ICD-10: C84.67

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The specific subtype known as ALK-positive anaplastic large cell lymphoma is defined by the presence of the anaplastic lymphoma kinase (ALK) gene rearrangement, which is a critical factor in the pathogenesis of this disease. Below is a detailed clinical description and relevant information regarding ICD-10 code C84.67, which pertains to ALK-positive anaplastic large cell lymphoma affecting the spleen.

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

Definition and Characteristics

• Anaplastic Large Cell Lymphoma (ALCL): This is a subtype of peripheral T-cell lymphoma that is aggressive and can present in various forms, including systemic and localized disease. ALK-positive ALCL is particularly noted for its association with the ALK gene rearrangement, which leads to the production of a fusion protein that promotes cell proliferation and survival.
• ALK Gene Rearrangement: The presence of the ALK gene rearrangement is a hallmark of this lymphoma subtype, typically found in younger patients. It is associated with a better prognosis compared to ALK-negative forms of the disease.

Symptoms

Patients with ALK-positive ALCL may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, which may be painless.
- Splenomegaly: Enlargement of the spleen, which can lead to discomfort or a feeling of fullness.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many lymphomas.
- Skin Lesions: Some patients may develop skin manifestations, such as nodules or plaques.

Diagnosis

Diagnosis typically involves:
- Histopathological Examination: A biopsy of affected tissue (e.g., lymph nodes, spleen) is essential for diagnosis. The presence of large, atypical lymphoid cells and the characteristic "hallmark" cells are indicative of ALCL.
- Immunophenotyping: Flow cytometry or immunohistochemistry is used to confirm the presence of ALK protein and to differentiate ALCL from other lymphomas.
- Cytogenetic Analysis: Detection of the ALK gene rearrangement can be performed using fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR).

Treatment

Treatment options for ALK-positive ALCL may include:
- Chemotherapy: Multi-agent chemotherapy regimens are commonly used, often including drugs such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or more intensive regimens.
- Targeted Therapy: ALK inhibitors, such as crizotinib, may be used in cases of relapsed or refractory disease.
- Stem Cell Transplantation: In eligible patients, autologous stem cell transplantation may be considered after achieving remission.

ICD-10 Code C84.67

• Code Description: The ICD-10 code C84.67 specifically refers to "Anaplastic large cell lymphoma, ALK-positive, spleen." This classification indicates that the lymphoma is localized to the spleen and is characterized by the presence of the ALK gene rearrangement.
• Clinical Significance: Accurate coding is essential for treatment planning, epidemiological studies, and insurance reimbursement. The designation of ALK-positive status is crucial for determining the most effective therapeutic strategies.

Conclusion

Anaplastic large cell lymphoma, ALK-positive, affecting the spleen, is a distinct and aggressive form of lymphoma that requires prompt diagnosis and treatment. Understanding the clinical features, diagnostic criteria, and treatment options is vital for healthcare providers managing patients with this condition. The ICD-10 code C84.67 serves as an important tool for categorizing this specific lymphoma subtype, facilitating appropriate clinical management and research efforts.

Anaplastic Large Cell Lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by specific clinical presentations, signs, symptoms, and patient demographics. The ICD-10 code C84.67 specifically refers to ALCL that is localized in the spleen. Below is a detailed overview of the clinical aspects associated with this condition.

Clinical Presentation

Overview of ALCL

Anaplastic Large Cell Lymphoma is a subtype of mature T/NK-cell lymphomas, which are aggressive lymphoproliferative disorders. The ALK-positive variant is associated with a chromosomal translocation involving the anaplastic lymphoma kinase (ALK) gene, leading to the production of a fusion protein that promotes tumor growth[1][9].

Signs and Symptoms

Patients with ALCL, particularly those with ALK-positive disease affecting the spleen, may present with a variety of symptoms, including:

• Lymphadenopathy: Swelling of lymph nodes is common, particularly in the cervical, axillary, or inguinal regions.
• Splenomegaly: Enlargement of the spleen is a hallmark of this condition, which may lead to abdominal discomfort or pain[1].
• Fever: Patients often experience unexplained fevers, which can be intermittent.
• Night Sweats: Profuse sweating during the night is frequently reported.
• Weight Loss: Unintentional weight loss is a common symptom, often due to the systemic effects of the lymphoma.
• Fatigue: A general sense of tiredness or lack of energy is prevalent among patients.
• Pruritus: Some patients may experience itching without a rash, which can be distressing[1][9].

Patient Characteristics

The demographic profile of patients with ALK-positive ALCL typically includes:

• Age: ALCL is more common in younger adults, particularly those in their 20s to 30s, although it can occur at any age[1].
• Gender: There is a slight male predominance in the incidence of ALCL.
• Ethnicity: The disease can affect individuals of any ethnicity, but certain subtypes may have varying prevalence across different populations[1][9].

Diagnosis and Evaluation

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

• Imaging: CT scans or PET scans are often used to assess lymphadenopathy and splenic involvement.
• Biopsy: A tissue biopsy of the affected lymph node or spleen is essential for histological confirmation. The presence of characteristic large anaplastic cells and immunohistochemical staining for ALK is critical for diagnosis[1][9].
• Laboratory Tests: Blood tests may reveal abnormalities such as elevated lactate dehydrogenase (LDH) levels, which can indicate tumor burden.

Conclusion

Anaplastic Large Cell Lymphoma, ALK-positive, affecting the spleen (ICD-10 code C84.67) presents with a range of symptoms including lymphadenopathy, splenomegaly, fever, and weight loss. It predominantly affects younger adults and shows a slight male predominance. Early diagnosis through imaging and biopsy is crucial for effective management and treatment of this aggressive lymphoma. Understanding the clinical presentation and patient characteristics is essential for healthcare providers in recognizing and addressing this condition promptly.

For further information or specific case studies, consulting hematology-oncology literature or clinical guidelines may provide additional insights into management strategies and outcomes for patients with ALCL.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by the presence of anaplastic lymphoma kinase (ALK) gene rearrangements. The ICD-10 code C84.67 specifically refers to ALCL that is localized to the spleen. Here are some alternative names and related terms associated with this condition:

Alternative Names

1. Anaplastic Large Cell Lymphoma, ALK-Positive: This is the primary designation for the condition, emphasizing the ALK positivity.
2. ALK-Positive Anaplastic Lymphoma: A variation that highlights the ALK gene's role in the disease.
3. Systemic Anaplastic Large Cell Lymphoma: While this term generally refers to ALCL that is not confined to a specific site, it can sometimes be used in broader discussions about the disease.
4. Anaplastic Lymphoma: A more general term that may refer to various forms of anaplastic lymphoma, 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 Rearrangement: Refers to the genetic alteration that is a hallmark of ALK-positive anaplastic large cell lymphoma.
4. Spleen Lymphoma: This term may be used to describe lymphomas that primarily affect the spleen, including ALCL.
5. Peripheral T-cell Lymphoma (PTCL): ALCL is classified as a subtype of PTCL, which includes various types of 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 terminology ensures proper communication among medical teams and aids in the effective management of patient care.

In summary, the ICD-10 code C84.67 for Anaplastic large cell lymphoma, ALK-positive, spleen, is associated with various alternative names and related terms that reflect its classification, genetic characteristics, and clinical implications.

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) protein. The diagnosis of ALK-positive ALCL, specifically when it involves the spleen, is based on a combination of clinical, histopathological, and immunophenotypic criteria.

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

1. Clinical Presentation

• Patients often present with lymphadenopathy, splenomegaly, and systemic symptoms such as fever, night sweats, and weight loss. The involvement of the spleen may be noted during physical examination or imaging studies.

2. Histopathological Examination

• Tissue Biopsy: A biopsy of the affected lymph node or spleen is essential. The histological examination typically reveals a polymorphous infiltrate of large atypical lymphoid cells.
• Cell Morphology: The presence of large pleomorphic cells with abundant cytoplasm and prominent nucleoli is characteristic. These cells may also exhibit a "starry sky" appearance due to the presence of macrophages.

3. Immunophenotyping

• Immunohistochemistry: The diagnosis is confirmed through immunohistochemical staining. ALK-positive ALCL cells typically express:
  • CD30 (a hallmark of ALCL)
  • CD45
  • EMA (epithelial membrane antigen)
  • ALK protein (detected using specific antibodies)
• Negative Markers: The absence of markers such as CD3, CD5, and CD23 helps differentiate ALCL from other lymphomas.

4. Cytogenetic and Molecular Studies

• Genetic Testing: The presence of the ALK gene rearrangement, most commonly the t(2;5)(p23;q35) translocation, is a critical diagnostic criterion. This can be identified through fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) techniques.

5. Imaging Studies

• Imaging modalities such as CT scans or PET scans may be utilized to assess the extent of disease, including splenic involvement and any other lymphatic or extranodal sites.

6. Exclusion of Other Conditions

• It is important to rule out other types of lymphomas and reactive lymphoid hyperplasia through differential diagnosis, ensuring that the clinical and laboratory findings align with ALK-positive ALCL.

Conclusion

The diagnosis of ICD-10 code C84.67, which refers to Anaplastic large cell lymphoma, ALK-positive, involving the spleen, relies on a comprehensive approach that includes clinical evaluation, histopathological analysis, immunophenotyping, and genetic testing. Accurate diagnosis is crucial for determining the appropriate treatment strategy and prognosis for patients with this aggressive lymphoma subtype.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-positive variant, is a type of non-Hodgkin lymphoma characterized by the presence of the anaplastic lymphoma kinase (ALK) gene rearrangement. The ICD-10 code C84.67 specifically refers to ALCL localized in the spleen. Treatment approaches for this condition typically involve a combination of chemotherapy, targeted therapy, and, in some cases, stem cell transplantation. Below is a detailed overview of the standard treatment strategies for ALK-positive ALCL.

Standard Treatment Approaches

1. Chemotherapy

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

• CHOP Regimen: This includes Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. It is often the first-line treatment for aggressive lymphomas, including ALCL.
• 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 poor prognostic factors.

2. Targeted Therapy

Targeted therapies have significantly improved outcomes for patients with ALK-positive ALCL:

• Brentuximab Vedotin (Adcetris): This is an antibody-drug conjugate that targets CD30, a marker expressed on ALCL cells. It is often used in combination with chemotherapy or as a single agent in relapsed or refractory cases. Studies have shown that it can lead to high response rates and improved survival outcomes[1][2].

3. Stem Cell Transplantation

For patients with high-risk disease or those who do not achieve a complete response to initial therapy, stem cell transplantation may be considered:

• Autologous Stem Cell Transplant (ASCT): This is often recommended for patients who have achieved a complete or partial response after first-line therapy. It involves harvesting the patient's own stem cells, followed by high-dose chemotherapy to eradicate the lymphoma before reinfusing the stem cells.
• Allogeneic Stem Cell Transplant: This may be considered in cases of relapse or refractory disease, particularly in younger patients with a suitable donor.

4. Radiation Therapy

While not a primary treatment modality for systemic ALCL, radiation therapy may be used in specific situations:

• Localized Disease: If the lymphoma is confined to the spleen and there is no evidence of systemic involvement, radiation therapy may be considered as part of the treatment plan.
• Palliative Care: Radiation can also be utilized to relieve symptoms in cases of bulky disease or localized pain.

5. Clinical Trials

Participation in clinical trials may be an option for patients, especially those with relapsed or refractory disease. New therapies, including novel agents and combinations, are continually being investigated to improve outcomes for ALCL patients.

Conclusion

The treatment of ALK-positive anaplastic large cell lymphoma, particularly when localized to the spleen, involves a multidisciplinary approach that includes chemotherapy, targeted therapies like Brentuximab Vedotin, and potentially 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 shape the landscape of treatment options, offering hope for improved outcomes in this challenging malignancy.

For the most current treatment guidelines and recommendations, consulting with a hematologist or oncologist specializing in lymphomas is essential, as they can provide personalized treatment plans based on the latest evidence and clinical practices.