ICD-10: C84.7

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 C84.7 refers to ALK-negative anaplastic large cell lymphoma, which is notable for the absence of the anaplastic lymphoma kinase (ALK) gene rearrangement. This distinction is crucial as it influences both the clinical presentation and treatment strategies.

Clinical Description

Definition and Characteristics

ALK-negative anaplastic large cell lymphoma is a hematological malignancy that primarily affects T-cells. It is classified under peripheral T-cell lymphomas and is characterized by the proliferation of large, pleomorphic cells that express the CD30 antigen. Unlike its ALK-positive counterpart, ALK-negative ALCL does not have the genetic alteration associated with the ALK gene, which has implications for prognosis and treatment response.

Epidemiology

This subtype of ALCL is more commonly observed in adults, although it can occur in children and adolescents. The incidence is relatively low compared to other lymphomas, but it is significant enough to warrant attention in clinical practice. The median age at diagnosis is typically in the 30s to 40s, and there is a slight male predominance.

Symptoms

Patients with ALK-negative ALCL may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, often painless.
- B symptoms: Fever, night sweats, and weight loss.
- Skin lesions: Some patients may develop cutaneous manifestations, which can appear as nodules or plaques.
- Organ involvement: In advanced cases, the lymphoma may infiltrate other organs, leading to additional symptoms depending on the affected areas.

Diagnosis

Diagnosis of ALK-negative anaplastic large cell lymphoma typically involves:
- Histopathological examination: Biopsy of affected lymph nodes or tissues, revealing characteristic large cells.
- Immunophenotyping: Flow cytometry or immunohistochemistry to confirm the expression of CD30 and the absence of ALK.
- Cytogenetic studies: To rule out other lymphomas and confirm the diagnosis.

Treatment Options

Chemotherapy

The primary treatment for ALK-negative ALCL often involves chemotherapy regimens, which may include:
- CHOP regimen: Cyclophosphamide, doxorubicin, vincristine, and prednisone.
- Escalated BEACOPP: A more intensive regimen that may be considered in certain cases.

Targeted Therapy

Recent advancements have introduced targeted therapies, such as:
- Brentuximab vedotin: An antibody-drug conjugate targeting CD30, which has shown efficacy in relapsed or refractory cases.

Stem Cell Transplantation

For patients with high-risk disease or those who do not respond to initial therapy, allogeneic stem cell transplantation may be considered as a curative option.

Prognosis

The prognosis for ALK-negative anaplastic large cell lymphoma can vary significantly based on several factors, including the stage at diagnosis, the presence of B symptoms, and the patient's overall health. Generally, the absence of the ALK gene rearrangement is associated with a poorer prognosis compared to ALK-positive cases, but individual outcomes can differ widely.

In summary, ICD-10 code C84.7 identifies ALK-negative anaplastic large cell lymphoma, a distinct subtype of lymphoma with specific clinical features, treatment approaches, and prognostic implications. Understanding these details is essential for effective diagnosis and management of this condition.

Anaplastic large cell lymphoma (ALCL), specifically the ALK-negative subtype coded as C84.7 in the ICD-10 classification, is a rare and aggressive form of non-Hodgkin lymphoma. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for timely diagnosis and management.

Clinical Presentation

Overview of Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphoma is characterized by the proliferation of large, atypical lymphoid cells. The ALK-negative variant is particularly notable for its distinct clinical and biological features compared to the ALK-positive subtype. ALK-negative ALCL typically occurs in older adults and is associated with a poorer prognosis than its ALK-positive counterpart[1][2].

Signs and Symptoms

The clinical manifestations of ALK-negative ALCL can vary widely among patients, but common signs and symptoms include:

• Lymphadenopathy: Swollen lymph nodes are often the first noticeable sign. These can be localized or generalized, affecting multiple regions of the body.
• B Symptoms: Patients may experience systemic symptoms such as:
• Fever
• Night sweats
• Unexplained weight loss
• Skin Involvement: Some patients may present with skin lesions, which can appear as nodules or plaques. This is particularly common in primary cutaneous ALCL, a variant of the disease[3].
• Fatigue: A general sense of tiredness or lack of energy is frequently reported.
• Pruritus: Itching may occur, especially in cases with skin involvement.
• Organ Involvement: In advanced stages, ALCL can affect various organs, leading to symptoms related to those specific areas, such as respiratory distress if the lungs are involved or abdominal pain if the liver or spleen is affected[4].

Patient Characteristics

Demographics

• Age: ALK-negative ALCL is more prevalent in adults, particularly those aged 40 and older. It is less common in children and adolescents compared to the ALK-positive variant[5].
• Gender: There is a slight male predominance in the incidence of ALK-negative ALCL, although it can affect individuals of any gender[6].

Risk Factors

While the exact etiology of ALK-negative ALCL remains unclear, several risk factors have been identified:
- Immune System Status: Patients with compromised immune systems, such as those with HIV/AIDS or those undergoing immunosuppressive therapy, may have an increased risk of developing lymphomas, including ALCL[7].
- Environmental Exposures: Some studies suggest a potential link between exposure to certain chemicals or toxins and the development of lymphomas, although specific associations with ALK-negative ALCL are still under investigation[8].

Prognosis

The prognosis for patients with ALK-negative ALCL tends to be less favorable than for those with the ALK-positive variant. Factors influencing prognosis include the stage of the disease at diagnosis, the presence of B symptoms, and the patient's overall health and response to treatment[9].

Conclusion

Anaplastic large cell lymphoma, ALK-negative (ICD-10 code C84.7), presents with a range of clinical features, primarily lymphadenopathy and systemic symptoms. Understanding the signs, symptoms, and patient demographics is essential for healthcare providers to facilitate early diagnosis and appropriate management. Given the aggressive nature of this lymphoma subtype, timely intervention can significantly impact patient outcomes. Further research into the underlying mechanisms and risk factors associated with ALK-negative ALCL is warranted to improve treatment strategies and prognostic assessments.

Anaplastic large cell lymphoma (ALCL), specifically the ALK-negative subtype, is a type of non-Hodgkin lymphoma characterized by the presence of large, abnormal lymphocytes. The ICD-10 code for this condition is C84.7. Below are alternative names and related terms associated with this diagnosis.

Alternative Names

1. Anaplastic Large Cell Lymphoma (ALCL): This is the broader term that encompasses both ALK-positive and ALK-negative subtypes.
2. ALK-Negative Anaplastic Large Cell Lymphoma: This specifies the subtype that lacks the anaplastic lymphoma kinase (ALK) gene rearrangement.
3. Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL-NOS): ALK-negative ALCL is often classified under this broader category of peripheral T-cell lymphomas.
4. Systemic Anaplastic Large Cell Lymphoma: This term is used when the lymphoma is present in multiple areas of the body.
5. Cutaneous Anaplastic Large Cell Lymphoma: Refers to cases where the lymphoma manifests primarily in the skin.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): A general category of lymphomas that includes ALCL.
2. T-Cell Lymphoma: Since ALCL is a type of T-cell lymphoma, this term is often used in related discussions.
3. Lymphoproliferative Disorders: A broader category that includes various types of lymphomas and leukemias.
4. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.
5. C84.7: The specific ICD-10 code that identifies ALK-negative anaplastic large cell lymphoma.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C84.7 is crucial for accurate diagnosis, treatment planning, and communication among healthcare professionals. This knowledge aids in the classification and management of the disease, ensuring that patients receive appropriate care tailored to their specific lymphoma subtype.

Anaplastic large cell lymphoma (ALCL), specifically the ALK-negative subtype, is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The diagnosis of ALK-negative ALCL, which is classified under the ICD-10 code C84.7, involves several criteria that encompass clinical, histopathological, and immunophenotypic evaluations.

Clinical Criteria

1. Symptoms: Patients may present with various symptoms, including lymphadenopathy (swollen lymph nodes), fever, night sweats, and weight loss. These systemic symptoms are often referred to as "B symptoms" and can indicate a more aggressive disease.

2. Age and Demographics: ALK-negative ALCL typically occurs in older adults, with a median age of diagnosis around 50 years. It is more common in males than females, although it can occur in any demographic group.

Histopathological Criteria

1. Tissue Biopsy: A definitive diagnosis requires a biopsy of the affected lymph node or tissue. The histological examination reveals a proliferation of large atypical lymphoid cells, often with a characteristic "hallmark" cell appearance, which includes a horseshoe-shaped nucleus.

2. Immunohistochemistry: The biopsy sample is subjected to immunohistochemical staining to identify specific markers. In ALK-negative ALCL, the tumor cells typically express:
   - CD30 (a hallmark of ALCL)
   - CD45
   - Various T-cell markers (e.g., CD3, CD4, CD5)
   - Lack of ALK protein expression, which differentiates it from ALK-positive ALCL.

Molecular and Genetic Criteria

1. Genetic Testing: While ALK-positive ALCL is characterized by the presence of the ALK gene rearrangement, ALK-negative ALCL does not exhibit this alteration. Genetic testing may be performed to confirm the absence of ALK rearrangements.

2. Cytogenetic Analysis: Additional cytogenetic abnormalities may be identified, but these are not specific to ALK-negative ALCL and can vary among patients.

Differential Diagnosis

1. Exclusion of Other Lymphomas: It is crucial to differentiate ALK-negative ALCL from other types of lymphomas, such as peripheral T-cell lymphoma (PTCL) and Hodgkin lymphoma. This is achieved through a combination of clinical presentation, histological features, and immunophenotyping.

2. Clinical Staging: Staging of the disease using imaging studies (e.g., CT scans, PET scans) is also important to assess the extent of the disease and guide treatment decisions.

Conclusion

The diagnosis of ALK-negative anaplastic large cell lymphoma (ICD-10 code C84.7) is a multifaceted process that requires careful clinical evaluation, histopathological examination, and immunophenotypic characterization. Accurate diagnosis is essential for determining the appropriate treatment strategy and improving patient outcomes. If you suspect ALK-negative ALCL, it is crucial to consult with a healthcare professional who specializes in hematology or oncology for further evaluation and management.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a rare and aggressive form of non-Hodgkin lymphoma. The management of this condition involves a combination of chemotherapy, targeted therapy, and, in some cases, stem cell transplantation. Below is a detailed overview of the standard treatment approaches for ICD-10 code C84.7, which pertains to ALCL, ALK-negative.

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 particularly notable for its poor prognosis compared to its ALK-positive counterpart. The treatment strategy often depends on the stage of the disease, the patient's overall health, and specific clinical features.

Standard Treatment Approaches

1. Chemotherapy

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

• CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. It is often the first-line treatment for patients with advanced-stage disease.
• EPOCH Regimen: This is a more intensive regimen that includes Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin. It may be considered for patients with high-risk features or those who do not respond adequately to CHOP.

2. Targeted Therapy

Targeted therapies have emerged as a significant advancement in the treatment of various lymphomas, including ALCL. For ALK-negative ALCL, the following agents may be utilized:

• Brentuximab Vedotin (Adcetris): This is an antibody-drug conjugate that targets CD30, a marker expressed on ALCL cells. It is often used in relapsed or refractory cases and can be combined with chemotherapy for better outcomes[7].
• Other Investigational Agents: Ongoing clinical trials are exploring the efficacy of novel agents and combinations, including immune checkpoint inhibitors and other targeted therapies.

3. Stem Cell Transplantation

For patients with relapsed or refractory ALK-negative ALCL, autologous stem cell transplantation (ASCT) may be considered. This approach is typically reserved for younger patients or those who achieve a complete remission after initial therapy. The timing and eligibility for transplantation depend on the patient's response to initial treatments and overall health status.

4. Radiation Therapy

Radiation therapy may be employed in specific scenarios, particularly for localized disease or as a palliative measure to relieve symptoms. It is not typically the primary treatment modality for ALK-negative ALCL but can be beneficial in certain cases.

Conclusion

The treatment of ALK-negative anaplastic large cell lymphoma is multifaceted, involving chemotherapy, targeted therapies, and potentially stem cell transplantation. The choice of treatment is tailored to the individual patient based on disease characteristics and response to therapy. Ongoing research continues to explore new therapeutic options, aiming to improve outcomes for patients with this challenging lymphoma subtype. For the most current treatment protocols and clinical trials, healthcare providers should refer to the latest guidelines and literature in oncology.