1].
2. Radiation Therapy
Radiation therapy may be employed in conjunction with chemotherapy, particularly for localized disease or when there is a significant mass effect from the lymphoma. It can be used as:
- Consolidation therapy: Following chemotherapy to eliminate residual disease.
- Palliative treatment: To relieve symptoms caused by tumor mass in solid organs[1].
3. Stem Cell Transplantation
For patients with high-risk or relapsed DLBCL, autologous stem cell transplantation (ASCT) may be considered. This involves:
- High-dose chemotherapy: To eradicate the lymphoma.
- Stem cell rescue: Using the patient’s own stem cells previously collected and stored.
This approach is typically reserved for younger patients or those with good performance status[1].
4. Targeted Therapies and Immunotherapy
Recent advancements have introduced targeted therapies and immunotherapies for DLBCL:
- CAR T-cell therapy: This involves modifying a patient’s T-cells to better recognize and attack lymphoma cells. It is particularly effective in relapsed or refractory cases.
- Novel agents: Such as ibrutinib, which targets specific pathways involved in B-cell proliferation, may be used in certain cases, especially when traditional therapies fail[1][2].
5. Clinical Trials
Participation in clinical trials may be an option for patients, providing access to cutting-edge therapies and novel combinations that are not yet widely available. These trials often explore new drugs, combinations, or treatment strategies aimed at improving outcomes for DLBCL patients[2].
Conclusion
The treatment of diffuse large B-cell lymphoma, particularly in extranodal and solid organ sites, involves a multifaceted approach that includes chemotherapy, radiation therapy, and potentially stem cell transplantation or targeted therapies. The choice of treatment is influenced by various factors, including the patient's overall health, the extent of the disease, and specific characteristics of the lymphoma. Ongoing research and clinical trials continue to shape the landscape of DLBCL treatment, offering hope for improved outcomes in this aggressive disease. For patients diagnosed with C83.398, a personalized treatment plan developed by a multidisciplinary team is essential for optimal management.
Diagnostic Criteria
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 C83.398 specifically refers to DLBCL that occurs in extranodal and solid organ sites other than those specifically classified elsewhere. Diagnosing this condition involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria used for diagnosis:
Clinical Evaluation
-
Symptoms: Patients may present with various symptoms, including:
- Painless lymphadenopathy (swelling of lymph nodes)
- Fever
- Night sweats
- Unexplained weight loss
- Fatigue -
Physical Examination: A thorough physical examination is essential to identify any enlarged lymph nodes or organomegaly (enlargement of organs) that may indicate lymphoma.
Imaging Studies
-
CT Scans: Computed tomography (CT) scans of the chest, abdomen, and pelvis are commonly used to identify the extent of disease and to locate extranodal involvement.
-
PET Scans: Positron emission tomography (PET) scans can help assess metabolic activity in suspected lymphoma sites, providing information on the aggressiveness of the disease.
Histopathological Examination
-
Biopsy: A definitive diagnosis of DLBCL requires a biopsy of the affected tissue. This can be done through:
- Excisional biopsy of lymph nodes
- Core needle biopsy of solid organ involvement
- Fine-needle aspiration (FNA) in certain cases -
Immunophenotyping: The biopsy sample is analyzed using immunohistochemistry to identify specific markers characteristic of DLBCL, such as:
- CD19
- CD20
- CD22
- CD79a -
Genetic Studies: Additional tests, such as fluorescence in situ hybridization (FISH), may be performed to detect chromosomal abnormalities associated with DLBCL, including the presence of the MYC gene rearrangement.
Exclusion of Other Conditions
-
Differential Diagnosis: It is crucial to rule out other types of lymphomas and malignancies that may present similarly. This includes:
- Other subtypes of non-Hodgkin lymphoma
- Hodgkin lymphoma
- Solid tumors that may mimic lymphoma -
Clinical History: A detailed patient history, including previous malignancies, autoimmune diseases, and family history of lymphoproliferative disorders, can provide context for the diagnosis.
Conclusion
The diagnosis of diffuse large B-cell lymphoma of other extranodal and solid organ sites (ICD-10 code C83.398) is a multifaceted process that relies on clinical assessment, imaging studies, and histopathological confirmation. Accurate diagnosis is essential for determining the appropriate treatment strategy and improving patient outcomes. If you have further questions or need more specific information, feel free to ask!
Related Information
Description
- Aggressive type of non-Hodgkin lymphoma
- Rapid proliferation of large B-cells
- Diffuse involvement of body organs
- Variable presentation with symptoms like fever, weight loss
- Lymphadenopathy and extranodal involvement common
- Histopathological examination confirms diagnosis
- Immunophenotyping identifies specific lymphoma type
- ICD-10 code C83.398 for extranodal sites
Clinical Information
- Lymphadenopathy can occur in extranodal sites
- B symptoms include fever and night sweats
- Abdominal pain can be a localized symptom
- Respiratory symptoms vary with lymphoma site
- Neurological symptoms may include headaches
- DLBCL is more common in older adults
- Male patients have a higher incidence rate
- Comorbidities increase risk of DLBCL development
Approximate Synonyms
- Large Cell Lymphoma
- B-cell Lymphoma
- Non-Hodgkin Lymphoma (NHL)
- Extranodal Lymphoma
- Solid Organ Lymphoma
- Aggressive B-cell Lymphoma
- Primary Extranodal Lymphoma
Treatment Guidelines
- Chemotherapy R-CHOP regimen
- Rituximab monoclonal antibody
- Cyclophosphamide DNA replication
- Doxorubicin anthracycline disruption
- Vincristine cell division inhibition
- Prednisone inflammation suppression
- Radiation therapy for localized disease
- Stem cell transplantation high-dose chemotherapy
- CAR T-cell immunotherapy relapsed cases
- Novel agents ibrutinib targeted pathways
Diagnostic Criteria
Related Diseases
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