ICD-10: C84.76

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma characterized by the presence of large, atypical lymphoid cells. The specific variant known as ALK-negative ALCL is defined by the absence of the anaplastic lymphoma kinase (ALK) gene rearrangement, which is a significant factor in the classification and prognosis of this disease. The ICD-10 code C84.76 specifically refers to ALCL that is localized in the intrapelvic lymph nodes.

Clinical Description of C84.76

Definition and Classification

C84.76 denotes Anaplastic large cell lymphoma, ALK-negative, intrapelvic lymph nodes. This classification falls under the broader category of mature T/NK-cell lymphomas, which are a group of lymphomas that arise from T-cells or natural killer (NK) cells. ALCL is notable for its aggressive nature and can present in various anatomical locations, including lymph nodes, skin, and other organs.

Pathophysiology

In ALK-negative ALCL, the absence of the ALK gene rearrangement distinguishes it from its ALK-positive counterpart. This variant is often associated with a poorer prognosis and is more common in older adults. The pathophysiology involves the proliferation of malignant T-cells that can infiltrate lymphatic tissues, leading to lymphadenopathy and systemic symptoms.

Symptoms

Patients with C84.76 may present with:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the pelvic region.
- B symptoms: These include fever, night sweats, and unexplained weight loss, which are common in lymphomas.
- Abdominal discomfort: Due to the involvement of intrapelvic lymph nodes, patients may experience pain or fullness in the abdomen.

Diagnosis

Diagnosis of ALCL, including the ALK-negative variant, typically involves:
- Histopathological examination: A biopsy of the affected lymph nodes is essential for confirming the diagnosis. The presence of characteristic large cells and a specific immunophenotype (CD30 positive, ALK negative) is indicative of ALCL.
- Imaging studies: CT scans or PET scans may be utilized to assess the extent of disease and involvement of lymph nodes.

Treatment

The treatment for C84.76 generally includes:
- Chemotherapy: Multi-agent chemotherapy regimens are commonly employed, often including drugs such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone).
- Targeted therapies: Research is ongoing into the efficacy of targeted therapies for ALK-negative ALCL, given its distinct molecular profile.
- Stem cell transplantation: In cases of relapsed or refractory disease, allogeneic hematopoietic cell transplantation may be considered.

Prognosis

The prognosis for patients with ALK-negative ALCL can vary significantly based on factors such as age, stage at diagnosis, and response to initial treatment. Generally, this subtype is associated with a more aggressive clinical course compared to ALK-positive ALCL.

Conclusion

ICD-10 code C84.76 encapsulates a specific and clinically significant variant of anaplastic large cell lymphoma, highlighting its unique characteristics and clinical implications. Understanding the nuances of this diagnosis is crucial for effective management and treatment planning. As research continues to evolve, new therapeutic strategies may emerge, potentially improving outcomes for patients diagnosed with this challenging lymphoma subtype.

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a type of non-Hodgkin lymphoma characterized by specific clinical presentations, signs, symptoms, and patient demographics. The ICD-10 code C84.76 specifically refers to ALCL that involves intrapelvic lymph nodes. Below is a detailed overview of the clinical aspects associated with this condition.

Clinical Presentation

General Characteristics

• Anaplastic Large Cell Lymphoma (ALK-negative): This subtype of ALCL is defined by the absence of the anaplastic lymphoma kinase (ALK) gene rearrangement, which differentiates it from the ALK-positive variant. ALK-negative ALCL is often more aggressive and has distinct clinical features.

Involvement of Intrapelvic Lymph Nodes

• Location: The designation of "intrapelvic lymph nodes" indicates that the lymphoma is localized within the pelvic region, affecting lymph nodes situated in this area, which can include nodes in the iliac, inguinal, and pelvic regions.

Signs and Symptoms

Common Symptoms

• Lymphadenopathy: Patients typically present with enlarged lymph nodes, which may be palpable in the pelvic region or elsewhere in the body.
• B Symptoms: These include systemic symptoms such as:
• Fever: Often intermittent and may be associated with night sweats.
• Weight Loss: Unintentional weight loss is common, often exceeding 10% of body weight over six months.
• Fatigue: Patients frequently report significant fatigue and malaise.

Localized Symptoms

• Pelvic Pain or Discomfort: Due to the involvement of intrapelvic lymph nodes, patients may experience localized pain or discomfort in the pelvic area.
• Urinary Symptoms: If the lymph nodes compress surrounding structures, symptoms such as urinary frequency or urgency may occur.
• Gastrointestinal Symptoms: Compression of the intestines can lead to symptoms like abdominal pain or changes in bowel habits.

Patient Characteristics

Demographics

• Age: ALK-negative ALCL can occur in adults of various ages, but it is more commonly diagnosed in young adults and middle-aged individuals, typically between the ages of 20 and 50.
• Gender: There is a slight male predominance in the incidence of ALCL, including the ALK-negative subtype.

Risk Factors

• Immunocompromised State: Patients with weakened immune systems, such as those with HIV/AIDS or those undergoing immunosuppressive therapy, may have an increased risk of developing ALCL.
• Previous Lymphoproliferative Disorders: A history of other lymphomas or hematological malignancies can predispose individuals to ALCL.

Conclusion

Anaplastic large cell lymphoma, ALK-negative, particularly when involving intrapelvic lymph nodes (ICD-10 code C84.76), presents with a range of clinical features that include lymphadenopathy, systemic B symptoms, and localized pelvic symptoms. Understanding these characteristics is crucial for timely diagnosis and management. If you suspect ALCL or have concerns regarding symptoms, it is essential to consult a healthcare professional for further evaluation and potential diagnostic testing.

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 C84.76 specifically refers to ALCL located in the intrapelvic 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 for the condition, emphasizing the absence of the anaplastic lymphoma kinase (ALK) gene rearrangement.
2. 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 into other specific subtypes.
3. Systemic Anaplastic Large Cell Lymphoma: This term may be used when the lymphoma is not localized and affects multiple lymph nodes or organs.

Related Terms

1. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes various types of lymphomas, including Hodgkin and non-Hodgkin lymphomas.
2. Non-Hodgkin Lymphoma (NHL): A category of lymphomas that includes ALCL and other types of lymphatic cancers that do not involve Reed-Sternberg cells, which are characteristic of Hodgkin lymphoma.
3. Intraperitoneal Lymphadenopathy: This term may be used to describe the presence of enlarged lymph nodes within the pelvic cavity, which can be associated with ALCL.
4. Lymph Node Involvement: Refers to the spread of lymphoma to lymph nodes, which is a common feature of many lymphomas, including ALCL.

Clinical Context

Anaplastic large cell lymphoma, particularly the ALK-negative variant, is known for its aggressive nature and can present with various symptoms, including lymphadenopathy, fever, and weight loss. The involvement of intrapelvic lymph nodes can complicate the clinical picture, necessitating careful diagnostic and therapeutic approaches.

Understanding these alternative names and related terms can aid in better communication among healthcare professionals and enhance the clarity of medical documentation and coding practices.

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 C84.76 (Anaplastic large cell lymphoma, ALK-negative, intrapelvic lymph nodes), involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria used for diagnosis:

Clinical Presentation

1. Symptoms: Patients may present with generalized symptoms such as fever, night sweats, and weight loss, commonly referred to as "B symptoms." Localized symptoms may arise depending on the lymph node involvement, such as abdominal pain or swelling if intrapelvic lymph nodes are affected[1].

2. Physical Examination: A thorough physical examination may reveal lymphadenopathy (swollen lymph nodes) in various regions, including the cervical, axillary, or inguinal areas, as well as abdominal or pelvic masses[1].

Imaging Studies

1. CT Scans: Computed tomography (CT) scans of the chest, abdomen, and pelvis are essential for assessing lymph node enlargement and the extent of disease. In the case of C84.76, specific attention is given to the intrapelvic lymph nodes[1].

2. PET Scans: Positron emission tomography (PET) scans can help evaluate metabolic activity in lymph nodes and other tissues, aiding in the assessment of disease spread and response to treatment[1].

Histopathological Examination

1. Biopsy: A definitive diagnosis of ALCL requires a biopsy of the affected lymph nodes. This can be done through excisional biopsy, core needle biopsy, or fine-needle aspiration, depending on the clinical scenario[1].

2. Microscopic Features: Pathological examination typically reveals large atypical lymphoid cells with a characteristic "hallmark" cell appearance. These cells are often pleomorphic and may exhibit a prominent nucleolus[1].

3. Immunophenotyping: Immunohistochemical staining is crucial for confirming the diagnosis. ALK-negative ALCL typically expresses CD30 and may express other markers such as CD4, but lacks ALK protein expression, which distinguishes it from ALK-positive variants[1].

4. Cytogenetic Analysis: While not always performed, cytogenetic studies can help identify specific chromosomal abnormalities associated with ALCL, although ALK-negative cases may not show the same translocations as ALK-positive cases[1].

Differential Diagnosis

It is essential to differentiate ALCL from other lymphomas and malignancies, such as peripheral T-cell lymphoma and other subtypes of non-Hodgkin lymphoma. This differentiation is based on clinical features, histological characteristics, and immunophenotyping results[1].

Conclusion

The diagnosis of C84.76: Anaplastic large cell lymphoma, ALK-negative, intrapelvic lymph nodes, relies on a comprehensive approach that includes clinical evaluation, imaging studies, and detailed histopathological analysis. Accurate diagnosis is critical for determining the appropriate treatment strategy and improving patient outcomes. If you have further questions or need more specific information, feel free to ask!

Anaplastic large cell lymphoma (ALCL), particularly the ALK-negative subtype, is a rare and aggressive form of non-Hodgkin lymphoma. The ICD-10 code C84.76 specifically refers to ALCL that presents in the intrapelvic 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. This form of lymphoma can manifest in various anatomical locations, including lymph nodes, skin, and other organs, with intrapelvic lymph node involvement being less common but significant.

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. CHOP is often the first-line treatment for aggressive lymphomas, including ALCL.
• EPOCH Regimen: Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin (EPOCH) is another regimen that may be used, particularly in cases where a more intensive approach is warranted.

2. Targeted Therapy

While traditional chemotherapy is effective, targeted therapies are increasingly being explored. For ALK-negative ALCL, the following agents may be considered:

• Brentuximab Vedotin: This is an antibody-drug conjugate that targets CD30, a marker commonly expressed in ALCL cells. It is particularly useful in relapsed or refractory cases and can be used in combination with chemotherapy.
• Checkpoint Inhibitors: Emerging therapies, such as PD-1 inhibitors, are being investigated in clinical trials for their efficacy in treating ALCL.

3. Radiation Therapy

Radiation therapy may be employed in specific scenarios, particularly for localized disease or as a consolidation treatment after chemotherapy. It can help manage symptoms and reduce tumor burden in the intrapelvic region.

4. Stem Cell Transplantation

For patients with relapsed or refractory ALK-negative ALCL, high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be considered. This approach aims to provide a curative option for patients who do not respond adequately to initial treatments.

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 often explore novel therapies and combinations that could improve outcomes.

Conclusion

The management of anaplastic large cell lymphoma, particularly the ALK-negative variant affecting intrapelvic lymph nodes, involves a multifaceted approach primarily centered on chemotherapy, with additional options including targeted therapies, radiation, and stem cell transplantation. Given the complexity and variability of this disease, treatment should be tailored to the individual patient, considering factors such as disease stage, overall 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 lymphoma subtype.