ICD-10: C81.32

Lymphocyte depleted Hodgkin lymphoma (LDHL) is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant reduction in 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 C81.32 specifically refers to cases of lymphocyte depleted Hodgkin lymphoma that are localized to the intrathoracic lymph nodes.

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. The histological features include a scarcity of lymphocytes, which distinguishes it from other forms of Hodgkin lymphoma. This subtype is often associated with a more advanced stage at diagnosis and a poorer prognosis due to its aggressive nature and tendency to present with systemic symptoms.

Symptoms

Patients with LDHL may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the thoracic region.
- B symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement.
- Respiratory symptoms: Due to the involvement of intrathoracic lymph nodes, patients may experience cough, chest pain, or difficulty breathing.

Diagnosis

Diagnosis typically involves:
- Imaging studies: CT scans or PET scans are used to assess the extent of lymph node involvement and to identify any mediastinal or intrathoracic masses.
- Biopsy: A lymph node biopsy is essential for histological confirmation, where the characteristic Reed-Sternberg cells can be identified.

Staging

The staging of Hodgkin lymphoma follows the Ann Arbor classification, which considers the number of lymph node regions involved and the presence of systemic symptoms. LDHL often presents at an advanced stage (III or IV).

Treatment Options

The treatment for lymphocyte depleted Hodgkin lymphoma generally includes:
- Chemotherapy: Multi-agent chemotherapy regimens, such as ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine), are commonly used, although LDHL may require more intensive regimens due to its aggressive nature.
- Radiation therapy: This may be employed in conjunction with chemotherapy, particularly for localized disease.
- Stem cell transplant: In cases of relapsed or refractory disease, autologous stem cell transplantation may be considered.

Prognosis

The prognosis for patients with lymphocyte depleted Hodgkin lymphoma is generally poorer than for 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 C81.32 is specifically designated for lymphocyte depleted Hodgkin lymphoma affecting the intrathoracic lymph nodes. This subtype is characterized by a distinct clinical presentation and requires a tailored approach to treatment due to its aggressive nature. Early diagnosis and appropriate management are crucial for improving outcomes in affected patients.

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 C81.32 specifically refers to cases where the lymphoma is located in the intrathoracic lymph nodes. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for accurate 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:
   - Mediastinal Mass: Patients may experience symptoms related to a mediastinal mass, such as cough, chest pain, or dyspnea due to compression of surrounding structures.
   - Lymphadenopathy: Enlargement of lymph nodes in the thoracic region may be palpable or detected incidentally on imaging studies.

2. Systemic Symptoms:
   - B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many types of lymphoma, including LDHL.
   - Fatigue: Patients often report significant fatigue, which can be debilitating and affect quality of life.

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 at diagnosis often in the 30s to 50s range, although it can occur at any age.
• Gender: There is a slight male predominance in cases 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, may have a higher incidence of this subtype of lymphoma.

Diagnosis

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

• Imaging: CT scans of the chest, abdomen, and pelvis are commonly used to assess the extent of disease and identify lymph node involvement.
• Biopsy: A definitive diagnosis is made through a lymph node biopsy, where histological examination reveals the characteristic Reed-Sternberg cells amidst a background of few lymphocytes.

Conclusion

Lymphocyte depleted Hodgkin lymphoma, particularly when involving intrathoracic lymph nodes, presents with a unique set of clinical features. Patients may exhibit both local symptoms related to lymphadenopathy and systemic symptoms indicative of lymphoma. Understanding these characteristics is essential for healthcare providers to ensure timely diagnosis and appropriate treatment. Given the rarity of this subtype, awareness of its presentation can aid in distinguishing it from other forms of lymphoma and guide further management strategies.

ICD-10 code C81.32 refers specifically to "Lymphocyte depleted Hodgkin lymphoma, intrathoracic 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

1. Lymphocyte Depleted Hodgkin's Disease: This term is often used interchangeably with Hodgkin lymphoma, particularly in older literature.
2. Lymphocyte Depleted Type of Hodgkin Lymphoma: A more descriptive term that emphasizes the specific subtype of Hodgkin lymphoma.
3. Hodgkin Lymphoma, Lymphocyte Depleted Variant: This variant name highlights the unique histological features of the disease.

Related Terms

1. Hodgkin Lymphoma: The broader category under which C81.32 falls, encompassing various subtypes, including lymphocyte depleted.
2. Reed-Sternberg Cells: The hallmark cells found in Hodgkin lymphoma, crucial for diagnosis.
3. Intrathoracic Lymph Nodes: Refers to the specific location of the lymph nodes affected in this case, which is significant for staging and treatment considerations.
4. Stage IIIB Hodgkin Lymphoma: Depending on the extent of the disease, lymphocyte depleted Hodgkin lymphoma may be classified under this stage, which indicates involvement of lymph nodes on both sides of the diaphragm.
5. Non-Hodgkin Lymphoma: While distinct from Hodgkin lymphoma, this term is often mentioned in discussions about lymphatic cancers, providing a contrast to Hodgkin's disease.

Clinical Context

Lymphocyte depleted Hodgkin lymphoma is a rare subtype, accounting for a small percentage of all Hodgkin lymphoma cases. It is characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes, which can lead to a more aggressive clinical course compared to other subtypes. Understanding the terminology and related terms is essential for accurate diagnosis, treatment planning, and coding in medical records.

In summary, the ICD-10 code C81.32 is associated with several alternative names and related terms that reflect its clinical and pathological characteristics. These terms are important for healthcare professionals involved in the diagnosis and treatment of this specific type of lymphoma.

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 intrathoracic lymph nodes, follows specific criteria outlined in the ICD-10 coding system, particularly under the code C81.32.

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. Localized symptoms may arise from the involvement of intrathoracic lymph nodes, including cough, chest pain, or respiratory distress due to mediastinal mass effects.
• Physical Examination: Lymphadenopathy, particularly in the cervical, axillary, or mediastinal regions, is often noted during the physical examination.

2. Histopathological Examination

• Biopsy: A definitive diagnosis requires a biopsy of the affected lymph node. Histological examination reveals the presence of Reed-Sternberg cells, which are large, atypical lymphoid cells.
• Cellularity: In LDHL, there is a marked reduction in the number of lymphocytes, with a predominance of Reed-Sternberg cells and a fibrotic background. This is a key distinguishing feature of this subtype.

3. Imaging Studies

• CT Scan: Imaging studies, particularly CT scans of the chest, abdomen, and pelvis, are essential for assessing the extent of disease and identifying the involvement of intrathoracic lymph nodes. The presence of enlarged lymph nodes in the mediastinum or hilum can support the diagnosis.
• PET Scan: Positron Emission Tomography (PET) scans may be utilized to evaluate metabolic activity in lymph nodes and to assess the response to treatment.

4. Staging

• Ann Arbor Staging System: The staging of Hodgkin lymphoma, including LDHL, follows the Ann Arbor classification, which considers the number of lymph node regions involved and the presence of systemic symptoms. For intrathoracic involvement, the disease may be classified as Stage II (involvement of two or more lymph node regions on the same side of the diaphragm) or Stage III (involvement of lymph nodes on both sides of the diaphragm).

5. Laboratory Tests

• Blood Tests: Routine blood tests may show anemia, elevated lactate dehydrogenase (LDH), and other abnormalities that can indicate the presence of malignancy.
• Bone Marrow Biopsy: In some cases, a bone marrow biopsy may be performed to rule out bone marrow involvement.

Conclusion

The diagnosis of Lymphocyte Depleted Hodgkin Lymphoma, particularly with intrathoracic lymph node involvement, is a multifaceted process that includes clinical evaluation, histopathological confirmation, imaging studies, and staging. Accurate diagnosis is crucial for determining the appropriate treatment strategy and improving patient outcomes. If you have further questions or need more specific information regarding treatment options or prognosis, feel free to ask!

Lymphocyte depleted Hodgkin lymphoma (LDHL), particularly when it involves intrathoracic lymph nodes, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes. The management of this condition typically involves a combination of chemotherapy, radiation therapy, and, in some cases, stem cell transplantation. Below is a detailed overview of the standard treatment approaches for ICD-10 code C81.32.

Overview of Lymphocyte Depleted Hodgkin Lymphoma

LDHL is known for its aggressive nature and is often diagnosed at an advanced stage. The treatment strategy is influenced by various factors, including the stage of the disease, the patient's overall health, and the presence of any comorbid conditions.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy is the cornerstone of treatment for LDHL. 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.

• Escalated BEACOPP: 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. Studies suggest that escalated BEACOPP may improve outcomes in aggressive forms of Hodgkin lymphoma, including LDHL[1].

2. Radiation Therapy

Radiation therapy may be employed as a consolidative treatment following chemotherapy, especially in cases where there is residual disease or in localized stages. The use of involved-field radiation therapy (IFRT) is common, targeting only the areas affected by the lymphoma.

• Role in Treatment: Radiation can help reduce the risk of relapse in patients who achieve a complete response to chemotherapy. However, the decision to use radiation therapy is carefully considered, particularly in younger patients, due to the potential long-term side effects, including secondary malignancies[2].

3. Stem Cell Transplantation

For patients with relapsed or refractory LDHL, autologous stem cell transplantation (ASCT) may be indicated. This approach is typically considered after the patient has undergone salvage chemotherapy.

• Indications: ASCT is often recommended for patients who have not responded to initial treatment or who experience a relapse after first-line therapy. The procedure 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[3].

4. Targeted Therapy and Immunotherapy

While traditional chemotherapy remains the mainstay of treatment, there is growing interest in targeted therapies and immunotherapies for Hodgkin lymphoma, including LDHL.

• Brentuximab Vedotin (Adcetris): This is an antibody-drug conjugate that targets CD30, a marker present on Reed-Sternberg cells. It is used in cases of relapsed or refractory Hodgkin lymphoma and may be considered in combination with chemotherapy or as a single agent[4].

• Checkpoint Inhibitors: Agents such as nivolumab and pembrolizumab, which target PD-1, have shown promise in treating relapsed Hodgkin lymphoma and may be considered in clinical trials or after other treatments have failed[5].

Conclusion

The treatment of lymphocyte depleted Hodgkin lymphoma, particularly when it involves intrathoracic lymph nodes, requires a multidisciplinary approach tailored to the individual patient's needs. Chemotherapy remains the primary treatment modality, often supplemented by radiation therapy and, in select cases, stem cell transplantation. Emerging therapies, including targeted agents and immunotherapies, are expanding the treatment landscape, offering hope for improved outcomes in this challenging subtype of Hodgkin lymphoma. Ongoing clinical trials continue to explore the most effective combinations and sequences of these therapies to enhance patient survival and quality of life.

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.
5. Checkpoint inhibitors in Hodgkin lymphoma.