ICD-10: C84.79

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma characterized by the proliferation of large, atypical lymphoid cells. The ICD-10 code C84.79 specifically refers to ALCL that is ALK-negative and occurs in extranodal and solid organ sites. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Anaplastic Large Cell Lymphoma (ALK-Negative)

Definition and Classification

Anaplastic large cell lymphoma is classified under the category of mature T/NK-cell lymphomas. The ALK-negative variant is distinguished by the absence of the anaplastic lymphoma kinase (ALK) gene rearrangement, which is typically present in the ALK-positive subtype. This absence has implications for prognosis and treatment strategies[1][2].

Epidemiology

ALCL is relatively rare, accounting for approximately 2-8% of all non-Hodgkin lymphomas. The ALK-negative variant tends to occur more frequently in adults compared to children, with a median age of diagnosis in the 40s. It is more common in males than females[1].

Clinical Presentation

Patients with ALK-negative ALCL may present with a variety of symptoms, which can include:
- Lymphadenopathy: Swelling of lymph nodes, which may be localized or generalized.
- Extranodal Involvement: This subtype often presents with involvement of extranodal sites, including the skin, lungs, liver, and gastrointestinal tract. Symptoms may vary depending on the organ affected.
- B Symptoms: Fever, night sweats, and weight loss may be present, indicating a more systemic involvement of the disease[1][2].

Diagnosis

Diagnosis of ALK-negative ALCL typically involves:
- Histopathological Examination: Biopsy of affected tissue is essential for diagnosis, revealing characteristic large pleomorphic cells.
- Immunophenotyping: Immunohistochemical staining is used to identify specific markers, such as CD30 positivity, which is a hallmark of ALCL.
- Molecular Studies: While ALK rearrangement is assessed, the absence of this rearrangement confirms the ALK-negative subtype[1][2].

Treatment

The treatment approach for ALK-negative ALCL may include:
- Chemotherapy: Multi-agent chemotherapy regimens are commonly employed, often including CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or other combinations.
- Targeted Therapy: Emerging therapies may target specific pathways involved in the disease, although options are more limited compared to ALK-positive ALCL.
- Stem Cell Transplantation: In cases of relapsed or refractory disease, autologous stem cell transplantation may be considered[1][2].

Prognosis

The prognosis for patients with ALK-negative ALCL can vary significantly based on several factors, including the stage of the disease at diagnosis, the presence of B symptoms, and the response to initial treatment. Generally, the prognosis is considered less favorable compared to the ALK-positive variant, with a higher likelihood of relapse[1][2].

Conclusion

ICD-10 code C84.79 encapsulates the clinical complexities of anaplastic large cell lymphoma, ALK-negative, particularly when it manifests in extranodal and solid organ sites. Understanding the clinical presentation, diagnostic criteria, treatment options, and prognosis is crucial for effective management of this lymphoma subtype. Ongoing research continues to explore more targeted therapies and improve outcomes for affected patients.

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

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative variant coded as ICD-10 C84.79, is a type of non-Hodgkin lymphoma characterized by specific clinical presentations, signs, symptoms, and patient demographics. Understanding these aspects is crucial for accurate diagnosis and management.

Clinical Presentation

Overview of ALCL

Anaplastic large cell lymphoma is a subtype of mature T-cell lymphoma, which can manifest in various forms, including systemic and localized presentations. The ALK-negative variant is particularly noted for its aggressive nature and association with extranodal involvement, affecting solid organ sites such as the skin, lungs, and gastrointestinal tract[1][2].

Signs and Symptoms

Patients with ALK-negative ALCL may present with a range of symptoms, which can vary based on the site of involvement:

• Lymphadenopathy: Swelling of lymph nodes is common, often presenting as painless masses in the neck, axilla, or groin[1].
• Extranodal Involvement: Symptoms may arise from affected organs, including:
• Skin: Lesions may appear as nodules or plaques, often with ulceration[2].
• Lungs: Patients may experience cough, dyspnea, or chest pain if pulmonary involvement occurs[1].
• Gastrointestinal Tract: Symptoms can include abdominal pain, nausea, vomiting, or gastrointestinal bleeding[2].
• B Symptoms: Fever, night sweats, and weight loss are common systemic symptoms associated with lymphoma, indicating a more aggressive disease course[1][2].
• Fatigue: Generalized fatigue is frequently reported, often due to the systemic nature of the disease and its metabolic demands[1].

Patient Characteristics

Demographics

• Age: ALK-negative ALCL typically affects adults, with a peak incidence in the third to fifth decades of life. However, it can occur in children and older adults as well[1][2].
• Gender: There is a slight male predominance in cases of ALK-negative ALCL, although it can affect individuals of any gender[2].
• Ethnicity: Some studies suggest variations in incidence based on ethnicity, with higher rates observed in certain populations, although more research is needed to clarify these trends[1].

Risk Factors

• Immunosuppression: Patients with compromised immune systems, such as those with HIV/AIDS or those undergoing immunosuppressive therapy, are at increased risk for developing ALCL[2].
• Previous Lymphoproliferative Disorders: A history of other lymphomas or hematological malignancies may predispose individuals to ALCL[1].
• Environmental Exposures: Certain environmental factors, including exposure to chemicals or radiation, have been implicated in the development of lymphomas, including ALCL[2].

Conclusion

Anaplastic large cell lymphoma, ALK-negative, presents with a diverse array of clinical features, primarily characterized by lymphadenopathy and potential extranodal involvement. Understanding the signs, symptoms, and patient demographics is essential for healthcare providers to facilitate timely diagnosis and appropriate management. Given the aggressive nature of this lymphoma subtype, early recognition and intervention are critical to improving patient outcomes. Further research into the epidemiology and pathophysiology of ALCL will enhance our understanding and treatment strategies for this complex disease.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative variant, is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The ICD-10 code C84.79 specifically refers to ALCL that occurs in extranodal and solid organ sites. Here are some alternative names and related terms associated with this condition:

Alternative Names for Anaplastic Large Cell Lymphoma, ALK-Negative

1. Anaplastic Large Cell Lymphoma (ALCL): This is the broader term that encompasses both ALK-positive and ALK-negative variants.
2. ALK-Negative Anaplastic Large Cell Lymphoma: This specifies the subtype of ALCL that lacks the anaplastic lymphoma kinase (ALK) gene rearrangement.
3. Systemic ALCL: Refers to cases where the lymphoma is disseminated throughout the body, including extranodal sites.
4. Extranodal ALCL: Highlights the occurrence of lymphoma in sites outside of lymph nodes, such as skin, lungs, or other solid organs.
5. Peripheral T-cell Lymphoma, Not Otherwise Specified (PTCL-NOS): ALCL is classified under this broader category of T-cell lymphomas when it does not fit other specific subtypes.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): A general term for a diverse group of blood cancers that includes ALCL.
2. Lymphoma: A type of cancer that originates in the lymphatic system, which includes various subtypes, including ALCL.
3. Extranodal Lymphoma: Refers to lymphomas that arise in tissues outside of the lymph nodes, which is relevant for ALCL cases coded under C84.79.
4. Solid Organ Lymphoma: This term can be used to describe lymphomas that manifest in solid organs, which is pertinent to the ALK-negative variant.
5. T-cell Lymphoma: A category of lymphomas that includes ALCL, as it arises from T-lymphocytes.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C84.79 is crucial for accurate diagnosis, treatment planning, and coding in medical records. This knowledge aids healthcare professionals in communicating effectively about the condition and ensures that patients receive appropriate care tailored to their specific lymphoma subtype.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The ICD-10 code C84.79 specifically refers to ALCL that occurs in extranodal and solid organ sites. The diagnostic criteria for this condition involve a combination of clinical, histopathological, and immunophenotypic evaluations.

Diagnostic Criteria for ALK-Negative Anaplastic Large Cell Lymphoma

1. Clinical Presentation

• Symptoms: Patients may present with lymphadenopathy, fever, weight loss, night sweats, and fatigue. Extranodal involvement can lead to symptoms specific to the affected organ, such as respiratory distress if the lungs are involved or abdominal pain if the liver or spleen is affected[1].
• Physical Examination: A thorough physical examination is essential to identify lymphadenopathy and any signs of organ involvement.

2. Histopathological Examination

• Biopsy: A tissue biopsy from an affected lymph node or extranodal site is crucial. The histological examination typically reveals a polymorphous infiltrate of large atypical lymphoid cells, often with a characteristic "hallmark" cell appearance, which is a large cell with abundant cytoplasm and a horseshoe-shaped nucleus[2].
• Immunohistochemistry: The diagnosis is confirmed through immunohistochemical staining. ALCL cells are usually positive for CD30 and may express other markers such as CD4, CD43, and EMA (epithelial membrane antigen). Importantly, ALK (anaplastic lymphoma kinase) expression is absent in ALK-negative cases[3].

3. Cytogenetic and Molecular Studies

• Genetic Testing: While ALK-positive ALCL is associated with specific chromosomal translocations (most commonly t(2;5)(p23;q35)), ALK-negative cases do not have these alterations. Genetic studies may be performed to rule out other lymphomas and confirm the diagnosis[4].
• Next-Generation Sequencing: This may be utilized to identify mutations or other genetic abnormalities that could support the diagnosis of ALCL.

4. Exclusion of Other Conditions

• Differential Diagnosis: It is essential to differentiate ALCL from other types of lymphomas and malignancies, such as peripheral T-cell lymphoma (PTCL) and other non-Hodgkin lymphomas. This may involve additional immunophenotyping and molecular studies[5].
• Clinical Correlation: The clinical context, including the patient's history and presentation, is vital in confirming the diagnosis.

5. Staging and Assessment

• Imaging Studies: Imaging techniques such as CT scans, PET scans, or MRI may be employed to assess the extent of disease, particularly in extranodal sites[6].
• Staging: The Ann Arbor staging system is commonly used to determine the stage of lymphoma, which is crucial for treatment planning.

Conclusion

The diagnosis of ALK-negative anaplastic large cell lymphoma, particularly in extranodal and solid organ sites, relies on a comprehensive approach that includes clinical evaluation, histopathological analysis, immunophenotyping, and exclusion of other conditions. Accurate diagnosis is essential for effective treatment and management of this aggressive lymphoma subtype. If you have further questions or need more specific information, feel free to ask!

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a rare and aggressive form of non-Hodgkin lymphoma that can manifest in extranodal and solid organ sites. The management of this condition involves a combination of chemotherapy, targeted therapies, and, in some cases, stem cell transplantation. Below is a detailed overview of the standard treatment approaches for ICD10 code C84.79.

Overview of Anaplastic Large Cell Lymphoma (ALK-Negative)

Anaplastic large cell lymphoma is characterized by the presence of large, atypical lymphoid cells and is classified into two main subtypes based on the presence of the anaplastic lymphoma kinase (ALK) gene rearrangement. The ALK-negative variant is often associated with a poorer prognosis compared to its ALK-positive counterpart. This subtype can occur in various extranodal sites, including the skin, lungs, and gastrointestinal tract, making its treatment more complex due to the involvement of solid organs.

Standard Treatment Approaches

1. Chemotherapy

The cornerstone of treatment for ALK-negative ALCL typically involves chemotherapy. The most commonly used regimens include:

• CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. CHOP is a standard treatment for many types of non-Hodgkin lymphoma and is often the first-line therapy for ALCL[1].

• EPOCH Regimen: Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin (EPOCH) is another regimen that may be used, particularly in cases with more aggressive disease or those that do not respond to CHOP[2].

• Dose-Adjusted EPOCH: This variant of EPOCH involves dose adjustments based on the patient's response and tolerance, aiming to enhance efficacy while minimizing toxicity[3].

2. Targeted Therapy

Targeted therapies have emerged as a significant advancement in the treatment of various lymphomas, including ALCL. While ALK-positive ALCL has specific targeted options, ALK-negative ALCL may benefit from:

• Brentuximab Vedotin (Adcetris): This is an antibody-drug conjugate that targets CD30, a marker commonly expressed in ALCL. Brentuximab is often used in relapsed or refractory cases and can be combined with chemotherapy for improved outcomes[4].

3. Stem Cell Transplantation

For patients with high-risk or relapsed ALK-negative ALCL, autologous stem cell transplantation (ASCT) may be considered. This approach is typically reserved for those who have achieved a complete or partial response to initial chemotherapy. ASCT can provide a curative option for select patients, particularly those with aggressive disease or those who have not responded adequately to standard therapies[5].

4. Radiation Therapy

Radiation therapy may be utilized in specific scenarios, particularly for localized disease or as a consolidation treatment following chemotherapy. It is generally not the primary treatment modality but can be effective in managing localized extranodal sites[6].

Conclusion

The treatment of anaplastic large cell lymphoma, ALK-negative, particularly in extranodal and solid organ sites, requires a multidisciplinary approach tailored to the individual patient's disease characteristics and overall health. Standard treatment typically involves chemotherapy, with options for targeted therapy and stem cell transplantation for high-risk cases. Ongoing research and clinical trials continue to explore new therapeutic strategies to improve outcomes for patients with this challenging lymphoma subtype.

For patients diagnosed with C84.79, it is crucial to work closely with a hematologist/oncologist to determine the most appropriate treatment plan based on the latest clinical guidelines and individual patient factors.