ICD-10: C81.38
Lymphocyte depleted Hodgkin lymphoma, lymph nodes of multiple sites
Additional Information
Description
Lymphocyte Depleted Hodgkin Lymphoma (LDHL) is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. This specific condition is classified under the ICD-10 code C81.38, which denotes "Lymphocyte depleted Hodgkin lymphoma, lymph nodes of multiple sites." Below is a detailed clinical description and relevant information regarding this diagnosis.
Clinical Description of Lymphocyte Depleted Hodgkin Lymphoma
Pathophysiology
Lymphocyte Depleted Hodgkin Lymphoma is marked by the presence of large, atypical Reed-Sternberg cells, which are derived from B lymphocytes. In this subtype, there is a notable reduction in the number of normal lymphocytes, leading to a characteristic histological appearance. The disease often presents with a more aggressive clinical course compared to other types of Hodgkin lymphoma, such as mixed cellularity or nodular sclerosis.
Epidemiology
LDHL is relatively uncommon, accounting for approximately 1% to 2% of all Hodgkin lymphoma cases. It is more frequently diagnosed in older adults and is often associated with immunocompromised states, such as HIV infection or post-transplantation scenarios.
Clinical Presentation
Patients with LDHL may present with:
- Lymphadenopathy: Swollen lymph nodes, often in multiple regions, including cervical, axillary, and mediastinal areas.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement.
- Splenomegaly: Enlargement of the spleen may occur, contributing to abdominal discomfort or fullness.
- Fatigue: Generalized fatigue is common due to the systemic nature of the disease.
Diagnosis
Diagnosis of LDHL typically involves:
- Histopathological Examination: A biopsy of affected lymph nodes is essential for confirming the presence of Reed-Sternberg cells and the characteristic lymphocyte depletion.
- Imaging Studies: CT scans or PET scans are utilized to assess the extent of lymphadenopathy and to evaluate for any extranodal involvement.
- Laboratory Tests: Blood tests may reveal anemia or other abnormalities associated with the disease.
Staging
Staging of Hodgkin lymphoma, including LDHL, follows the Ann Arbor classification system, which considers the number of affected lymph node regions and the presence of systemic symptoms. The stage at diagnosis is crucial for determining the appropriate treatment approach.
Treatment Options
The treatment for Lymphocyte Depleted Hodgkin Lymphoma typically involves:
- Chemotherapy: Multi-agent chemotherapy regimens, such as ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine), are commonly used. However, due to the aggressive nature of LDHL, more intensive regimens may be considered.
- Radiation Therapy: In some cases, radiation therapy may be employed, particularly if there is localized disease or residual mass post-chemotherapy.
- Stem Cell Transplantation: For relapsed or refractory cases, autologous or allogeneic stem cell transplantation may be indicated.
Prognosis
The prognosis for patients with Lymphocyte Depleted Hodgkin Lymphoma tends to be less favorable compared to other subtypes of Hodgkin lymphoma. Factors influencing prognosis include the stage at diagnosis, the patient's age, and overall health status.
Conclusion
ICD-10 code C81.38 encapsulates the diagnosis of Lymphocyte Depleted Hodgkin Lymphoma affecting multiple lymph node sites. Understanding the clinical features, diagnostic criteria, and treatment options is essential for healthcare providers managing patients with this rare and aggressive form of lymphoma. Early diagnosis and appropriate treatment are critical for improving patient outcomes.
Diagnostic Criteria
Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.38, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. The diagnosis of LDHL involves several criteria, which are essential for accurate identification and classification of the disease. Below are the key diagnostic criteria and considerations:
Diagnostic Criteria for Lymphocyte Depleted Hodgkin Lymphoma
1. Clinical Presentation
- Symptoms: Patients may present with symptoms such as fever, night sweats, weight loss, and pruritus. Lymphadenopathy is often noted, particularly in multiple lymph node regions.
- Physical Examination: A thorough physical examination is crucial to identify enlarged lymph nodes, splenomegaly, or hepatomegaly.
2. Histopathological Examination
- Biopsy: A lymph node biopsy is essential for diagnosis. The histological examination should reveal the presence of Reed-Sternberg cells, which are large, atypical cells that are characteristic of Hodgkin lymphoma.
- Lymphocyte Depletion: In LDHL, there is a marked reduction in the number of lymphocytes surrounding the Reed-Sternberg cells, which distinguishes it from other subtypes of Hodgkin lymphoma.
3. Immunophenotyping
- Immunohistochemistry: The use of immunohistochemical stains is critical to confirm the diagnosis. Reed-Sternberg cells typically express CD30 and CD15, while they lack B-cell markers (e.g., CD20).
- Flow Cytometry: This technique can be used to analyze the cell populations in the biopsy sample, further supporting the diagnosis.
4. Staging and Imaging Studies
- Imaging: CT scans or PET scans are often employed to assess the extent of disease involvement in lymph nodes and other organs. LDHL can present with multiple sites of lymph node involvement.
- Staging: The Ann Arbor staging system is commonly used to classify the extent of Hodgkin lymphoma, which is crucial for treatment planning.
5. Exclusion of Other Conditions
- Differential Diagnosis: It is important to rule out other lymphoproliferative disorders and non-Hodgkin lymphomas that may present similarly. This may involve additional laboratory tests and imaging studies.
6. Laboratory Tests
- Blood Tests: Complete blood counts (CBC) may show anemia or thrombocytopenia, which can be associated with LDHL. Other tests may include liver function tests and lactate dehydrogenase (LDH) levels, which can indicate tumor burden.
Conclusion
The diagnosis of lymphocyte depleted Hodgkin lymphoma (ICD-10 code C81.38) relies on a combination of clinical evaluation, histopathological findings, immunophenotyping, imaging studies, and the exclusion of other conditions. Given the rarity of this subtype, a multidisciplinary approach involving oncologists, pathologists, and radiologists is often necessary to ensure accurate diagnosis and appropriate management. If you have further questions or need more specific information, feel free to ask!
Clinical Information
Lymphocyte Depleted Hodgkin Lymphoma (LDHL) is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. This condition is classified under the ICD-10 code C81.38, which specifically refers to lymphocyte depleted Hodgkin lymphoma affecting lymph nodes at multiple sites. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with this condition.
Clinical Presentation
Overview of Lymphocyte Depleted Hodgkin Lymphoma
LDHL is known for its aggressive nature and is often diagnosed at an advanced stage. It typically presents with systemic symptoms and lymphadenopathy, which can be widespread. The disease is more common in older adults and is associated with a poorer prognosis compared to other subtypes of Hodgkin lymphoma.
Signs and Symptoms
Patients with LDHL may exhibit a variety of signs and symptoms, which can be categorized as follows:
1. Lymphadenopathy
- Peripheral Lymph Nodes: Patients often present with enlarged lymph nodes, which may be palpable in the neck, axilla, or groin.
- Mediastinal Lymph Nodes: Enlargement of mediastinal lymph nodes can lead to respiratory symptoms due to compression of the airways.
2. Systemic Symptoms
- Fever: Patients may experience unexplained fevers, often referred to as "B symptoms."
- Night Sweats: Profuse sweating during the night is common.
- Weight Loss: Unintentional weight loss is frequently reported, contributing to the overall decline in health.
3. Other Symptoms
- Fatigue: A general sense of tiredness and lack of energy is prevalent among patients.
- Pruritus: Some patients may experience itching, which can be generalized or localized.
- Pain: Pain may occur in areas where lymph nodes are enlarged or affected.
Patient Characteristics
Demographics
- Age: LDHL is more commonly diagnosed in older adults, typically those over the age of 50, although it can occur at any age.
- Gender: There is a slight male predominance in the incidence of Hodgkin lymphoma, including the lymphocyte depleted subtype.
Risk Factors
- HIV Infection: There is a notable association between LDHL and HIV, as immunocompromised individuals are at a higher risk for developing this aggressive form of lymphoma.
- Previous Chemotherapy or Radiation: Patients with a history of treatment for other cancers may have an increased risk of developing LDHL.
Histological Features
- Reed-Sternberg Cells: The presence of these characteristic cells is a hallmark of Hodgkin lymphoma, and in LDHL, they are often found in a background of sparse lymphocytes.
- Fibrosis: There may be significant fibrotic changes in the lymph nodes, contributing to the depletion of lymphocytes.
Conclusion
Lymphocyte Depleted Hodgkin Lymphoma, classified under ICD-10 code C81.38, presents with distinct clinical features, including significant lymphadenopathy and systemic symptoms such as fever, night sweats, and weight loss. The patient demographic typically includes older adults, with a notable association with immunocompromised states, particularly HIV. Understanding these characteristics is crucial for timely diagnosis and management of this aggressive lymphoma subtype. Early intervention and appropriate treatment strategies are essential to improve patient outcomes.
Approximate Synonyms
Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.38, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. This specific type of lymphoma primarily affects lymph nodes in multiple sites throughout the body. Below are alternative names and related terms associated with this condition.
Alternative Names for Lymphocyte Depleted Hodgkin Lymphoma
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Lymphocyte Depleted Hodgkin's Disease: This term is often used interchangeably with lymphocyte depleted Hodgkin lymphoma, emphasizing the disease aspect.
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Hodgkin Lymphoma, Lymphocyte Depleted Type: This name highlights the specific subtype of Hodgkin lymphoma.
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LDHL: An acronym commonly used in medical literature and discussions to refer to lymphocyte depleted Hodgkin lymphoma.
Related Terms
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Hodgkin Lymphoma: A broader category that includes various subtypes, including lymphocyte depleted Hodgkin lymphoma.
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Reed-Sternberg Cells: The characteristic cells found in Hodgkin lymphoma, which are crucial for diagnosis.
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Non-Specific Lymphadenopathy: A term that may be used in clinical settings to describe swollen lymph nodes, which can be a symptom of LDHL.
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Advanced Stage Hodgkin Lymphoma: LDHL is often diagnosed at an advanced stage, so this term may be relevant in discussions about prognosis and treatment.
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B-cell Lymphoma: While LDHL is a specific type of Hodgkin lymphoma, it is sometimes discussed in the context of B-cell lymphomas due to the nature of the Reed-Sternberg cells.
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Lymphoma with Lymphocyte Depletion: A descriptive term that may be used in clinical documentation or discussions.
Conclusion
Understanding the alternative names and related terms for ICD-10 code C81.38 is essential for accurate diagnosis, treatment planning, and communication among healthcare professionals. Lymphocyte depleted Hodgkin lymphoma, while a specific subtype, is part of the broader category of Hodgkin lymphomas and is characterized by unique histological features and clinical presentations. If you need further information or specific details about treatment options or prognosis, feel free to ask!
Treatment Guidelines
Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.38, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. This subtype often presents with advanced disease and can be more aggressive than other forms of Hodgkin lymphoma. The treatment approaches for LDHL typically involve a combination of chemotherapy, radiation therapy, and, in some cases, stem cell transplantation. Below is a detailed overview of the standard treatment strategies.
Standard Treatment Approaches
1. Chemotherapy
Chemotherapy is the cornerstone of treatment for lymphocyte depleted Hodgkin lymphoma. The most commonly used regimens include:
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ABVD Regimen: This includes Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. While ABVD is the standard for many Hodgkin lymphoma subtypes, its effectiveness in LDHL may be limited due to the aggressive nature of this variant.
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BEACOPP Regimen: This regimen (Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, and Prednisone) is often considered for patients with advanced disease or those who do not respond adequately to ABVD. BEACOPP has shown improved outcomes in some studies for aggressive forms of Hodgkin lymphoma, including LDHL[1].
2. Radiation Therapy
Radiation therapy may be employed in conjunction with chemotherapy, particularly for localized disease or as consolidation therapy after chemotherapy. The use of involved-field radiation therapy (IFRT) can help target specific lymph node regions affected by the lymphoma, reducing the risk of recurrence[2].
3. Stem Cell Transplantation
For patients with relapsed or refractory lymphocyte depleted Hodgkin lymphoma, autologous stem cell transplantation (ASCT) may be considered. This approach involves harvesting the patient's stem cells, administering high-dose chemotherapy to eradicate the lymphoma, and then reinfusing the stem cells to restore bone marrow function. ASCT has been associated with improved survival rates in patients with aggressive forms of Hodgkin lymphoma[3].
4. Targeted Therapy and Immunotherapy
Recent advancements in targeted therapies and immunotherapy have opened new avenues for treating Hodgkin lymphoma. While not standard for LDHL specifically, agents like brentuximab vedotin (Adcetris®), an antibody-drug conjugate targeting CD30, have shown promise in treating relapsed or refractory cases. This therapy may be considered for patients who do not respond to conventional treatments[4].
5. Clinical Trials
Given the rarity of lymphocyte depleted Hodgkin lymphoma, participation in clinical trials may be an option for patients. These trials often explore novel therapies or combinations that could provide better outcomes than standard treatments. Patients are encouraged to discuss clinical trial opportunities with their healthcare providers[5].
Conclusion
The treatment of lymphocyte depleted Hodgkin lymphoma (ICD-10 code C81.38) typically involves a multi-faceted approach, primarily focusing on chemotherapy, with radiation therapy and stem cell transplantation as adjuncts for specific cases. As research continues to evolve, new therapies and clinical trials may offer additional options for patients facing this challenging diagnosis. It is crucial for patients to work closely with their oncology team to determine the most appropriate treatment plan tailored to their individual circumstances.
For further information or specific treatment recommendations, consulting with a hematologist or oncologist specializing in lymphomas is advisable.
Related Information
Description
- Rare subtype of Hodgkin lymphoma
- Predominance of Reed-Sternberg cells
- Significant depletion of lymphocytes
- Large, atypical Reed-Sternberg cells
- Aggressive clinical course
- Swollen lymph nodes in multiple regions
- Systemic symptoms like fever and weight loss
- Enlargement of spleen
- Generalized fatigue
Diagnostic Criteria
- Fever
- Night sweats
- Weight loss
- Pruritus
- Lymphadenopathy
- Splenomegaly
- Hepatomegaly
- Reed-Sternberg cells present
- Lymphocyte depletion observed
- CD30 and CD15 expression
- B-cell marker negativity
- Multiple lymph node involvement
- Anemia or thrombocytopenia
- Elevated LDH levels
Clinical Information
- Aggressive nature of LDHL
- Typically diagnosed at advanced stage
- Widespread lymphadenopathy common
- Systemic symptoms present in most cases
- Fever, night sweats, and weight loss prevalent
- Lymph node enlargement in multiple sites
- Immunocompromised states increase risk
- HIV infection a notable association
- Previous chemotherapy or radiation increases risk
Approximate Synonyms
- Lymphocyte Depleted Hodgkin's Disease
- Hodgkin Lymphoma, Lymphocyte Depleted Type
- LDHL
- Non-Specific Lymphadenopathy
- Advanced Stage Hodgkin Lymphoma
- B-cell Lymphoma
- Lymphoma with Lymphocyte Depletion
Treatment Guidelines
- Chemotherapy cornerstone of treatment
- ABVD Regimen common but limited effectiveness
- BEACOPP Regimen considered for advanced disease
- Radiation Therapy used with chemotherapy
- Involved-Field Radiation reduces recurrence risk
- Autologous Stem Cell Transplantation for relapsed/refractory cases
- Targeted Therapies and Immunotherapy emerging options
- Clinical Trials available for novel therapies
Related Diseases
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