ICD-10: C84.63

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 C84.63 specifically refers to Anaplastic large cell lymphoma, ALK-positive, involving intra-abdominal lymph nodes. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Anaplastic Large Cell Lymphoma (ALCL)

Overview

Anaplastic large cell lymphoma is a subtype of peripheral T-cell lymphoma (PTCL) and is notable for its aggressive nature. It is classified into two main categories based on the presence of the anaplastic lymphoma kinase (ALK) gene rearrangement:

1. ALK-positive ALCL: This form is more common in younger patients and is associated with a better prognosis compared to its ALK-negative counterpart.
2. ALK-negative ALCL: Typically occurs in older adults and has a more variable prognosis.

Pathophysiology

The ALK gene rearrangement leads to the production of a fusion protein that promotes cell proliferation and survival, contributing to the malignant transformation of lymphocytes. The presence of this genetic alteration is a key diagnostic criterion and influences treatment decisions.

Clinical Presentation

Patients with ALK-positive ALCL may present with:

• Lymphadenopathy: Swollen lymph nodes, particularly in the cervical, axillary, or inguinal regions.
• Intra-abdominal involvement: This can manifest as abdominal pain, distension, or palpable masses due to lymph node enlargement in the abdominal cavity.
• B symptoms: These include fever, night sweats, and weight loss, which are common in many lymphomas.

Diagnosis

Diagnosis typically involves:

• Histopathological examination: A biopsy of affected lymph nodes is essential for confirming the diagnosis. The presence of large pleomorphic cells with a characteristic "hallmark" cell appearance is indicative of ALCL.
• Immunophenotyping: ALK-positive cells typically express CD30 and may express other T-cell markers.
• Cytogenetic analysis: Detection of the ALK gene rearrangement through fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) techniques.

Staging

Staging of ALCL is crucial for treatment planning and involves imaging studies such as CT scans or PET scans to assess the extent of disease, particularly in the abdomen and other regions.

Treatment Options

The treatment for ALK-positive ALCL often includes:

• Chemotherapy: The most common regimen includes CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or variations thereof, sometimes combined with targeted therapies.
• Targeted therapy: Agents such as crizotinib, which targets the ALK protein, may be used in relapsed or refractory cases.
• Radiation therapy: This may be employed in localized disease or as a consolidation treatment after chemotherapy.

Prognosis

The prognosis for patients with ALK-positive ALCL is generally favorable, especially when diagnosed early and treated appropriately. The overall survival rates are higher compared to ALK-negative ALCL, but outcomes can vary based on factors such as age, stage at diagnosis, and response to initial therapy.

Conclusion

ICD-10 code C84.63 captures the specific diagnosis of Anaplastic large cell lymphoma, ALK-positive, involving intra-abdominal lymph nodes. Understanding the clinical features, diagnostic criteria, and treatment options is essential for effective management of this aggressive lymphoma subtype. Early diagnosis and tailored treatment strategies are critical for improving patient outcomes in ALCL.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive subtype, is a rare and aggressive form of non-Hodgkin lymphoma. The ICD-10 code C84.63 specifically refers to ALCL that presents 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

Overview of Anaplastic Large Cell Lymphoma

ALCL is characterized by the proliferation of large, atypical lymphoid cells that express the anaplastic lymphoma kinase (ALK) protein due to chromosomal translocations, most commonly t(2;5)(p23;q35). This subtype is more prevalent in younger individuals and is known for its aggressive behavior and potential for extranodal involvement, including the gastrointestinal tract and abdominal lymph nodes[1].

Signs and Symptoms

Patients with ALK-positive ALCL involving intra-abdominal lymph nodes may present with a variety of signs and symptoms, which can include:

• Lymphadenopathy: Swelling of lymph nodes, particularly in the abdomen, which may be palpable during physical examination.
• Abdominal Pain: Patients often report discomfort or pain in the abdominal region due to lymph node enlargement or associated organ involvement.
• B Symptoms: These systemic symptoms include fever, night sweats, and unexplained weight loss, which are common in lymphomas and indicate a more aggressive disease process[2].
• Gastrointestinal Symptoms: Depending on the extent of lymph node involvement, patients may experience nausea, vomiting, or changes in bowel habits due to compression of the gastrointestinal tract[3].
• Fatigue: A common symptom in many cancer patients, fatigue can be significant and debilitating in those with ALCL.

Patient Characteristics

Certain demographic and clinical characteristics are often associated with ALK-positive ALCL:

• Age: This lymphoma subtype predominantly affects younger individuals, typically those in their late teens to early 30s, although it can occur in older adults as well[4].
• Gender: There is a slight male predominance in cases of ALCL, including the ALK-positive variant[5].
• Ethnicity: Some studies suggest variations in incidence based on ethnicity, with higher rates observed in certain populations, although more research is needed to clarify these trends[6].
• Immunocompromised Status: Patients with compromised immune systems, such as those with HIV/AIDS or those on immunosuppressive therapy, may have an increased risk of developing lymphomas, including ALCL[7].

Conclusion

Anaplastic large cell lymphoma, ALK-positive, with intra-abdominal lymph node involvement presents with a range of symptoms that can significantly impact a patient's quality of life. Early recognition of the clinical signs, such as lymphadenopathy and systemic B symptoms, is essential for prompt diagnosis and treatment. Understanding the typical patient characteristics can aid healthcare providers in identifying at-risk individuals and implementing appropriate diagnostic strategies. Given the aggressive nature of this lymphoma, timely intervention is critical to improve patient outcomes.

References

1. Anaplastic Large Cell Lymphoma Overview.
2. B Symptoms in Lymphoma.
3. Gastrointestinal Symptoms in Lymphoma Patients.
4. Age Distribution in Anaplastic Large Cell Lymphoma.
5. Gender Differences in Lymphoma Incidence.
6. Ethnic Variations in Lymphoma Incidence.
7. Immunocompromised Patients and Lymphoma Risk.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by the presence of anaplastic large cells and the expression of the anaplastic lymphoma kinase (ALK) gene. The ICD-10 code C84.63 specifically refers to ALK-positive anaplastic large cell lymphoma located in the intra-abdominal lymph nodes. Below are alternative names and related terms associated with this condition.

Alternative Names for C84.63

1. Anaplastic Large Cell Lymphoma (ALK-positive): This is the most direct alternative name, emphasizing the ALK-positive status of the lymphoma.
2. ALK-positive ALCL: A shorthand version that highlights the significance of the ALK gene in the pathology of the lymphoma.
3. Systemic Anaplastic Large Cell Lymphoma: This term may be used when referring to cases that involve multiple lymph nodes or systemic involvement, including intra-abdominal nodes.
4. Anaplastic Lymphoma: A broader term that can refer to various forms of anaplastic lymphomas, including ALK-positive and ALK-negative types.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): ALCL is classified under the broader category of non-Hodgkin lymphomas, which encompasses a variety of lymphoid malignancies.
2. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes ALCL.
3. ALK Gene: Refers to the anaplastic lymphoma kinase gene, which is often involved in the pathogenesis of ALK-positive anaplastic large cell lymphoma.
4. Intra-abdominal Lymph Nodes: This term specifies the anatomical location of the lymphoma, indicating that the affected lymph nodes are located within the abdominal cavity.
5. Peripheral T-cell Lymphoma (PTCL): ALCL is a subtype of PTCL, which includes various lymphomas derived from T-cells.

Clinical Context

Understanding these alternative names and related terms is crucial for healthcare professionals involved in the diagnosis, treatment, and coding of this specific lymphoma type. Accurate coding and terminology ensure proper communication among medical professionals and facilitate appropriate treatment plans for patients diagnosed with C84.63.

In summary, the ICD-10 code C84.63 for anaplastic large cell lymphoma, ALK-positive, intra-abdominal lymph nodes, is associated with various alternative names and related terms that reflect its classification, genetic characteristics, and anatomical location.

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 diagnosis of ALCL, specifically coded as ICD-10 C84.63 for ALK-positive cases involving intra-abdominal lymph nodes, involves a combination of clinical, histopathological, and immunophenotypic criteria. Below is a detailed overview of the diagnostic criteria used for this condition.

Clinical Presentation

1. Symptoms: Patients may present with nonspecific symptoms such as:
   - Lymphadenopathy (swelling of lymph nodes)
   - Fever
   - Night sweats
   - Weight loss
   - Abdominal pain or discomfort, particularly if intra-abdominal lymph nodes are involved.

2. Physical Examination: A thorough physical examination may reveal enlarged lymph nodes, hepatosplenomegaly (enlargement of the liver and spleen), or other signs of systemic illness.

Diagnostic Imaging

1. Imaging Studies: Imaging techniques such as CT scans or PET scans are essential for assessing the extent of lymphadenopathy and identifying intra-abdominal lymph nodes. These studies help visualize the size and location of lymph nodes and any associated masses.

Histopathological Examination

1. Biopsy: A definitive diagnosis of ALCL requires a biopsy of the affected lymph node or tissue. The biopsy can be performed via:
   - Excisional biopsy
   - Core needle biopsy
   - Fine needle aspiration (FNA), although this may be less definitive.

2. Histological Features: The histopathological examination typically reveals:
   - A predominance of large atypical lymphoid cells.
   - A characteristic "starry sky" appearance due to the presence of macrophages.
   - A mixed inflammatory background.

Immunophenotyping

1. Immunohistochemistry: The diagnosis is confirmed through immunophenotyping, which involves staining the biopsy sample with specific antibodies. Key markers include:
   - ALK Protein: Positive staining for ALK (usually detected using the D5F3 antibody) is a hallmark of ALK-positive ALCL.
   - CD30: Strong expression of CD30 is also a characteristic feature of ALCL.
   - Other Markers: The tumor cells may also express CD3, CD4, and CD8, but typically lack B-cell markers (e.g., CD19, CD20).

Molecular Studies

1. Genetic Testing: The presence of ALK gene rearrangements can be confirmed through molecular techniques such as:
   - Fluorescence in situ hybridization (FISH)
   - Reverse transcription polymerase chain reaction (RT-PCR)

These tests help confirm the diagnosis and differentiate ALK-positive ALCL from other lymphomas.

Conclusion

The diagnosis of Anaplastic Large Cell Lymphoma, ALK-positive, involving intra-abdominal lymph nodes (ICD-10 code C84.63) is a multifaceted process that combines clinical evaluation, imaging studies, histopathological examination, immunophenotyping, and molecular testing. Each of these components plays a crucial role in establishing an accurate diagnosis, guiding treatment decisions, and predicting patient outcomes. If you suspect ALCL or have further questions about the diagnostic process, consulting a hematologist or oncologist is advisable for personalized assessment and management.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive subtype, is a rare and aggressive form of non-Hodgkin lymphoma characterized by the presence of the anaplastic lymphoma kinase (ALK) gene rearrangement. The ICD-10 code C84.63 specifically refers to ALCL affecting 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-positive)

Characteristics

ALK-positive ALCL typically occurs in younger individuals and is associated with a better prognosis compared to ALK-negative forms. The disease often presents with lymphadenopathy, which can be localized or disseminated, and may involve extranodal sites, including the skin, lungs, and abdominal lymph nodes[1].

Standard Treatment Approaches

1. Chemotherapy

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. It is a standard treatment for various types of non-Hodgkin lymphoma and has been effective in treating ALCL[2].

• CHOP-like Regimens: Variations of the CHOP regimen, such as EPOCH (Etoposide, Prednisone, Oncovin, Cyclophosphamide, and Doxorubicin), may also be employed, particularly in cases with high tumor burden or aggressive disease[3].

• Escalated BEACOPP: In some cases, a more intensive regimen like BEACOPP (Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, and Prednisone) may be considered, especially for patients with advanced disease[4].

2. Targeted Therapy

Targeted therapies have emerged as a significant advancement in the treatment of ALK-positive ALCL:

• Crizotinib: This ALK inhibitor has shown efficacy in treating ALK-positive lymphomas. It is particularly beneficial for patients who have relapsed or are refractory to standard chemotherapy[5].

• Brentuximab Vedotin: This antibody-drug conjugate targets CD30, a marker expressed in ALCL cells. It is often used in relapsed or refractory cases and can be combined with chemotherapy for improved outcomes[6].

3. Radiation Therapy

Radiation therapy may be utilized in specific scenarios, particularly for localized disease or as a consolidation treatment after chemotherapy. It is less commonly used as a primary treatment modality but can be effective in managing localized lymph node involvement[7].

4. Stem Cell Transplantation

For patients with high-risk disease or those who do not achieve complete remission with initial therapy, autologous stem cell transplantation (ASCT) may be considered. This approach is typically reserved for relapsed cases or those with significant disease burden at diagnosis[8].

Monitoring and Follow-Up

Post-treatment monitoring is essential to assess for disease recurrence and manage any long-term effects of therapy. Regular follow-up appointments, imaging studies, and laboratory tests are critical components of ongoing care for patients with ALK-positive ALCL.

Conclusion

The treatment of ALK-positive anaplastic large cell lymphoma, particularly when involving intra-abdominal lymph nodes, involves a multi-faceted 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 age, and overall health. Ongoing research continues to refine these treatment strategies, aiming to improve outcomes for patients diagnosed with this aggressive lymphoma subtype.

For further information or specific case management, consulting with a hematologist or oncologist specializing in lymphomas is recommended.