ICD-10: C84.68

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by the presence of anaplastic large cells and the expression of the anaplastic lymphoma kinase (ALK) protein. The ICD-10 code C84.68 specifically refers to ALCL affecting lymph nodes at multiple sites. Here are some alternative names and related terms associated with this condition:

Alternative Names

1. Anaplastic Large Cell Lymphoma, ALK-Positive: This is the most direct alternative name, emphasizing the ALK-positive status of the lymphoma.
2. ALK-Positive Anaplastic Lymphoma: A variation that highlights the ALK positivity.
3. Systemic Anaplastic Large Cell Lymphoma: This term may be used to describe cases where the lymphoma is widespread, affecting multiple lymph nodes.
4. Peripheral T-Cell Lymphoma, Not Otherwise Specified (NOS): ALCL is classified under this broader category in some contexts, although it is a distinct entity.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): ALCL is a subtype of non-Hodgkin lymphoma, which encompasses a variety of lymphoid malignancies.
2. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes ALCL.
3. Lymphadenopathy: Refers to the enlargement of lymph nodes, which is a common symptom in patients with ALCL.
4. T-Cell Lymphoma: Since ALCL is derived from T-cells, this term is often used in discussions about the disease.
5. ALK Gene Rearrangement: This term refers to the genetic alteration that is characteristic of ALK-positive anaplastic large cell lymphoma.

Clinical Context

Anaplastic large cell lymphoma, particularly the ALK-positive variant, is known for its aggressive nature and can present with various clinical features, including lymphadenopathy, systemic symptoms, and potential extranodal involvement. The identification of ALK positivity is crucial for diagnosis and can influence treatment decisions, as targeted therapies may be more effective in these cases.

In summary, the ICD-10 code C84.68 encompasses a specific diagnosis within the broader category of lymphomas, and understanding its alternative names and related terms can aid in better communication and management of the condition.

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The specific subtype denoted by ICD-10 code C84.68 refers to ALCL that is anaplastic and positive for anaplastic lymphoma kinase (ALK), affecting lymph nodes in multiple sites.

Clinical Description

Definition

Anaplastic large cell lymphoma is a malignancy that arises from T-cells and is often associated with a specific genetic alteration involving the ALK gene. The ALK-positive variant is particularly notable for its distinct clinical and pathological features, which differentiate it from other types of lymphomas.

Epidemiology

ALCL is relatively rare, accounting for approximately 2-3% of all non-Hodgkin lymphomas. The ALK-positive subtype is more common in younger individuals, particularly in children and young adults, and has a better prognosis compared to its ALK-negative counterpart[1].

Pathophysiology

The hallmark of ALK-positive ALCL is the presence of a chromosomal translocation that results in the fusion of the ALK gene with other genes, leading to the production of an abnormal protein that promotes cell proliferation and survival. This genetic alteration is a key factor in the pathogenesis of the disease[2].

Clinical Presentation

Symptoms

Patients with ALK-positive ALCL may present with:
- Lymphadenopathy: Swollen lymph nodes, often in multiple regions of the body.
- B symptoms: Fever, night sweats, and weight loss, which are common in lymphomas.
- Skin lesions: In some cases, patients may develop skin manifestations, including nodules or plaques.

Diagnosis

Diagnosis typically involves:
- Histopathological examination: A biopsy of affected lymph nodes is essential for confirming the diagnosis. The presence of large, pleomorphic cells with a characteristic "hallmark" appearance is indicative of ALCL.
- Immunophenotyping: Immunohistochemical staining is used to identify ALK positivity, which is a critical factor in diagnosing this subtype.
- Cytogenetic analysis: Detection of ALK gene rearrangements can further confirm the diagnosis.

Treatment

Therapeutic Approaches

The treatment of ALK-positive ALCL often includes:
- Chemotherapy: Multi-agent chemotherapy regimens are commonly employed, with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) being a standard approach.
- Targeted therapy: ALK inhibitors, such as crizotinib, have shown efficacy in treating ALK-positive lymphomas and may be used in relapsed or refractory cases.
- Stem cell transplantation: In certain cases, especially for relapsed disease, autologous stem cell transplantation may be considered.

Prognosis

The prognosis for patients with ALK-positive ALCL is generally favorable, with a higher overall survival rate compared to other lymphoma subtypes. Early diagnosis and appropriate treatment are crucial for improving outcomes[3].

Conclusion

ICD-10 code C84.68 encapsulates the clinical complexities of anaplastic large cell lymphoma, ALK-positive, affecting multiple lymph node sites. Understanding the clinical presentation, diagnostic criteria, and treatment options is essential for healthcare providers managing this condition. Continuous research into targeted therapies and genetic profiling holds promise for enhancing treatment strategies and patient outcomes in ALCL.

[1] Source: Epidemiology of Anaplastic Large Cell Lymphoma
[2] Source: Pathophysiology of ALK-positive ALCL
[3] Source: Treatment and Prognosis of Anaplastic Large Cell Lymphoma

Anaplastic Large Cell Lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by specific clinical presentations, signs, symptoms, and patient demographics. The ICD-10 code C84.68 specifically refers to ALCL affecting lymph nodes at multiple sites. Below is a detailed overview of the clinical aspects associated with this condition.

Clinical Presentation

Signs and Symptoms

Patients with ALCL, ALK-positive, often present with a range of symptoms that can vary in severity. Common signs and symptoms include:

• Lymphadenopathy: The most prominent feature is the enlargement of lymph nodes, which may be painless and can occur in multiple regions, including cervical, axillary, and inguinal areas[1].
• Fever: Many patients experience unexplained fevers, which can be intermittent or persistent[1].
• Night Sweats: Profuse sweating during the night is a common symptom, often leading to discomfort and sleep disturbances[1].
• Weight Loss: Unintentional weight loss is frequently reported, contributing to the overall decline in health status[1].
• Fatigue: Patients often feel unusually tired or weak, which can be debilitating and affect daily activities[1].
• Pruritus: Some individuals may experience itching, which can be generalized or localized[1].

Additional Symptoms

In advanced cases, patients may also exhibit:
- Abdominal Pain or Distension: If lymph nodes in the abdomen are involved, this can lead to discomfort or a feeling of fullness[1].
- Respiratory Symptoms: If lymph nodes in the thoracic region are affected, patients may experience cough or difficulty breathing[1].

Patient Characteristics

Demographics

• Age: ALCL, particularly the ALK-positive subtype, is more commonly diagnosed in younger individuals, typically between the ages of 20 and 40 years[1].
• Gender: There is a slight male predominance in the incidence of ALCL[1].
• Ethnicity: While ALCL can affect individuals of any ethnicity, certain studies suggest variations in incidence rates among different populations[1].

Risk Factors

• Genetic Factors: The presence of the ALK gene rearrangement is a hallmark of this lymphoma subtype, which is crucial for diagnosis and treatment planning[1].
• Immune Status: Patients with compromised immune systems, such as those with HIV/AIDS or those undergoing immunosuppressive therapy, may have an increased risk of developing ALCL[1].

Diagnosis and Evaluation

Diagnosis typically involves a combination of clinical evaluation, imaging studies (such as CT scans), and histopathological examination of lymph node biopsies. Immunohistochemistry is used to confirm the presence of ALK protein, which is indicative of the ALK-positive variant of ALCL[1].

Conclusion

Anaplastic Large Cell Lymphoma, ALK-positive, affecting multiple lymph node sites, presents with a distinct set of clinical features, primarily characterized by lymphadenopathy, systemic symptoms like fever and night sweats, and significant demographic trends. Understanding these aspects is crucial for timely diagnosis and effective management of the disease. Early recognition of symptoms and appropriate diagnostic measures can lead to better outcomes for patients diagnosed with this aggressive lymphoma subtype.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by the presence of anaplastic large cells and the expression of the anaplastic lymphoma kinase (ALK) protein. The diagnosis of ALCL, specifically coded as ICD-10 C84.68 for "Anaplastic large cell lymphoma, ALK-positive, lymph nodes of multiple sites," involves several clinical, histological, and immunophenotypic criteria.

Diagnostic Criteria for ALK-Positive Anaplastic Large Cell Lymphoma

1. Clinical Presentation

• Symptoms: Patients often present with lymphadenopathy, which may be localized or generalized. Other symptoms can include fever, night sweats, weight loss, and fatigue, which are common in lymphoproliferative disorders.
• Physical Examination: A thorough examination may reveal enlarged lymph nodes in multiple regions, hepatosplenomegaly, or other systemic signs.

2. Histopathological Examination

• Biopsy: A lymph node biopsy is essential for diagnosis. The histological examination typically shows a polymorphous infiltrate of large atypical lymphoid cells.
• Cell Morphology: The presence of large pleomorphic cells with abundant cytoplasm and prominent nucleoli is characteristic. The cells may exhibit a "hallmark" cell morphology, which is a distinctive feature of ALCL.

3. Immunophenotyping

• Immunohistochemistry: The diagnosis is confirmed through immunohistochemical staining. ALK-positive ALCL typically shows:
• Positive for ALK: The presence of ALK protein is confirmed using specific antibodies (e.g., ALK-1).
• T-cell Markers: The tumor cells may express T-cell markers such as CD30 and CD3, while being negative for B-cell markers.
• Other Markers: Commonly positive markers include CD4, CD5, and CD43, while negative markers may include CD20.

4. Molecular Studies

• Genetic Testing: The presence of ALK gene rearrangements can be confirmed through fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) techniques. The most common rearrangement involves the ALK gene on chromosome 2p23.

5. Staging and Imaging

• Imaging Studies: CT scans or PET scans are often performed to assess the extent of disease involvement in lymph nodes and other organs. The staging is crucial for treatment planning and prognosis.

6. Exclusion of Other Conditions

• Differential Diagnosis: It is important to rule out other lymphomas and reactive lymphadenopathy. Conditions such as peripheral T-cell lymphoma and other subtypes of non-Hodgkin lymphoma may present similarly.

Conclusion

The diagnosis of ALK-positive anaplastic large cell lymphoma, particularly in cases involving multiple lymph node sites, relies on a combination of clinical evaluation, histopathological findings, immunophenotyping, and molecular studies. Accurate diagnosis is essential for appropriate management and treatment planning, as ALCL can have distinct therapeutic approaches compared to other lymphomas. If you have further questions or need more specific information, feel free to ask!

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive subtype, is a rare and aggressive form of non-Hodgkin lymphoma characterized by the presence of the anaplastic lymphoma kinase (ALK) gene rearrangement. The ICD-10 code C84.68 specifically refers to ALCL affecting lymph nodes at multiple sites. The treatment approaches for this condition are multifaceted and typically involve a combination of chemotherapy, targeted therapy, and, in some cases, stem cell transplantation.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains the cornerstone of treatment for ALK-positive ALCL. The most commonly used regimens include:

• CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. It is often the first-line treatment for aggressive lymphomas, including ALCL.
• EPOCH Regimen: This is a more intensive regimen that includes Etoposide, Prednisone, Vincristine, Doxorubicin, and Cyclophosphamide. It may be used in cases where the disease is more aggressive or has not responded to initial treatment.

2. Targeted Therapy

Targeted therapies have significantly improved outcomes for patients with ALK-positive ALCL:

• Brentuximab Vedotin (Adcetris®): This is an antibody-drug conjugate that targets CD30, a protein expressed on the surface of ALCL cells. It is often used in combination with chemotherapy or as a single agent in relapsed or refractory cases. Studies have shown that it can lead to high response rates and improved survival outcomes[7][8].

3. Stem Cell Transplantation

For patients with high-risk disease or those who do not achieve a complete response to initial therapy, autologous stem cell transplantation (ASCT) may be considered. This approach involves harvesting the patient's stem cells, administering high-dose chemotherapy to eradicate the lymphoma, and then reinfusing the stem cells to restore bone marrow function. ASCT has been associated with improved long-term outcomes in patients with ALCL[4].

4. Radiation Therapy

While not a primary treatment modality, radiation therapy may be used in specific situations, such as localized disease or to manage symptoms. It can be particularly effective in treating localized lymph node involvement or residual disease after chemotherapy[1].

5. Clinical Trials

Given the evolving nature of treatment for ALCL, participation in clinical trials may be an option for patients. These trials often explore new therapies, combinations of existing treatments, or novel agents that target specific pathways involved in ALCL.

Conclusion

The treatment of ALK-positive anaplastic large cell lymphoma, particularly with involvement of multiple lymph node sites, is complex and requires a multidisciplinary approach. Standard treatment typically involves a combination of chemotherapy and targeted therapies, with stem cell transplantation considered for high-risk patients. Ongoing research and clinical trials continue to refine these approaches, aiming to improve outcomes and reduce treatment-related toxicity. Patients should discuss their treatment options with their healthcare team to determine the best individualized plan based on their specific circumstances and disease characteristics.