ICD-10: C81.34

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 type of lymphoma is often associated with a more aggressive clinical course compared to other subtypes of Hodgkin lymphoma. The ICD-10 code C81.34 specifically refers to cases of lymphocyte depleted Hodgkin lymphoma that are localized to the lymph nodes of the axilla (armpit area) and upper limb.

Clinical Features

Symptoms

Patients with lymphocyte depleted Hodgkin lymphoma may present with a variety of symptoms, including:

• Lymphadenopathy: Swelling of lymph nodes, particularly in the axillary region.
• B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in Hodgkin lymphoma.
• Fatigue: Generalized tiredness and weakness.
• Pruritus: Itching without an apparent rash, which can be a symptom of lymphoma.

Diagnosis

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

• Biopsy: A lymph node biopsy is essential for confirming the diagnosis. The histological examination will reveal Reed-Sternberg cells and a marked reduction in lymphocytes.
• Imaging: CT scans or PET scans may be used to assess the extent of disease and involvement of lymph nodes, particularly in the axillary and upper limb regions.

Staging

Hodgkin lymphoma is staged using the Ann Arbor system, which considers the number of lymph node regions involved and the presence of systemic symptoms. LDHL can be classified as:

• Stage I: Involvement of a single lymph node region (e.g., axilla).
• Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm.

Treatment Options

Treatment for lymphocyte depleted Hodgkin lymphoma typically involves a combination of chemotherapy and radiation therapy. The specific regimen may vary based on the stage of the disease and the patient's overall health. Common treatment approaches include:

• Chemotherapy: Regimens such as ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine) are often used, although more aggressive regimens may be necessary for LDHL due to its aggressive nature.
• Radiation Therapy: In localized cases, radiation may be used to target affected lymph nodes, particularly in the axillary region.

Prognosis

The prognosis for patients with lymphocyte depleted Hodgkin lymphoma can be less favorable compared to other subtypes of Hodgkin lymphoma. Factors influencing prognosis include:

• Stage at Diagnosis: Early-stage disease generally has a better outcome.
• Response to Treatment: Patients who achieve a complete response to initial therapy tend to have improved survival rates.

Conclusion

ICD-10 code C81.34 identifies lymphocyte depleted Hodgkin lymphoma localized to the lymph nodes of the axilla and upper limb. This subtype is characterized by a unique histological profile and often requires aggressive treatment due to its clinical behavior. Early diagnosis and appropriate management are crucial for improving patient outcomes. For further information on coding and billing related to this condition, healthcare providers should refer to relevant medical policies and guidelines.

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. The ICD-10 code C81.34 specifically refers to cases where this lymphoma affects the lymph nodes of the axilla and upper limb. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for diagnosis and management.

Clinical Presentation

Signs and Symptoms

Patients with lymphocyte depleted Hodgkin lymphoma may present with a variety of symptoms, which can be categorized into local and systemic manifestations:

1. Local Symptoms:
   - Lymphadenopathy: The most common presentation is painless swelling of lymph nodes, particularly in the axillary region. Patients may notice a lump in the armpit or upper limb.
   - Localized Pain or Discomfort: Some patients may experience discomfort or pain in the affected area, although this is less common.

2. Systemic Symptoms:
   - B Symptoms: These include fever, night sweats, and unexplained weight loss. The presence of B symptoms is significant as it may indicate a more aggressive disease.
   - Fatigue: Patients often report a general sense of fatigue or malaise, which can be debilitating.
   - Pruritus: Itching without a rash can occur, although it is less common in this subtype compared to other forms of Hodgkin lymphoma.

Patient Characteristics

Certain demographic and clinical characteristics are often associated with lymphocyte depleted Hodgkin lymphoma:

• Age: LDHL typically occurs in older adults, with a median age of diagnosis in the 50s to 60s. It is less common in younger populations.
• Gender: There is a slight male predominance in the incidence of Hodgkin lymphoma, including the lymphocyte depleted subtype.
• Immunocompromised Status: Patients with compromised immune systems, such as those with HIV/AIDS or those who have undergone organ transplantation, are at a higher risk for developing this subtype of lymphoma.
• Histological Features: The diagnosis is confirmed through histological examination, which reveals a predominance of Reed-Sternberg cells and a marked reduction in lymphocytes, often accompanied by fibrosis.

Diagnosis and Management

Diagnosis typically involves imaging studies, such as CT scans, to assess the extent of lymphadenopathy and potential involvement of other organs. A biopsy of the affected lymph node is essential for histological confirmation.

Management of lymphocyte depleted Hodgkin lymphoma may include:

• Chemotherapy: Often the first line of treatment, with regimens tailored to the individual patient based on disease stage and overall health.
• Radiation Therapy: May be used in conjunction with chemotherapy, particularly for localized disease.
• Supportive Care: Addressing symptoms such as pain and fatigue, and monitoring for complications related to treatment.

Conclusion

Lymphocyte depleted Hodgkin lymphoma, particularly affecting the lymph nodes of the axilla and upper limb, presents with distinct clinical features and patient characteristics. Early recognition of symptoms, particularly lymphadenopathy and systemic B symptoms, is crucial for timely diagnosis and effective management. Given its rarity and the complexity of treatment, a multidisciplinary approach involving oncologists, radiologists, and pathologists is often necessary to optimize patient outcomes.

ICD-10 code C81.34 refers specifically to "Lymphocyte depleted Hodgkin lymphoma, lymph nodes of axilla and upper limb." This classification is part of the broader category of Hodgkin lymphoma, which is a type of cancer that originates in the lymphatic system. Below are alternative names and related terms associated with this specific diagnosis.

Alternative Names for C81.34

1. Lymphocyte Depleted Hodgkin Lymphoma (LDHL): This is the most direct alternative name, emphasizing the specific subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes.

2. Hodgkin's Disease, Lymphocyte Depleted Type: This term is often used interchangeably with lymphocyte depleted Hodgkin lymphoma, highlighting its classification as a type of Hodgkin's disease.

3. Hodgkin Lymphoma, Lymphocyte Depletion Variant: This name reflects the variant nature of the lymphoma, indicating its unique histological features.

Related Terms

1. Hodgkin Lymphoma: A broader term that encompasses all types of Hodgkin lymphoma, including lymphocyte depleted, mixed cellularity, and nodular sclerosis types.

2. Reed-Sternberg Cells: These are the abnormal cells found in Hodgkin lymphoma, and their presence is a defining characteristic of the disease.

3. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in Hodgkin lymphoma, including in the axillary and upper limb regions.

4. Axillary Lymph Nodes: Specific reference to the lymph nodes located in the armpit area, which can be affected in cases of lymphocyte depleted Hodgkin lymphoma.

5. Upper Limb Lymph Nodes: This term refers to the lymph nodes in the upper extremities, which may also be involved in the disease process.

6. Stage II Hodgkin Lymphoma: Depending on the extent of the disease, lymphocyte depleted Hodgkin lymphoma may be classified as Stage II if it involves lymph nodes on both sides of the diaphragm, including the axilla.

7. Chemotherapy and Radiation Therapy: Common treatment modalities for Hodgkin lymphoma, including lymphocyte depleted types, which may be referenced in treatment plans.

Understanding these alternative names and related terms can be crucial for healthcare professionals involved in the diagnosis, treatment, and coding of lymphocyte depleted Hodgkin lymphoma. Accurate coding and terminology ensure proper communication among medical professionals and facilitate appropriate patient care.

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. The diagnosis of LDHL, particularly when it involves the lymph nodes of the axilla and upper limb, follows specific criteria outlined in the ICD-10 coding system, particularly under code C81.34.

Diagnostic Criteria for Lymphocyte Depleted Hodgkin Lymphoma

Clinical Presentation

1. Symptoms: Patients may present with symptoms such as:
   - Painless lymphadenopathy, particularly in the axillary region.
   - Systemic symptoms like fever, night sweats, and weight loss (often referred to as "B symptoms").
   - Fatigue and pruritus may also be reported.

2. Physical Examination: A thorough physical examination is essential to identify enlarged lymph nodes, especially in the axilla and upper limb, which are indicative of lymphatic involvement.

Histopathological Examination

1. Biopsy: A definitive diagnosis requires a lymph node biopsy. The histological examination will reveal:
   - A predominance of Reed-Sternberg cells.
   - A marked depletion of small lymphocytes, which is characteristic of the lymphocyte depleted variant.
   - The presence of a fibrotic background and inflammatory cells, which may include eosinophils and plasma cells.

2. Immunohistochemistry: Immunophenotyping is crucial for confirming the diagnosis. Reed-Sternberg cells typically express:
   - CD30 (a hallmark of Hodgkin lymphoma).
   - CD15.
   - They may also show variable expression of other markers such as PAX5 and CD20.

Imaging Studies

1. CT or PET Scans: Imaging studies are used to assess the extent of disease. These scans help in:
   - Identifying lymph node involvement in the axilla and upper limb.
   - Evaluating for any mediastinal or abdominal lymphadenopathy and potential extranodal involvement.

2. Staging: The Ann Arbor staging system is commonly used to classify the extent of the disease, which is critical for treatment planning.

Laboratory Tests

1. Blood Tests: Routine blood tests may show:
   - Anemia or thrombocytopenia, which can occur due to bone marrow involvement.
   - Elevated lactate dehydrogenase (LDH) levels, which may indicate a more aggressive disease.

2. Bone Marrow Biopsy: In some cases, a bone marrow biopsy may be performed to assess for involvement of the bone marrow, which can influence staging and treatment decisions.

Conclusion

The diagnosis of lymphocyte depleted Hodgkin lymphoma, particularly in the lymph nodes of the axilla and upper limb, relies on a combination of clinical evaluation, histopathological findings, imaging studies, and laboratory tests. Accurate diagnosis is essential for appropriate management and treatment planning, as LDHL can have different prognostic implications compared to other subtypes of Hodgkin lymphoma. For coding purposes, the ICD-10 code C81.34 specifically denotes this subtype and its anatomical involvement, which is crucial for accurate medical billing and record-keeping.

Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.34, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes. This specific type 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 modalities.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy is the cornerstone of treatment for lymphocyte depleted Hodgkin lymphoma. The most commonly used regimens include:

• ABVD Regimen: This includes Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. While ABVD is the standard for many Hodgkin lymphoma subtypes, its efficacy in LDHL may be limited due to the aggressive nature of this variant.

• BEACOPP Regimen: This regimen (Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, and Prednisone) is often considered for patients with advanced disease or those who are at high risk of treatment failure. 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 a consolidation treatment after chemotherapy. The use of radiation can help target residual disease in lymph nodes, especially in the axilla and upper limb, where LDHL may present. The decision to use radiation therapy depends on the extent of the disease and the response to initial chemotherapy[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 outcomes in patients with aggressive forms of Hodgkin lymphoma[3].

4. Targeted Therapy and Immunotherapy

While traditional chemotherapy remains the primary treatment, there is growing interest in targeted therapies and immunotherapies. Agents such as brentuximab vedotin (Adcetris®), an antibody-drug conjugate targeting CD30, have shown promise in treating relapsed or refractory Hodgkin lymphoma, including LDHL. This therapy may be used in conjunction with chemotherapy or as a standalone treatment in certain cases[4].

Conclusion

The management of lymphocyte depleted Hodgkin lymphoma requires a tailored approach based on the individual patient's disease characteristics and overall health. Standard treatment typically involves a combination of chemotherapy, radiation therapy, and potentially stem cell transplantation for advanced cases. Emerging therapies, including targeted agents and immunotherapies, are also being explored to improve outcomes for patients with this challenging subtype of Hodgkin lymphoma. Ongoing clinical trials continue to evaluate the efficacy of these treatments, providing hope for better management strategies in the future.

References

1. [Chemotherapy regimens for Hodgkin lymphoma]
2. [Role of radiation therapy in Hodgkin lymphoma]
3. [Stem cell transplantation in Hodgkin lymphoma]
4. [Brentuximab vedotin in Hodgkin lymphoma treatment]