ICD-10: C84.73

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a rare and aggressive form of non-Hodgkin lymphoma. The ICD-10 code C84.73 specifically refers to ALCL that presents with involvement of intra-abdominal lymph nodes. Understanding the standard treatment approaches for this condition is crucial for effective management and patient outcomes.

Overview of Anaplastic Large Cell Lymphoma (ALK-Negative)

Anaplastic large cell lymphoma is characterized by the presence of large, atypical lymphoid cells. The ALK-negative variant is notable for its poor prognosis compared to the ALK-positive subtype, which is often more responsive to treatment. ALCL can manifest in various anatomical locations, and when it involves intra-abdominal lymph nodes, it may present with symptoms such as abdominal pain, swelling, or systemic symptoms like fever and weight loss.

Standard Treatment Approaches

1. Chemotherapy

The cornerstone of treatment for ALCL, including the ALK-negative variant, is chemotherapy. The most commonly used regimens include:

• CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. CHOP is a standard treatment for aggressive lymphomas 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 where CHOP is not effective or in patients with high-risk features[1].

• Dose-Adjusted EPOCH: This variant of EPOCH has shown improved outcomes in some studies and may be considered for patients with more advanced disease[1].

2. Targeted Therapy

For patients with ALK-negative ALCL, targeted therapies are being explored, although they are not yet standard practice. Some options include:

• Brentuximab Vedotin: This is an antibody-drug conjugate that targets CD30, a marker expressed on ALCL cells. It has shown efficacy in relapsed or refractory cases and may be used in combination with chemotherapy or as a single agent[1][2].

3. Radiation Therapy

Radiation therapy may be considered in specific scenarios, particularly for localized disease or as a consolidation treatment after chemotherapy. It is not typically the primary treatment for advanced disease but can be beneficial in managing localized lymph node involvement[1].

4. Stem Cell Transplantation

For patients with relapsed or refractory ALCL, high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be an option. This approach is generally reserved for younger patients or those with good performance status[1][2].

5. Clinical Trials

Given the aggressive nature of ALK-negative ALCL and the ongoing research in this area, participation in clinical trials may be a viable option for patients. These trials may offer access to novel therapies and combinations that are not yet widely available[2].

Conclusion

The treatment of anaplastic large cell lymphoma, ALK-negative, particularly when involving intra-abdominal lymph nodes, typically involves a combination of chemotherapy, potential targeted therapies, and possibly radiation or stem cell transplantation for select patients. Given the complexity and variability of this disease, treatment should be tailored to the individual patient, considering factors such as disease stage, patient health, and response to initial therapies. Ongoing research and clinical trials continue to shape the landscape of treatment options, providing hope for improved outcomes in this challenging lymphoma subtype.

For the most current treatment protocols and clinical trial opportunities, consultation with a hematologist or oncologist specializing in lymphomas is recommended.

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 C84.73 specifically refers to ALCL that is ALK-negative and involves intra-abdominal 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 particularly significant as it lacks the anaplastic lymphoma kinase (ALK) gene rearrangement, which is often associated with a better prognosis in ALK-positive cases. 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][5].

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 fevers that are often unexplained.
- Night Sweats: Excessive sweating during the night.
- Weight Loss: Unintentional weight loss over a short period.
- Abdominal Symptoms: In cases involving intra-abdominal lymph nodes, patients may experience abdominal pain, distension, or discomfort due to lymph node enlargement[1][6].

Diagnosis

Diagnosis of ALCL typically involves:
- Histopathological Examination: A biopsy of affected lymph nodes is essential for diagnosis, where the presence of large atypical lymphoid cells is confirmed.
- Immunophenotyping: This process helps to identify the specific type of lymphoma by analyzing the proteins expressed on the surface of the cells. ALK-negative ALCL typically expresses CD30 and may express other T-cell markers.
- Imaging Studies: CT scans or PET scans are often used to assess the extent of the disease, particularly to evaluate intra-abdominal lymph node involvement[1][4].

Treatment

The treatment for ALCL, particularly the ALK-negative variant, may include:
- Chemotherapy: Multi-agent chemotherapy regimens are commonly employed, often including drugs such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone).
- Targeted Therapy: Newer therapies may be considered, especially in relapsed cases, including agents like brentuximab vedotin, which targets CD30.
- Stem Cell Transplantation: In cases of refractory disease or relapse, allogeneic stem cell transplantation may be considered as a curative option[2][3][6].

Prognosis

The prognosis for patients with ALK-negative ALCL is generally less favorable than for those with the ALK-positive variant. Factors influencing prognosis include the stage of the disease at diagnosis, the patient's overall health, and response to initial treatment. The five-year survival rate can vary significantly based on these factors, with advanced-stage disease typically associated with poorer outcomes[1][5].

Conclusion

ICD-10 code C84.73 designates anaplastic large cell lymphoma, ALK-negative, specifically affecting intra-abdominal lymph nodes. This condition is characterized by aggressive behavior and requires prompt diagnosis and treatment. Understanding the clinical features, diagnostic methods, and treatment options is crucial for managing this lymphoma effectively. Ongoing research continues to explore better therapeutic strategies and improve outcomes for patients with this challenging diagnosis.

References

1. Mature T/NK-cell lymphomas C84 - ICD-10-CM Codes.
2. Folotyn® (pralatrexate).
3. Istodax®; Romidepsin (liquid).
4. Billing and Coding: Allogeneic Hematopoietic Cell.
5. C84.73 - Anaplastic large cell lymphoma, ALK-negative, intra-abdominal lymph nodes.
6. NON-HODGKIN LYMPHOMA Includes Follicular.

Anaplastic Large Cell Lymphoma (ALCL), particularly the ALK-negative subtype, is a rare and aggressive form of non-Hodgkin lymphoma that primarily affects T-cells. The ICD-10 code C84.73 specifically refers to ALCL with involvement of intra-abdominal 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 ALCL, ALK-negative, may present with a variety of symptoms, which can vary significantly among individuals. Common signs and symptoms include:

• Lymphadenopathy: Swelling of lymph nodes, particularly in the abdomen, which may be palpable during physical examination.
• Abdominal Pain or Discomfort: Patients often report pain or a feeling of fullness in the abdominal area due to enlarged lymph nodes.
• 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.
• Skin Lesions: Some patients may develop skin rashes or nodules, although this is less common in the intra-abdominal presentation.

Patient Characteristics

The demographic and clinical characteristics of patients diagnosed with ALCL, ALK-negative, can include:

• Age: ALCL can occur in both adults and children, but the ALK-negative subtype is more commonly diagnosed in adults, typically between the ages of 40 and 60.
• Gender: There is a slight male predominance in cases of ALCL.
• History of Immunosuppression: Patients with a history of immunosuppressive conditions or treatments (e.g., organ transplant recipients) may be at higher risk.
• Associated Conditions: Some patients may have a history of autoimmune diseases or other malignancies, which can complicate the clinical picture.

Diagnosis and Evaluation

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

• Imaging Studies: CT scans or PET scans are often utilized to assess lymph node involvement and to evaluate the extent of disease.
• Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph nodes, where histological examination reveals characteristic features of ALCL.
• Immunophenotyping: Flow cytometry and immunohistochemistry are used to confirm the presence of T-cell markers and the absence of ALK protein, which distinguishes ALK-negative ALCL from its ALK-positive counterpart.

Conclusion

Anaplastic Large Cell Lymphoma, ALK-negative, with intra-abdominal lymph node involvement presents with a range of symptoms, primarily related to lymphadenopathy and systemic manifestations such as fever and weight loss. Understanding the clinical presentation and patient characteristics is essential for healthcare providers to facilitate early diagnosis and appropriate treatment strategies. Given the aggressive nature of this lymphoma subtype, timely intervention can significantly impact patient outcomes.

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 C84.73 specifically refers to ALCL that is localized in the intra-abdominal lymph nodes. Here are some alternative names and related terms associated with this condition:

Alternative Names

1. Anaplastic Large Cell Lymphoma (ALK-Negative): This is the primary name used to describe the condition, emphasizing the absence of the anaplastic lymphoma kinase (ALK) gene rearrangement.
2. ALCL, ALK-Negative: A shorthand version that is commonly used in clinical settings.
3. 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 other specific subtypes.
4. Systemic Anaplastic Large Cell Lymphoma: This term may be used when the lymphoma is not confined to a specific area and can affect multiple lymph nodes or organs.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): ALCL is a subtype of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers that include various types of lymphomas.
2. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with ALCL.
3. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes ALCL.
4. T-Cell Lymphoma: Since ALCL is derived from T-cells, this term is often used in discussions about its pathology and treatment.
5. Intra-Abdominal Lymphadenopathy: This term specifically describes the enlargement of lymph nodes within the abdominal cavity, which is relevant for the C84.73 code.

Conclusion

Understanding the various names and related terms for ICD-10 code C84.73 can aid in better communication among healthcare professionals and enhance patient education. These terms reflect the specific characteristics of the disease, its classification, and its clinical presentation. If you need further information on treatment options or prognosis related to ALCL, feel free to ask!

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 C84.73 (Anaplastic large cell lymphoma, ALK-negative, intra-abdominal lymph nodes), involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria used for diagnosis:

Clinical Criteria

1. Symptoms: Patients may present with systemic symptoms such as fever, night sweats, and weight loss, which are common in lymphoproliferative disorders. Localized symptoms may arise from lymphadenopathy or organ involvement, particularly in the abdominal region.

2. Physical Examination: A thorough physical examination may reveal lymphadenopathy, hepatosplenomegaly, or other signs of systemic illness.

Imaging Studies

1. CT or PET Scans: Imaging studies, particularly computed tomography (CT) or positron emission tomography (PET) scans, are essential for assessing the extent of lymphadenopathy and any intra-abdominal involvement. These scans help identify enlarged lymph nodes and other potential sites of disease.

Histopathological Examination

1. Biopsy: A definitive diagnosis of ALCL requires a biopsy of the affected lymph nodes or tissue. The biopsy specimen is examined microscopically for characteristic features.

2. Morphological Features: Pathologists look for large pleomorphic cells, often with a hallmark cell appearance, which are typically large, anaplastic, and may exhibit a "starry sky" pattern due to the presence of macrophages.

3. Immunophenotyping: Immunohistochemical staining is crucial for confirming the diagnosis. ALCL cells typically express CD30 and may express other markers such as CD4, CD43, and EMA (epithelial membrane antigen). The absence of ALK (anaplastic lymphoma kinase) expression is a defining feature of the ALK-negative subtype.

4. Cytogenetic Analysis: While not always performed, cytogenetic studies can help identify specific chromosomal abnormalities associated with ALCL. The presence of the t(2;5) translocation is common in ALK-positive cases but absent in ALK-negative cases.

Differential Diagnosis

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

2. Clinical Context: The patient's clinical history, including any previous malignancies or treatments, is also considered in the diagnostic process.

Conclusion

The diagnosis of C84.73 (Anaplastic large cell lymphoma, ALK-negative, intra-abdominal lymph nodes) relies on a combination of clinical presentation, imaging studies, and histopathological findings. The integration of these diagnostic criteria is crucial for accurate identification and subsequent management of this lymphoma subtype. If you have further questions or need more specific information, feel free to ask!