ICD-10: C81.36

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 known for its aggressive nature and is often associated with advanced disease at diagnosis. The ICD-10 code C81.36 specifically refers to cases of lymphocyte depleted Hodgkin lymphoma that are localized in the intrapelvic lymph nodes.

Clinical Description of Lymphocyte Depleted Hodgkin Lymphoma

Characteristics

• Histological Features: LDHL is marked by the presence of large Reed-Sternberg cells, which are the hallmark of Hodgkin lymphoma, alongside a notable scarcity of lymphocytes. This depletion can lead to a more pronounced inflammatory response and fibrosis within the lymphatic tissue.
• Symptoms: Patients may present with symptoms such as lymphadenopathy (swollen lymph nodes), fever, night sweats, weight loss, and pruritus (itching). The intrapelvic location may also lead to abdominal discomfort or pelvic pain due to the mass effect on surrounding structures.

Diagnosis

• Imaging Studies: Diagnosis typically involves imaging studies such as CT scans or PET scans to assess the extent of lymph node involvement and to identify any other sites of disease.
• Biopsy: A definitive diagnosis is made through a lymph node biopsy, where histological examination reveals the characteristic Reed-Sternberg cells and the lymphocyte depletion.

Staging

• Ann Arbor Staging System: LDHL is staged using the Ann Arbor classification, which considers the number of lymph node regions involved and whether the disease has spread to extranodal sites. The intrapelvic involvement would be classified based on the specific lymph node regions affected.

Treatment Options

• Chemotherapy: The primary treatment for LDHL often involves chemotherapy regimens, which may include combinations of agents such as doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD).
• Radiation Therapy: In some cases, radiation therapy may be utilized, particularly if there is localized disease or residual masses after chemotherapy.
• Stem Cell Transplant: For patients with relapsed or refractory disease, autologous stem cell transplantation may be considered as a treatment option.

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 overall health, and response to initial treatment.

Conclusion

ICD-10 code C81.36 is specifically designated for lymphocyte depleted Hodgkin lymphoma affecting the intrapelvic lymph nodes. This subtype is notable for its aggressive behavior and unique histological features, necessitating prompt diagnosis and treatment. Understanding the clinical characteristics, diagnostic criteria, and treatment options is crucial for managing this challenging condition effectively.

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 C81.36 specifically refers to cases where the disease is localized to the intrapelvic lymph nodes. 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 broadly categorized into local and systemic manifestations:

1. Local Symptoms:
   - Lymphadenopathy: Patients often exhibit enlarged lymph nodes, particularly in the pelvic region. This may lead to discomfort or a palpable mass in the lower abdomen.
   - Abdominal Pain or Discomfort: Due to the involvement of intrapelvic lymph nodes, patients may experience pain or a feeling of fullness in the lower abdomen.

2. Systemic Symptoms:
   - B Symptoms: These include fever, night sweats, and unexplained weight loss. B symptoms are significant as they can indicate a more aggressive disease course.
   - Fatigue: Generalized fatigue is common and can be debilitating for patients.
   - Pruritus: Some patients may experience itching, which is not necessarily related to the presence of skin lesions.

Patient Characteristics

Lymphocyte depleted Hodgkin lymphoma typically presents in specific demographic groups:

• Age: This subtype is more commonly diagnosed in older adults, particularly those over the age of 50. However, it can occur in younger individuals as well.
• Gender: There is a slight male predominance in the incidence of Hodgkin lymphoma, including the lymphocyte depleted subtype.
• Comorbidities: Patients may have underlying health conditions that can complicate the diagnosis and treatment, such as immunosuppression or other malignancies.

Diagnosis

The diagnosis of lymphocyte depleted Hodgkin lymphoma involves a combination of clinical evaluation, imaging studies, and histopathological examination:

• Imaging: CT scans or PET scans are often utilized to assess the extent of lymphadenopathy and to evaluate for any organ involvement.
• Biopsy: A definitive diagnosis is made through a lymph node biopsy, where the presence of Reed-Sternberg cells and the characteristic lymphocyte depletion can be confirmed.

Conclusion

Lymphocyte depleted Hodgkin lymphoma, particularly when localized to the intrapelvic lymph nodes, presents with distinct clinical features and patient characteristics. Recognizing the signs and symptoms, along with understanding the demographic trends, is essential for timely diagnosis and effective management. Given its rarity and the potential for aggressive behavior, a multidisciplinary approach involving oncologists, radiologists, and pathologists is often necessary to optimize patient outcomes.

ICD-10 code C81.36 specifically refers to "Lymphocyte depleted Hodgkin lymphoma, intrapelvic lymph nodes." This classification is part of the broader category of Hodgkin lymphoma, which is a type of lymphatic cancer characterized by the presence of Reed-Sternberg cells. Below are alternative names and related terms associated with this specific diagnosis.

Alternative Names for C81.36

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: This variation maintains the focus on the lymphocyte-depleted nature of the disease while still categorizing it under Hodgkin lymphoma.

Related Terms

1. Hodgkin Lymphoma: The broader category under which lymphocyte depleted Hodgkin lymphoma falls. Hodgkin lymphoma is divided into several subtypes, including lymphocyte depleted, mixed cellularity, and nodular sclerosis.

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

3. Intraperitoneal Lymph Nodes: While C81.36 specifies intrapelvic lymph nodes, it is important to note that lymphatic involvement can also occur in other regions, such as the intraperitoneal space.

4. Stage IV Hodgkin Lymphoma: In some cases, lymphocyte depleted Hodgkin lymphoma may be classified as Stage IV if it has spread to distant lymph nodes or organs, which can include intrapelvic lymph nodes.

5. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.

6. Lymphocyte Predominant Hodgkin Lymphoma: Although this is a different subtype, it is often discussed in contrast to lymphocyte depleted Hodgkin lymphoma, highlighting the diversity within Hodgkin lymphoma classifications.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C81.36 is crucial for accurate diagnosis, treatment planning, and coding in medical records. The terminology reflects the specific characteristics of lymphocyte depleted Hodgkin lymphoma and its classification within the broader context of lymphatic cancers. If you need further information or clarification on this topic, feel free to ask!

Lymphocyte depleted Hodgkin lymphoma (LDHL) is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes. The diagnosis of LDHL, particularly when it involves intrapelvic lymph nodes, follows specific criteria outlined in the ICD-10 coding system, particularly under the code C81.36. Below, we explore the diagnostic criteria and relevant considerations for this condition.

Diagnostic Criteria for Lymphocyte Depleted Hodgkin Lymphoma

1. Clinical Presentation

• Symptoms: Patients may present with symptoms such as lymphadenopathy (swollen lymph nodes), fever, night sweats, and weight loss, commonly referred to as "B symptoms." In cases involving intrapelvic lymph nodes, patients may also experience abdominal discomfort or pelvic pain due to lymph node enlargement.
• Physical Examination: A thorough physical examination is essential to identify lymphadenopathy in the cervical, axillary, or inguinal regions, as well as any abdominal or pelvic masses.

2. Histopathological Examination

• Biopsy: A definitive diagnosis of LDHL requires a biopsy of the affected lymph node or tissue. The histological examination will reveal the presence of Reed-Sternberg cells, which are large, atypical lymphoid cells.
• Cellularity: In LDHL, there is a marked depletion of lymphocytes, with a predominance of Reed-Sternberg cells and a fibrotic background. This is a key distinguishing feature of this subtype compared to other forms of Hodgkin lymphoma.

3. Immunophenotyping

• Immunohistochemistry: The use of immunohistochemical stains is crucial for confirming the diagnosis. Reed-Sternberg cells typically express CD30 and CD15, while they lack the expression of pan-B-cell markers (e.g., CD20). The immunophenotype helps differentiate LDHL from other lymphoproliferative disorders.

4. Staging and Imaging Studies

• Imaging: Imaging studies such as CT scans or PET scans are utilized to assess the extent of the disease, particularly to identify the involvement of intrapelvic lymph nodes. These imaging modalities help in staging the lymphoma according to the Ann Arbor classification.
• Staging: The staging process is critical for treatment planning and involves determining whether the lymphoma is localized (stage I or II) or has spread (stage III or IV).

5. Exclusion of Other Conditions

• Differential Diagnosis: It is essential to rule out other conditions that may mimic LDHL, such as non-Hodgkin lymphoma or other malignancies. This may involve additional laboratory tests and imaging studies.

Conclusion

The diagnosis of lymphocyte depleted Hodgkin lymphoma, particularly when it involves intrapelvic lymph nodes, is a multifaceted process that includes clinical evaluation, histopathological confirmation, immunophenotyping, and imaging studies. Accurate diagnosis is crucial for effective treatment planning and management of the disease. If you have further questions or need more specific information regarding treatment options or prognosis, feel free to ask!

Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.36, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. This specific type often presents with advanced disease and can involve various lymph node regions, including intrapelvic lymph nodes. The treatment approaches for LDHL typically align with those for other forms of Hodgkin lymphoma but may require tailored strategies due to its unique characteristics.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains 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 standard for many Hodgkin lymphoma cases, its efficacy in LDHL may be limited due to the aggressive nature of this subtype.

• 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 patients with more aggressive forms of Hodgkin lymphoma, including LDHL[1].

2. Radiation Therapy

Radiation therapy may be employed as part of the treatment plan, particularly in cases where localized disease is present or after chemotherapy to consolidate the response. Involved-field radiation therapy (IFRT) is often used to target specific lymph node regions, including intrapelvic nodes, to minimize exposure to surrounding healthy tissues[2].

3. Stem Cell Transplantation

For patients with relapsed or refractory lymphocyte depleted Hodgkin lymphoma, high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be considered. This approach can provide a chance for long-term remission, especially in younger patients or those with good performance status[3].

4. Targeted Therapy and Immunotherapy

Recent advancements in targeted therapies and immunotherapies have opened new avenues for treatment. 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].

5. Clinical Trials

Given the rarity of lymphocyte depleted Hodgkin lymphoma, participation in clinical trials may be a viable option for patients. These trials often explore novel therapies or combinations that could offer improved outcomes compared to standard treatments[5].

Conclusion

The management of lymphocyte depleted Hodgkin lymphoma, particularly when involving intrapelvic lymph nodes, requires a comprehensive approach that may include chemotherapy, radiation therapy, stem cell transplantation, and emerging targeted therapies. Due to the aggressive nature of this subtype, treatment plans should be individualized, and patients are encouraged to discuss all available options, including clinical trials, with their healthcare providers. Continuous monitoring and follow-up are essential to assess treatment response and manage any potential complications effectively.

For further information or specific treatment recommendations, consulting with a hematologist or oncologist specializing in lymphomas is advisable.