ICD-10: C84.62

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive subtype, is a type of non-Hodgkin lymphoma characterized by the presence of anaplastic lymphoma kinase (ALK) gene rearrangements. The ICD-10 code C84.62 specifically refers to ALCL affecting intrathoracic lymph nodes. The treatment approaches for this condition are multifaceted and typically involve a combination of chemotherapy, targeted therapy, and, in some cases, radiation therapy. Below is a detailed overview of the standard treatment strategies for ALK-positive ALCL.

1. Chemotherapy

First-Line Treatment

The cornerstone of treatment for ALK-positive ALCL is 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 initial approach for ALCL.
• CHOP-like Regimens: Variations of the CHOP regimen, such as EPOCH (Etoposide, Prednisone, Vincristine, Doxorubicin, Cyclophosphamide), may also be employed, particularly in cases with more aggressive disease.

Response Assessment

After the initial treatment cycle, imaging studies (such as PET scans) are typically performed to assess the response to therapy. A complete response may lead to further treatment adjustments.

2. Targeted Therapy

Brentuximab Vedotin

Brentuximab vedotin (Adcetris®) is a CD30-targeted antibody-drug conjugate that has shown significant efficacy in treating ALCL. It is often used in the following scenarios:

• As Consolidation Therapy: After initial chemotherapy, brentuximab may be used to reduce the risk of relapse.
• In Relapsed or Refractory Cases: For patients who do not respond to initial treatment or experience a relapse, brentuximab can be an effective option.

ALK Inhibitors

For patients with ALK-positive ALCL, ALK inhibitors such as crizotinib or ceritinib may be considered, especially in cases where the disease is resistant to standard chemotherapy.

3. Radiation Therapy

Indications for Radiation

Radiation therapy may be utilized in specific situations, including:

• Localized Disease: If the lymphoma is confined to a limited area, radiation can be effective in achieving local control.
• Post-Chemotherapy Consolidation: In some cases, radiation may be administered after chemotherapy to eliminate residual disease, particularly in bulky disease or after achieving a partial response.

4. Stem Cell Transplantation

Hematopoietic Stem Cell Transplant (HSCT)

For patients with high-risk features or those who experience relapse after initial treatment, autologous stem cell transplantation may be considered. This approach is typically reserved for younger patients with good performance status and those who have responded well to initial therapy.

5. Clinical Trials

Participation in clinical trials may also be an option for patients with ALK-positive ALCL. These trials often explore new therapies or combinations that may offer improved outcomes compared to standard treatments.

Conclusion

The treatment of anaplastic large cell lymphoma, ALK-positive, affecting intrathoracic lymph nodes (ICD-10 code C84.62) involves a comprehensive approach that includes chemotherapy, targeted therapies, and potentially radiation or stem cell transplantation. The choice of treatment is influenced by various factors, including the stage of the disease, patient health, and response to initial therapies. Ongoing research and clinical trials continue to refine these treatment strategies, aiming to improve outcomes for patients with this aggressive lymphoma subtype.

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The specific subtype identified by the ICD-10 code C84.62 refers to ALCL that is positive for anaplastic lymphoma kinase (ALK) 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-positive)

Overview

Anaplastic large cell lymphoma is a hematological malignancy that primarily affects T-cells. The ALK-positive variant is particularly notable for its association with a chromosomal translocation involving the ALK gene, which leads to the production of an abnormal protein that promotes cell growth and survival. This subtype is more common in younger individuals and is often aggressive but can be responsive to treatment.

Symptoms

Patients with ALK-positive ALCL may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the neck, armpits, or groin.
- B symptoms: These include fever, night sweats, and unexplained weight loss.
- Respiratory symptoms: Due to the involvement of intrathoracic lymph nodes, patients may experience cough, chest pain, or difficulty breathing.
- Fatigue: Generalized weakness and fatigue are common.

Diagnosis

Diagnosis of ALK-positive ALCL typically involves:
- Histopathological examination: A biopsy of affected lymph nodes is essential for diagnosis, where the presence of large atypical cells is confirmed.
- Immunohistochemistry: Testing for ALK protein expression is crucial, as it distinguishes ALK-positive from ALK-negative variants.
- Imaging studies: CT scans or PET scans are often used to assess the extent of disease, particularly in the thoracic region.

Staging

The staging of ALCL is critical for determining treatment options and prognosis. The Ann Arbor staging system is commonly used, which considers the number of lymph node regions involved and whether the disease has spread to other organs.

Treatment Options

Treatment for ALK-positive ALCL typically includes:
- Chemotherapy: Multi-agent chemotherapy regimens, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), are standard.
- Targeted therapy: ALK inhibitors, such as crizotinib, may be used, particularly in relapsed or refractory cases.
- Radiation therapy: This may be employed for localized disease or to manage symptoms related to lymph node involvement.

Prognosis

The prognosis for patients with ALK-positive ALCL is generally favorable compared to other lymphoma types, especially when diagnosed early and treated appropriately. The overall survival rates can be high, with many patients achieving remission.

Conclusion

ICD-10 code C84.62 specifically identifies anaplastic large cell lymphoma that is ALK-positive and involves intrathoracic lymph nodes. 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 to detect any recurrence or complications early.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive subtype, is a type of non-Hodgkin lymphoma characterized by specific clinical presentations, signs, symptoms, and patient demographics. The ICD-10 code C84.62 specifically refers to ALCL that is localized in the intrathoracic lymph nodes. Below is a detailed overview of the clinical aspects associated with this condition.

Clinical Presentation

Signs and Symptoms

Patients with ALK-positive anaplastic large cell lymphoma may present with a variety of symptoms, which can vary based on the extent of the disease and the specific lymph nodes involved. Common signs and symptoms include:

• Lymphadenopathy: Swelling of lymph nodes, particularly in the neck, axilla, or groin, is often the first noticeable sign. In the case of intrathoracic involvement, mediastinal lymphadenopathy may be observed.
• Respiratory Symptoms: Patients may experience cough, shortness of breath, or chest pain due to the involvement of intrathoracic lymph nodes, which can compress surrounding structures.
• Fever: Persistent or intermittent fevers may occur, often accompanied by night sweats.
• Weight Loss: Unintentional weight loss is common and can be significant.
• Fatigue: A general sense of tiredness or lack of energy is frequently reported.

Additional Symptoms

• Skin Lesions: Some patients may develop skin lesions, which can appear as nodules or plaques, particularly in cutaneous forms of ALCL.
• Bone Pain: If the lymphoma spreads to the bones, patients may experience localized pain.

Patient Characteristics

Demographics

• Age: ALK-positive ALCL is more commonly diagnosed in younger individuals, typically between the ages of 10 and 35, although it can occur in older adults as well.
• Gender: There is a slight male predominance in the incidence of ALCL.
• Ethnicity: The condition is observed across various ethnic groups, but certain subtypes may have varying prevalence in different populations.

Risk Factors

• Genetic Factors: The presence of the ALK gene rearrangement is a hallmark of this subtype, which is often identified through genetic testing.
• Immune Status: Patients with compromised immune systems, such as those with HIV/AIDS or those who have undergone organ transplantation, may have an increased risk of developing lymphomas, including ALCL.

Diagnosis and Evaluation

Diagnosis typically involves a combination of imaging studies (such as CT scans to assess lymph node involvement), biopsy of affected lymph nodes, and immunohistochemical staining to confirm the presence of ALK positivity.

Staging

Staging of ALCL is crucial for determining treatment options and prognosis. The Ann Arbor staging system is commonly used, which considers the number of lymph node regions involved and whether the disease has spread to other organs.

Conclusion

Anaplastic large cell lymphoma, ALK-positive, particularly when involving intrathoracic lymph nodes, presents with a range of symptoms primarily related to lymphadenopathy and respiratory distress. Understanding the clinical presentation, signs, symptoms, and patient characteristics is essential for timely diagnosis and effective management of this aggressive lymphoma subtype. Early recognition and intervention can significantly impact patient outcomes, making awareness of these clinical features critical for healthcare providers.

ICD-10 code C84.62 specifically refers to Anaplastic Large Cell Lymphoma (ALCL), ALK-positive, involving intrathoracic lymph nodes. This classification is part of a broader category of non-Hodgkin lymphomas and has several alternative names and related terms that can be useful for understanding its context in medical coding and diagnosis.

Alternative Names for C84.62

1. Anaplastic Large Cell Lymphoma (ALCL): This is the primary name for the condition, emphasizing its classification as a type of lymphoma.
2. ALK-Positive Anaplastic Large Cell Lymphoma: This term highlights the presence of the anaplastic lymphoma kinase (ALK) gene rearrangement, which is a key characteristic of this lymphoma subtype.
3. Systemic Anaplastic Large Cell Lymphoma: While this term may refer to cases that are not limited to lymph nodes, it is sometimes used interchangeably in discussions about ALCL.
4. Primary Anaplastic Large Cell Lymphoma: This term can be used to denote cases where the lymphoma originates in the lymph nodes, including intrathoracic locations.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): C84.62 falls under the broader category of non-Hodgkin lymphomas, which includes various types of lymphatic cancers.
2. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with ALCL.
3. Intrathoracic Lymph Nodes: This term specifies the location of the lymph nodes affected by the lymphoma, which is crucial for diagnosis and treatment planning.
4. ALK Gene Rearrangement: This genetic alteration is a hallmark of ALK-positive anaplastic large cell lymphoma and is often tested for in diagnosis.
5. Peripheral T-cell Lymphoma: ALCL is classified as a type of peripheral T-cell lymphoma, which encompasses various lymphomas derived from T-cells.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C84.62 is essential for accurate diagnosis, treatment planning, and medical coding. These terms not only facilitate communication among healthcare professionals but also enhance the clarity of patient records and insurance documentation. If you need further details on treatment options or prognosis related to ALCL, feel free to ask!

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive subtype, 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 C84.62 specifically refers to ALCL that is localized in the intrathoracic lymph nodes. The diagnostic criteria for this condition involve a combination of clinical, histopathological, and immunophenotypic evaluations.

Diagnostic Criteria for Anaplastic Large Cell Lymphoma (ALK-Positive)

1. Clinical Presentation

• Symptoms: Patients may present with symptoms such as lymphadenopathy (swollen lymph nodes), fever, night sweats, and weight loss. In cases where the lymphoma is localized to intrathoracic lymph nodes, respiratory symptoms may also be present due to mediastinal involvement.
• Physical Examination: A thorough physical examination may reveal enlarged lymph nodes in the neck, axilla, or groin, as well as signs of respiratory distress if thoracic lymph nodes are involved.

2. Imaging Studies

• CT Scans: Imaging studies, particularly computed tomography (CT) scans of the chest, abdomen, and pelvis, are essential for assessing the extent of lymphadenopathy and determining the involvement of intrathoracic structures.
• PET Scans: Positron emission tomography (PET) scans may be utilized to evaluate metabolic activity in lymph nodes and to help stage the disease.

3. Histopathological Examination

• Biopsy: A definitive diagnosis requires a biopsy of the affected lymph node. This can be performed via excisional biopsy, core needle biopsy, or fine-needle aspiration.
• Histology: The biopsy specimen is examined microscopically for the presence of large atypical lymphoid cells. The hallmark of ALCL is the presence of pleomorphic, large cells with abundant cytoplasm and prominent nucleoli.

4. Immunophenotyping

• Immunohistochemistry: The biopsy sample is subjected to immunohistochemical staining to identify specific markers. ALK-positive ALCL typically shows:
  • CD30 positivity: A key marker for ALCL.
  • ALK positivity: Detected using antibodies against the ALK protein, confirming the diagnosis of ALK-positive ALCL.
  • Other markers: The tumor may also express other lymphoid markers such as CD3, CD4, and CD5, but is usually negative for CD15 and CD20.

5. Cytogenetic and Molecular Studies

• Genetic Testing: The presence of the ALK gene rearrangement can be confirmed through fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) techniques. This genetic alteration is a defining feature of ALK-positive ALCL.

6. Exclusion of Other Conditions

• Differential Diagnosis: It is crucial to differentiate ALK-positive ALCL from other lymphomas and reactive lymphadenopathies. This may involve additional immunophenotyping and molecular studies to rule out other subtypes of lymphoma.

Conclusion

The diagnosis of anaplastic large cell lymphoma, ALK-positive, particularly in the context of intrathoracic lymph nodes (ICD-10 code C84.62), relies on a comprehensive approach that includes clinical evaluation, imaging studies, histopathological examination, immunophenotyping, and genetic testing. 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!