ICD-10: C86.5

Angioimmunoblastic T-cell lymphoma (AITL), classified under ICD-10 code C86.5, is a rare and aggressive form of non-Hodgkin lymphoma. It is characterized by a proliferation of T-cells and is often associated with various clinical features, including autoimmune phenomena and systemic symptoms. Below are alternative names and related terms associated with this condition.

Alternative Names for Angioimmunoblastic T-cell Lymphoma

1. Angioimmunoblastic Lymphoma: This is a commonly used shorthand for AITL, emphasizing its immunological and vascular characteristics.
2. Angioimmunoblastic T-cell Lymphoma (AITL): The full name is often abbreviated to AITL in clinical and research contexts.
3. Immunoblastic Lymphoma: While this term can refer to various lymphomas, it is sometimes used interchangeably with AITL due to the immunoblastic features of the tumor cells.
4. Peripheral T-cell Lymphoma, Not Otherwise Specified (PTCL-NOS): AITL is classified under this broader category of T-cell lymphomas, which includes various subtypes that do not fit into more specific classifications.

Related Terms

1. T-cell Lymphoma: A general term for lymphomas that originate from T-cells, which includes AITL as a specific subtype.
2. Non-Hodgkin Lymphoma (NHL): AITL is a type of non-Hodgkin lymphoma, which encompasses a diverse group of blood cancers that include all lymphomas except Hodgkin's lymphoma.
3. Lymphoproliferative Disorders: This term refers to a group of conditions, including AITL, characterized by the excessive production of lymphocytes.
4. Autoimmune Lymphoproliferative Syndrome (ALPS): AITL can sometimes present with autoimmune features, linking it to conditions like ALPS, which involves abnormal lymphocyte proliferation.

Conclusion

Understanding the alternative names and related terms for Angioimmunoblastic T-cell lymphoma is crucial for accurate diagnosis and treatment. These terms reflect the complex nature of the disease and its classification within the broader spectrum of lymphomas. If you have further questions or need more specific information about AITL, feel free to ask!

Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive form of non-Hodgkin lymphoma characterized by the proliferation of T-cells, specifically CD4+ T-helper cells. This condition is classified under the ICD-10 code C86.5, which specifically denotes angioimmunoblastic T-cell lymphoma within the broader category of other specified types of T/NK-cell lymphomas.

Clinical Features

Symptoms

Patients with AITL often present with a variety of symptoms, which can include:

• Lymphadenopathy: Swelling of lymph nodes, particularly in the neck, armpits, or groin.
• Fever: Persistent or intermittent fevers that may be accompanied by night sweats.
• Weight Loss: Unintentional weight loss is common, often due to systemic illness.
• Skin Rash: Some patients may develop rashes or other skin lesions.
• Fatigue: A general feeling of tiredness or lack of energy is frequently reported.

Pathophysiology

AITL is associated with a complex interplay of immune dysregulation and genetic mutations. The lymphoma cells often express markers such as CD4, CD10, and PD-1, which can help in distinguishing AITL from other lymphomas. The disease is also linked to autoimmune disorders and may arise in the context of chronic inflammation or immune system activation.

Diagnosis

Diagnostic Criteria

The diagnosis of AITL typically involves:

• Histopathological Examination: A biopsy of affected lymph nodes or tissues is essential for confirming the diagnosis. Pathological examination reveals a polymorphous infiltrate of T-cells, often with a background of follicular dendritic cells and a prominent vascular component.
• Immunophenotyping: Flow cytometry is used to analyze the surface markers on the lymphoma cells, confirming the T-cell lineage and specific subtypes.
• Cytogenetic Analysis: Genetic studies may reveal chromosomal abnormalities that are characteristic of AITL, aiding in diagnosis and prognosis.

Staging

Staging of AITL follows the Ann Arbor classification, which assesses the extent of lymph node involvement and the presence of extranodal disease. This staging is crucial for determining the appropriate treatment approach.

Treatment Options

Therapeutic Approaches

The management of AITL typically involves:

• Chemotherapy: Combination chemotherapy regimens, such as CHOP (Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone), are commonly used. However, AITL may be less responsive to standard therapies compared to other lymphomas.
• Targeted Therapy: Newer agents, including monoclonal antibodies and immunotherapies, are being explored in clinical trials.
• Stem Cell Transplantation: In eligible patients, autologous or allogeneic stem cell transplantation may be considered, especially in cases of relapsed or refractory disease.

Prognosis

The prognosis for patients with AITL can vary significantly based on factors such as age, overall health, and response to initial treatment. Generally, AITL has a poorer prognosis compared to other types of non-Hodgkin lymphoma, with a median survival that can range from a few years to over a decade, depending on individual circumstances.

Conclusion

Angioimmunoblastic T-cell lymphoma (ICD-10 code C86.5) is a complex and challenging malignancy that requires a multidisciplinary approach for diagnosis and management. Understanding its clinical features, diagnostic criteria, and treatment options is essential for healthcare providers to optimize patient outcomes. Ongoing research into targeted therapies and novel treatment strategies continues to evolve, offering hope for improved management of this aggressive lymphoma.

Angioimmunoblastic T-cell lymphoma (AITL), classified under ICD-10 code C86.5, is a rare and aggressive form of peripheral T-cell lymphoma. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for timely diagnosis and management.

Clinical Presentation

AITL typically presents with a combination of systemic and localized symptoms. The clinical picture can vary significantly among patients, but several common features are often observed:

Systemic Symptoms

• Fever: Patients frequently experience unexplained fevers, which may be intermittent or persistent.
• Night Sweats: Profuse sweating during the night is a common complaint.
• Weight Loss: Unintentional weight loss is often noted, contributing to the overall decline in health.
• Fatigue: A general sense of tiredness and lack of energy is prevalent among affected individuals.

Lymphadenopathy

• Peripheral Lymphadenopathy: Patients typically present with enlarged lymph nodes, which can be found in various regions, including the neck, axilla, and groin. This lymphadenopathy is often painless and may be generalized or localized.

Skin Manifestations

• Rash: Some patients may develop a rash, which can vary in appearance and may be associated with pruritus (itching).
• Erythematous Lesions: These can occur and may resemble other dermatological conditions, complicating diagnosis.

Other Symptoms

• Splenomegaly: Enlargement of the spleen is common and can lead to abdominal discomfort or fullness.
• Hepatomegaly: Liver enlargement may also occur, contributing to abdominal symptoms.

Signs and Symptoms

The signs and symptoms of AITL can be categorized into general and specific manifestations:

General Signs

• Pallor: Due to anemia, patients may appear pale.
• Lymphadenopathy: As mentioned, this is a hallmark sign of AITL.
• Fever and Chills: These may be present, often indicating an underlying infection or inflammatory process.

Specific Symptoms

• Cytopenias: Blood tests may reveal low levels of red blood cells (anemia), white blood cells (leukopenia), or platelets (thrombocytopenia).
• Hypergammaglobulinemia: Elevated levels of immunoglobulins can be detected in laboratory tests, reflecting the immune dysregulation associated with AITL.

Patient Characteristics

AITL predominantly affects adults, with a higher incidence in individuals aged 60 years and older. However, it can occur in younger adults as well. The following characteristics are often observed in patients with AITL:

• Age: Most commonly diagnosed in older adults, particularly those over 60.
• Gender: There is a slight male predominance in the incidence of AITL.
• Immunocompromised Status: Patients with compromised immune systems, such as those with HIV/AIDS or those undergoing immunosuppressive therapy, may be at increased risk for developing AITL.
• History of Autoimmune Disorders: Some patients may have a history of autoimmune diseases, which can be associated with the development of lymphomas, including AITL.

Conclusion

Angioimmunoblastic T-cell lymphoma (ICD-10 code C86.5) presents with a range of systemic symptoms, lymphadenopathy, and specific signs that can aid in diagnosis. The condition primarily affects older adults and may be associated with immunocompromised states or autoimmune disorders. Early recognition of these clinical features is essential for effective management and treatment of AITL, highlighting the importance of thorough clinical evaluation in patients presenting with these symptoms.

Angioimmunoblastic T-cell lymphoma (AITL), classified under ICD-10 code C86.5, is a rare and aggressive form of non-Hodgkin lymphoma characterized by a specific set of clinical, histological, and immunophenotypic features. The diagnosis of AITL involves a combination of clinical evaluation, laboratory tests, and histopathological examination. Below are the key criteria used for diagnosing AITL:

Clinical Presentation

1. Symptoms: Patients often present with systemic symptoms such as fever, night sweats, and weight loss, commonly referred to as "B symptoms." Lymphadenopathy (swollen lymph nodes) is also a frequent finding, along with splenomegaly (enlarged spleen) and hepatomegaly (enlarged liver) in some cases[1].

2. Age and Demographics: AITL typically occurs in older adults, with a median age of diagnosis around 60 years. It is slightly more common in males than females[1].

Laboratory Findings

1. Blood Tests: Laboratory tests may reveal anemia, thrombocytopenia (low platelet count), and elevated lactate dehydrogenase (LDH) levels, which can indicate a more aggressive disease[1].

2. Immunophenotyping: Flow cytometry is used to analyze the surface markers on lymphocytes. AITL is characterized by the presence of CD4+ T-cells, often with co-expression of other markers such as CD10, CD5, and PD-1. The presence of these markers helps differentiate AITL from other types of lymphomas[1][2].

Histopathological Examination

1. Biopsy: A definitive diagnosis of AITL requires a biopsy of the affected lymph node or tissue. Histological examination typically shows a polymorphous infiltrate of T-cells, along with prominent follicular dendritic cells and a background of small B-cells[2].

2. Morphological Features: The histopathology may reveal a "starry sky" appearance due to the presence of tingible body macrophages, which are indicative of high levels of apoptosis and necrosis within the lymphoid tissue[2].

3. Genetic Studies: Molecular studies may identify specific genetic alterations associated with AITL, such as mutations in the TET2 gene, which can support the diagnosis[1].

Differential Diagnosis

It is crucial to differentiate AITL from other types of lymphomas, particularly peripheral T-cell lymphomas (PTCL) and other aggressive lymphoproliferative disorders. This differentiation is based on clinical presentation, immunophenotypic profile, and histological features[2].

Conclusion

The diagnosis of angioimmunoblastic T-cell lymphoma (ICD-10 code C86.5) is multifaceted, relying on a combination of clinical symptoms, laboratory findings, and histopathological analysis. Accurate diagnosis is essential for determining the appropriate treatment strategy and improving patient outcomes. If you suspect AITL, it is advisable to consult a hematologist or oncologist for further evaluation and management.

References

1. Billing and Coding: MolDX: Minimal Residual Disease.
2. CC-486 for angioimmunoblastic T-cell lymphoma.

Angioimmunoblastic T-cell lymphoma (AITL), classified under ICD-10 code C86.5, is a rare and aggressive form of peripheral T-cell lymphoma characterized by a complex clinical presentation and a poor prognosis. The treatment approaches for AITL typically involve a combination of chemotherapy, targeted therapies, and sometimes stem cell transplantation. Below is a detailed overview of the standard treatment strategies for AITL.

Chemotherapy

First-Line Treatment

The first-line treatment for AITL often includes combination chemotherapy regimens. The most commonly used regimens are:

• CHOP: This regimen consists of Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. CHOP is a standard treatment for various types of non-Hodgkin lymphoma and has been used in AITL with some success[1].
• EPOCH: This is a more intensive regimen that includes Etoposide, Prednisone, Oncovin (Vincristine), Cyclophosphamide, and Doxorubicin. EPOCH is sometimes preferred for patients with more aggressive disease[1].

Response Assessment

After the initial chemotherapy cycle, patients are typically assessed for their response to treatment through imaging studies and clinical evaluation. A complete response may lead to further consolidation therapy, while partial responders may require additional treatment options.

Targeted Therapies

Brentuximab Vedotin (Adcetris)

Brentuximab vedotin is an antibody-drug conjugate that targets CD30, a protein often expressed in certain lymphomas, including AITL. While primarily used for Hodgkin lymphoma and anaplastic large cell lymphoma, it may be considered in relapsed or refractory cases of AITL[2].

CC-486

CC-486, an oral formulation of azacitidine, is being investigated for its efficacy in treating AITL. Early studies suggest that it may improve outcomes in patients with relapsed or refractory disease, although more research is needed to establish its role in standard treatment protocols[3].

Stem Cell Transplantation

For patients who achieve a complete response to initial chemotherapy, autologous stem cell transplantation (ASCT) may be considered, especially in younger patients or those with high-risk features. ASCT can provide a chance for long-term remission, although the risks associated with the procedure must be carefully weighed against potential benefits[1].

Supportive Care and Management of Symptoms

Given the aggressive nature of AITL and its associated symptoms, supportive care is crucial. This may include:

• Management of Cytopenias: Patients often experience low blood cell counts due to the disease or treatment, necessitating transfusions or growth factor support.
• Infection Prophylaxis: Due to immunosuppression, prophylactic antibiotics and antifungals may be required to prevent infections.
• Symptom Management: Addressing symptoms such as fever, night sweats, and weight loss is essential for improving the quality of life.

Conclusion

The treatment of angioimmunoblastic T-cell lymphoma (ICD-10 code C86.5) involves a multifaceted approach that includes chemotherapy, targeted therapies, and potentially stem cell transplantation. Ongoing research continues to explore new treatment options and combinations to improve outcomes for patients with this challenging disease. As treatment protocols evolve, it is essential for patients to discuss their options with a hematologist or oncologist specializing in lymphomas to tailor the best approach for their individual circumstances.

For further information on clinical trials and emerging therapies, patients and healthcare providers can refer to resources such as the National Cancer Institute or specialized oncology centers.