ICD-10: C84.64

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The specific ICD-10 code C84.64 refers to ALCL that is ALK-positive and localized in the lymph nodes of the axilla and upper limb. Below is a detailed clinical description and relevant information regarding this condition.

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

Overview

Anaplastic large cell lymphoma is a subtype of peripheral T-cell lymphoma, which is a group of aggressive lymphomas. The "ALK-positive" designation indicates that the lymphoma cells express the anaplastic lymphoma kinase (ALK) protein due to a chromosomal rearrangement, specifically the translocation involving the ALK gene. This genetic alteration is a hallmark of a subset of ALCL and is associated with a better prognosis compared to ALK-negative forms of the disease.

Symptoms

Patients with ALK-positive ALCL may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the axillary (armpit) region and upper limbs.
- Systemic Symptoms: Fever, night sweats, and unexplained weight loss, which are common in many lymphomas.
- Skin Involvement: In some cases, patients may develop skin lesions or rashes.

Diagnosis

Diagnosis typically involves:
- Histopathological Examination: A biopsy of the affected lymph nodes is essential for confirming the diagnosis. The presence of large pleomorphic cells and characteristic "hallmark" cells is indicative of ALCL.
- Immunophenotyping: Flow cytometry and immunohistochemistry are used to identify the expression of CD30 and ALK proteins, which are critical for confirming ALK-positive ALCL.
- Genetic Testing: Detection of the ALK gene rearrangement can be performed using techniques such as fluorescence in situ hybridization (FISH).

Staging

Staging of ALCL is crucial for determining the appropriate treatment approach. The Ann Arbor staging system is commonly used, which assesses the extent of lymph node involvement and any extranodal disease.

Treatment Options

The treatment for ALK-positive ALCL typically includes:
- Chemotherapy: Multi-agent chemotherapy regimens, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), are standard. ALK-positive patients may respond well to these regimens.
- Targeted Therapy: The use of ALK inhibitors, such as crizotinib, has shown promise in treating ALK-positive lymphomas.
- Radiation Therapy: In localized cases, radiation may be used to target specific lymph node areas.

Prognosis

The prognosis for patients with ALK-positive ALCL is generally favorable, with higher response rates to treatment and better overall survival compared to ALK-negative cases. However, individual outcomes can vary based on factors such as age, overall health, and the extent of disease at diagnosis.

Conclusion

ICD-10 code C84.64 specifically identifies anaplastic large cell lymphoma that is ALK-positive and localized to the lymph nodes of the axilla and upper limb. Understanding the clinical features, diagnostic criteria, and treatment options for this lymphoma subtype is essential for effective management and improved patient outcomes. Regular follow-up and monitoring are crucial for detecting any potential recurrence or complications associated with the disease.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive subtype, is a type of non-Hodgkin lymphoma characterized by specific clinical and pathological features. The diagnosis of ALCL, including the variant coded as ICD-10 C84.64, involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria used for diagnosis:

Clinical Presentation

1. Symptoms: Patients often present with lymphadenopathy, which may be localized or generalized. In the case of C84.64, the lymph nodes affected are specifically in the axilla and upper limb. Other symptoms may include fever, night sweats, and weight loss, which are common in lymphoproliferative disorders.

2. Physical Examination: A thorough physical examination is essential to assess the extent of lymphadenopathy and to identify any other systemic signs of lymphoma.

Imaging Studies

1. CT or PET Scans: Imaging studies such as computed tomography (CT) or positron emission tomography (PET) scans are utilized to evaluate the extent of disease, including the involvement of lymph nodes and any extranodal sites.

2. Ultrasound: In some cases, ultrasound may be used to assess lymph node characteristics and guide biopsy procedures.

Histopathological Examination

1. Biopsy: A definitive diagnosis of ALCL requires a biopsy of the affected lymph node. This can be an excisional biopsy or a fine-needle aspiration (FNA) followed by core needle biopsy if necessary.

2. Histological Features: The biopsy specimen is examined microscopically for characteristic features of ALCL, including:
   - The presence of large atypical lymphoid cells.
   - A hallmark feature is the "starry sky" appearance due to the presence of macrophages.

3. Immunophenotyping: Immunohistochemical staining is crucial for confirming the diagnosis. ALK-positive ALCL typically shows:
   - Positive staining for CD30 and ALK (anaplastic lymphoma kinase).
   - Negative staining for other markers such as CD15 and CD45.

4. Cytogenetic Analysis: Detection of the ALK gene rearrangement, often through fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR), is a key diagnostic criterion for ALK-positive ALCL.

Differential Diagnosis

1. Exclusion of Other Lymphomas: It is important to differentiate ALCL from other types of lymphomas, such as peripheral T-cell lymphoma and Hodgkin lymphoma, which may present similarly.

2. Clinical and Laboratory Correlation: The diagnosis should be correlated with clinical findings and laboratory results to rule out other conditions that may mimic ALCL.

Conclusion

The diagnosis of Anaplastic Large Cell Lymphoma, ALK-positive (ICD-10 code C84.64), is a multifaceted process that relies on clinical evaluation, imaging studies, and detailed histopathological analysis. The integration of these diagnostic criteria is essential for accurate identification and appropriate management of this lymphoma subtype. If you have further questions or need more specific information, feel free to ask!

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 C84.64 specifically refers to ALCL affecting the lymph nodes of the axilla and upper limb. Below is a detailed overview of the clinical aspects associated with this condition.

Clinical Presentation

Overview of Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphoma is a subtype of peripheral T-cell lymphoma. The ALK-positive variant is associated with a chromosomal translocation involving the anaplastic lymphoma kinase (ALK) gene, which plays a crucial role in the pathogenesis of the disease. This subtype is more common in younger patients and is known for its aggressive behavior but can also be responsive to treatment.

Signs and Symptoms

Patients with ALK-positive ALCL may present with a variety of signs and symptoms, which can include:

• Lymphadenopathy: The most common presentation is the enlargement of lymph nodes, particularly in the axillary region and upper limbs. Patients may notice swollen lymph nodes that are firm and non-tender.
• Systemic Symptoms: Common systemic symptoms include:
• Fever
• Night sweats
• Unexplained weight loss
• Fatigue
• Skin Involvement: Some patients may develop skin lesions, which can appear as nodules or plaques. These lesions may be itchy or painful.
• B Symptoms: The presence of fever, drenching night sweats, and weight loss exceeding 10% of body weight in six months is often referred to as "B symptoms" and is significant in the staging and prognosis of lymphomas.

Patient Characteristics

The demographic profile of patients with ALK-positive ALCL typically includes:

• Age: This lymphoma predominantly affects younger individuals, often between the ages of 20 and 40, although it can occur in children and older adults as well.
• Gender: There is a slight male predominance in the incidence of ALCL.
• Ethnicity: While ALCL can affect individuals of any ethnicity, some studies suggest variations in incidence rates among different ethnic groups.

Diagnosis and Staging

Diagnosis of ALK-positive ALCL involves a combination of clinical evaluation, imaging studies, and histopathological examination. Key diagnostic steps include:

• Imaging Studies: CT scans or PET scans are often used to assess the extent of lymphadenopathy and any involvement of other organs.
• Biopsy: A lymph node biopsy is essential for histological confirmation. The presence of characteristic large atypical cells and immunohistochemical staining for ALK protein are critical for diagnosis.
• Staging: The Ann Arbor staging system is commonly used to determine the extent of the disease, which influences treatment decisions.

Conclusion

Anaplastic large cell lymphoma, ALK-positive, particularly affecting the lymph nodes of the axilla and upper limb, presents with distinct clinical features and patient demographics. Early recognition of symptoms such as lymphadenopathy and systemic B symptoms is crucial for timely diagnosis and management. Given its aggressive nature, understanding the clinical presentation and characteristics of this lymphoma subtype is essential for healthcare providers in order to optimize patient outcomes through appropriate treatment strategies.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by the presence of anaplastic lymphoma kinase (ALK) gene rearrangements. The ICD-10 code C84.64 specifically refers to ALCL located in the lymph nodes of the axilla and upper limb. 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 nature 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 when referring to cases that are not localized and involve systemic symptoms.
4. Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL-NOS): While not synonymous, ALCL is a subtype of PTCL, and this term may be used in broader discussions of T-cell lymphomas.

Related Terms

1. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes various types, including ALCL.
2. Non-Hodgkin Lymphoma (NHL): A broader category that includes ALCL among other types of lymphomas.
3. Lymphadenopathy: Refers to the enlargement of lymph nodes, which is a common symptom in lymphomas, including ALCL.
4. Cytogenetic Abnormalities: Refers to the genetic changes associated with ALK-positive ALCL, particularly the ALK gene rearrangement.
5. Histopathology: The study of the microscopic structure of tissues, which is crucial for diagnosing ALCL.

Clinical Context

Understanding these terms is essential for healthcare professionals involved in the diagnosis and treatment of lymphomas. The ALK-positive variant of ALCL is particularly notable for its distinct clinical behavior and response to targeted therapies, making accurate coding and terminology critical for effective patient management and research.

In summary, the ICD-10 code C84.64 is associated with various alternative names and related terms that reflect the nature of Anaplastic Large Cell Lymphoma, particularly its ALK-positive variant, and its clinical implications.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by the presence of anaplastic lymphoma kinase (ALK) gene rearrangements. The ICD-10 code C84.64 specifically refers to ALCL affecting the lymph nodes of the axilla and upper limb. The treatment approaches for this condition are multifaceted and typically involve a combination of chemotherapy, targeted therapy, and, in some cases, stem cell transplantation. Below is a detailed overview of the standard treatment strategies.

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. CHOP is often the initial treatment for patients with advanced-stage disease.
• CHOP-like Regimens: Variations of the CHOP regimen, such as CHOEP (adding Etoposide), may be used depending on the patient's specific circumstances and disease characteristics.

1.1 Response Assessment

After initial chemotherapy, patients are typically assessed for response through imaging studies (like PET scans) and clinical evaluation. A complete response may lead to further treatment decisions, including consolidation therapy.

2. Targeted Therapy

For patients with ALK-positive ALCL, targeted therapies have significantly improved outcomes:

• Brentuximab Vedotin (Adcetris®): This is an antibody-drug conjugate that targets CD30, a marker expressed on ALCL cells. It is often used in patients who have relapsed or are refractory to initial chemotherapy. It can also be used in combination with chemotherapy for newly diagnosed patients.

2.1 Combination Approaches

Brentuximab can be combined with chemotherapy regimens to enhance efficacy, particularly in patients with high-risk features or those with advanced disease.

3. Stem Cell Transplantation

For patients who do not achieve a complete response to initial therapy or those with relapsed disease, hematopoietic stem cell transplantation (HCT) may be considered:

• Autologous Stem Cell Transplantation: This is often performed after high-dose chemotherapy in patients who have responded well to initial treatment.
• Allogeneic Stem Cell Transplantation: This may be an option for patients with relapsed disease or those who have high-risk features.

4. Radiation Therapy

While not the primary treatment modality for systemic ALCL, radiation therapy may be utilized in specific scenarios:

• Localized Disease: Radiation can be effective for localized disease, particularly if there is a significant mass or if the lymphoma is confined to a specific area, such as the axilla.
• Palliative Care: In cases of symptomatic disease, radiation may help alleviate symptoms.

5. Clinical Trials

Patients with ALK-positive ALCL are encouraged to consider participation in clinical trials, which may offer access to novel therapies and treatment strategies that are not yet widely available. These trials can provide cutting-edge options and contribute to the advancement of treatment for this lymphoma subtype.

Conclusion

The treatment of anaplastic large cell lymphoma, ALK-positive, particularly in the lymph nodes of the axilla and upper limb, involves a comprehensive approach that includes chemotherapy, targeted therapies like brentuximab vedotin, and potential stem cell transplantation. The choice of treatment is tailored to the individual patient based on disease stage, response to initial therapy, and overall health. Ongoing research and clinical trials continue to refine these strategies, aiming to improve outcomes for patients with this aggressive lymphoma subtype.