ICD-10: C84.72

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The ICD-10 code C84.72 specifically refers to ALCL that is ALK-negative and involves the intrathoracic lymph nodes. Below is a detailed clinical description and relevant information regarding this condition.

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

Overview

Anaplastic large cell lymphoma is classified under mature T/NK-cell lymphomas and is known for its aggressive nature. The ALK-negative variant is distinguished by the absence of the anaplastic lymphoma kinase (ALK) gene rearrangement, which is often associated with a better prognosis in the ALK-positive subtype. The ALK-negative form tends to occur more frequently in adults and is associated with a poorer prognosis compared to its ALK-positive counterpart[1][2].

Symptoms

Patients with ALCL may present with a variety of symptoms, which can include:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the neck, armpits, or groin.
- Fever: Persistent or intermittent fevers that are unexplained.
- Night Sweats: Excessive sweating during the night.
- Weight Loss: Unintentional weight loss over a short period.
- Fatigue: Generalized weakness and lack of energy.

In the case of C84.72, the involvement of intrathoracic lymph nodes may lead to additional symptoms such as:
- Cough: Due to pressure on the airways.
- Chest Pain: Discomfort or pain in the chest area.
- Shortness of Breath: Difficulty breathing, which may occur if lymph nodes compress the lungs or airways[3].

Diagnosis

Diagnosis of ALCL, particularly the ALK-negative variant, typically involves:
- Histopathological Examination: A biopsy of affected lymph nodes is essential for diagnosis, where pathologists look for characteristic large atypical cells.
- Immunophenotyping: Flow cytometry and immunohistochemistry are used to identify specific markers on the lymphoma cells, confirming the diagnosis.
- Genetic Testing: While ALK rearrangement testing is crucial for distinguishing between ALK-positive and ALK-negative forms, other genetic markers may also be evaluated to understand the disease better[4].

Treatment

The treatment for ALCL, particularly the ALK-negative variant, often includes:
- Chemotherapy: Multi-agent chemotherapy regimens are commonly used, with CHOP (Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) being a standard approach.
- Targeted Therapy: Newer agents, such as brentuximab vedotin, may be utilized, especially in relapsed cases.
- Radiation Therapy: This may be considered in localized disease or as a palliative measure for symptomatic relief[5].

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 patient's overall health, and response to initial treatment. Generally, ALK-negative ALCL is associated with a more aggressive clinical course and a lower overall survival rate compared to ALK-positive cases[6].

Conclusion

ICD-10 code C84.72 identifies a specific subtype of anaplastic large cell lymphoma that is characterized by the absence of the ALK gene rearrangement and the involvement of intrathoracic lymph nodes. Understanding the clinical presentation, diagnostic criteria, treatment options, and prognosis is crucial for effective management of this aggressive lymphoma subtype. Early diagnosis and appropriate treatment are essential for improving patient outcomes.

References

1. C84.7 - Anaplastic large cell lymphoma, ALK-negative.
2. Mature T/NK-cell lymphomas C84 - ICD-10-CM Codes.
3. Clinical Criteria for Anaplastic Large Cell Lymphoma.
4. Common ICD-10 Codes for Flow Cytometry Cancer Testing.
5. Istodax (romidepsin) and its role in treatment.
6. Prognostic factors in Anaplastic Large Cell Lymphoma.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a rare and aggressive form of non-Hodgkin lymphoma. The ICD-10 code C84.72 specifically refers to ALCL that presents with involvement of intrathoracic lymph nodes. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for timely diagnosis and management.

Clinical Presentation

Signs and Symptoms

Patients with ALK-negative anaplastic large cell lymphoma may exhibit a variety of signs and symptoms, which can vary based on the extent of the disease and the specific lymph node involvement. Common clinical presentations include:

• Lymphadenopathy: Patients often present with enlarged lymph nodes, particularly in the cervical, axillary, or mediastinal regions. In the case of C84.72, there is a specific focus on intrathoracic lymph nodes, which may lead to respiratory symptoms due to compression of surrounding structures.

• Respiratory Symptoms: Due to the involvement of intrathoracic lymph nodes, patients may experience symptoms such as:

• Cough
• Shortness of breath (dyspnea)
• Chest pain

• Wheezing

• Systemic Symptoms: Like many lymphomas, ALCL can present with systemic symptoms, including:

• Fever
• Night sweats
• Unexplained weight loss
• Fatigue

Patient Characteristics

The demographic and clinical characteristics of patients with ALK-negative ALCL can provide insight into the typical profile of those affected:

• Age: ALK-negative ALCL is more commonly diagnosed in adults, particularly those in their 30s to 50s, although it can occur in older adults as well.

• Gender: There is a slight male predominance in the incidence of ALCL, with males being more frequently affected than females.

• Histological Features: The diagnosis is confirmed through histopathological examination, which typically reveals large atypical lymphoid cells with pleomorphic nuclei. Immunophenotyping is essential to differentiate ALCL from other lymphomas, with the absence of ALK protein expression being a key feature of the ALK-negative subtype.

• Comorbidities: Patients may have a history of autoimmune diseases or other malignancies, which can complicate the clinical picture and management.

Diagnosis and Management

Diagnosis of ALK-negative ALCL involves a combination of imaging studies, such as CT scans, to assess lymph node involvement, and biopsy of affected lymph nodes for histological examination. Treatment typically includes chemotherapy, and in some cases, targeted therapies or stem cell transplantation may be considered depending on the stage and overall health of the patient.

Conclusion

Anaplastic large cell lymphoma, ALK-negative, with intrathoracic lymph node involvement (ICD-10 code C84.72) presents with a range of symptoms primarily related to lymphadenopathy and respiratory distress. Understanding the clinical features and patient demographics is essential for healthcare providers to facilitate early diagnosis and appropriate treatment strategies. Given the aggressive nature of this lymphoma subtype, prompt recognition and intervention are critical for improving patient outcomes.

Anaplastic large cell lymphoma (ALCL), particularly the subtype identified by the ICD-10 code C84.72, is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. This specific code refers to ALCL that is ALK-negative and involves intrathoracic lymph nodes. Below are alternative names and related terms associated with this condition.

Alternative Names for C84.72

1. Anaplastic Large Cell Lymphoma (ALK-Negative): This is the primary name for the condition, emphasizing the absence of the anaplastic lymphoma kinase (ALK) gene rearrangement, which is a significant factor in the classification of ALCL.

2. ALK-Negative Anaplastic Lymphoma: Another variation that highlights the ALK-negative status of the lymphoma.

3. Systemic Anaplastic Large Cell Lymphoma: While this term can refer to ALCL that is not localized, it may sometimes be used in the context of ALK-negative cases.

4. Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL-NOS): ALCL is classified under this broader category of T-cell lymphomas, particularly when it does not fit into other specific subtypes.

5. Anaplastic Lymphoma: A more general term that may refer to various forms of anaplastic lymphomas, including ALK-positive and ALK-negative types.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): ALCL is a subtype of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers.

2. Intrathoracic Lymphadenopathy: This term refers to the enlargement of lymph nodes within the thoracic cavity, which is a characteristic feature of C84.72.

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

4. T-Cell Lymphoma: Since ALCL is a type of T-cell lymphoma, this term is often used in discussions about its classification and treatment.

5. Lymphoid Neoplasm: A broader category that includes all types of lymphomas and leukemias, including ALCL.

6. Histological Subtypes of Lymphoma: This includes various classifications based on the microscopic appearance of the lymphoma cells, which can be relevant in diagnosing and treating ALCL.

Understanding these alternative names and related terms can aid in better communication among healthcare professionals and enhance patient education regarding the diagnosis and treatment of ALCL, particularly the ALK-negative subtype affecting intrathoracic lymph nodes.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a type of non-Hodgkin lymphoma characterized by specific clinical and pathological features. The diagnosis of ALCL, including the variant coded as C84.72 (Anaplastic large cell lymphoma, ALK-negative, intrathoracic lymph nodes), involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria used for diagnosis:

Clinical Presentation

1. Symptoms: Patients may present with symptoms such as:
   - Lymphadenopathy (swollen lymph nodes)
   - Fever
   - Night sweats
   - Weight loss
   - Fatigue

2. Location of Lymphadenopathy: For C84.72, the lymphadenopathy is specifically noted to be in the intrathoracic region, which includes lymph nodes located in the chest cavity.

Imaging Studies

1. CT or PET Scans: Imaging studies are crucial for assessing the extent of lymphadenopathy and identifying the presence of any mediastinal or intrathoracic lymph nodes. These scans help in staging the disease and determining the involvement of other organs.

Histopathological Examination

1. Biopsy: A definitive diagnosis of ALCL requires a biopsy of the affected lymph nodes. The biopsy can be performed via excisional, incisional, or fine-needle aspiration methods.

2. Histological Features: The pathology report will typically describe:
   - The presence of large atypical lymphoid cells
   - A characteristic "hallmark" cell morphology, which includes a horseshoe-shaped nucleus

3. Immunophenotyping: This is essential for confirming the diagnosis:
   - CD30 positivity: ALCL cells typically express CD30, a surface marker that is a hallmark of this lymphoma.
   - ALK negativity: In the case of C84.72, the absence of anaplastic lymphoma kinase (ALK) protein expression is confirmed through immunohistochemistry.

4. Cytogenetic Studies: While not always necessary, cytogenetic analysis may be performed to identify specific chromosomal abnormalities associated with ALCL.

Differential Diagnosis

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

2. Clinical Criteria: The diagnosis may also consider clinical criteria such as age, gender, and other risk factors that could influence the likelihood of ALCL.

Conclusion

The diagnosis of C84.72 (Anaplastic large cell lymphoma, ALK-negative, intrathoracic lymph nodes) is a multifaceted process that relies on clinical evaluation, imaging studies, and detailed histopathological analysis. The combination of these criteria ensures an accurate diagnosis, which is crucial for determining the appropriate treatment strategy. If you have further questions or need more specific information regarding treatment options or prognosis, feel free to ask!

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a rare and aggressive form of non-Hodgkin lymphoma (NHL) characterized by the presence of large, atypical lymphoid cells. The ICD-10 code C84.72 specifically refers to ALCL that is localized in the intrathoracic lymph nodes. Understanding the standard treatment approaches for this condition is crucial for effective management.

Overview of Anaplastic Large Cell Lymphoma (ALK-Negative)

Anaplastic large cell lymphoma can be classified into two main subtypes based on the presence of the anaplastic lymphoma kinase (ALK) gene. The ALK-positive subtype is generally associated with a better prognosis and is more common in younger patients. In contrast, the ALK-negative subtype, which includes the C84.72 classification, tends to occur in older adults and is associated with a more aggressive clinical course and poorer prognosis[1].

Standard Treatment Approaches

1. Chemotherapy

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

• CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. CHOP is often the first-line treatment for aggressive lymphomas, including ALCL[2].
• EPOCH Regimen: Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin (EPOCH) is another regimen that may be used, particularly in cases that are more resistant to standard CHOP therapy[3].

2. Targeted Therapy

While traditional chemotherapy remains the primary treatment, targeted therapies are being explored. For ALK-negative ALCL, the use of agents such as:

• Brentuximab Vedotin: This is an antibody-drug conjugate that targets CD30, a marker expressed on ALCL cells. It is particularly effective in relapsed or refractory cases and may be used in combination with chemotherapy[4].

3. Radiation Therapy

Radiation therapy may be considered in specific scenarios, particularly for localized disease or as a consolidation treatment after chemotherapy. It can help reduce the risk of local recurrence, especially in patients with bulky disease or those who are not candidates for intensive chemotherapy[5].

4. Stem Cell Transplantation

For patients with relapsed or refractory ALK-negative ALCL, 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 can offer a chance for long-term remission[6].

5. Clinical Trials

Given the aggressive nature of ALK-negative ALCL and the ongoing research in the field, participation in clinical trials may be an option for patients. These trials often explore new combinations of therapies, novel agents, and innovative treatment strategies that may improve outcomes[7].

Conclusion

The management of anaplastic large cell lymphoma, ALK-negative, particularly when involving intrathoracic lymph nodes, requires a multidisciplinary approach. Standard treatment typically involves chemotherapy, with options for targeted therapy and radiation depending on the individual patient's circumstances. As research continues to evolve, new therapies and combinations may offer hope for improved outcomes in this challenging disease. Patients should discuss all available treatment options, including participation in clinical trials, with their healthcare team to determine the best course of action tailored to their specific situation.

References

1. Overview of Anaplastic Large Cell Lymphoma.
2. CHOP Regimen for Aggressive Lymphomas.
3. EPOCH Regimen in Lymphoma Treatment.
4. Brentuximab Vedotin in ALCL Management.
5. Role of Radiation Therapy in Lymphoma.
6. Stem Cell Transplantation for Relapsed ALCL.
7. Importance of Clinical Trials in Lymphoma Treatment.