ICD-10: C81.37

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. The ICD-10 code for this condition, specifically when it involves the spleen, is C81.37. Below is a detailed clinical description and relevant information regarding this diagnosis.

Clinical Description of Lymphocyte Depleted Hodgkin Lymphoma

Overview

Lymphocyte depleted Hodgkin lymphoma is one of the less common forms of Hodgkin lymphoma, accounting for approximately 1% to 2% of all cases. It is typically associated with a more aggressive clinical course compared to other subtypes of Hodgkin lymphoma, such as mixed cellularity or nodular sclerosis. Patients often present with advanced disease at diagnosis.

Pathophysiology

In LDHL, the histological features include:
- Reed-Sternberg Cells: These are large, atypical lymphoid cells that are a hallmark of Hodgkin lymphoma. In LDHL, these cells are often more numerous and can be found in a background of sparse lymphocytes.
- Fibrosis: There is often significant fibrosis in the tumor microenvironment, contributing to the depletion of lymphocytes.
- Inflammatory Background: The presence of inflammatory cells, including eosinophils and plasma cells, is common.

Symptoms

Patients with lymphocyte depleted Hodgkin lymphoma may experience a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, often in the neck, axilla, or groin.
- Splenomegaly: Enlargement of the spleen, which can lead to abdominal discomfort or fullness.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement.

Diagnosis

Diagnosis typically involves:
- Histopathological Examination: A biopsy of affected lymph nodes or spleen is essential for confirming the diagnosis. The presence of Reed-Sternberg cells amidst a background of few lymphocytes is characteristic.
- Imaging Studies: CT scans or PET scans may be used to assess the extent of disease and involvement of the spleen and other organs.

Treatment

The treatment for lymphocyte depleted Hodgkin lymphoma often includes:
- Chemotherapy: Regimens such as ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine) or escalated BEACOPP may be employed, depending on the stage and patient factors.
- Radiation Therapy: May be used in conjunction with chemotherapy, particularly for localized disease.
- Stem Cell Transplantation: 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, primarily due to its aggressive nature and tendency for late-stage presentation. Early diagnosis and treatment are crucial for improving outcomes.

Conclusion

ICD-10 code C81.37 specifically identifies lymphocyte depleted Hodgkin lymphoma involving the spleen, highlighting the need for careful clinical evaluation and management. Given its rarity and aggressive behavior, awareness and understanding of this subtype are essential for healthcare providers involved in the diagnosis and treatment of lymphoma. Regular follow-up and monitoring for treatment response and potential complications are critical components of 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. This specific type of lymphoma is associated with distinct clinical presentations, signs, symptoms, and patient characteristics, particularly when it involves the spleen, as indicated by the ICD-10 code C81.37.

Clinical Presentation

General Characteristics

Lymphocyte Depleted Hodgkin Lymphoma typically presents in older adults, often affecting individuals in their 50s and 60s. It is more common in males than females, with a male-to-female ratio of approximately 2:1[1]. Patients may present with advanced disease at diagnosis, which is often linked to the aggressive nature of this lymphoma subtype.

Symptoms

The symptoms of LDHL can vary but commonly include:

• Lymphadenopathy: Swelling of lymph nodes, particularly in the cervical, axillary, or mediastinal regions.
• Splenomegaly: Enlargement of the spleen, which can lead to abdominal discomfort or fullness[1].
• B Symptoms: These are systemic symptoms that may include:
• Fever
• Night sweats
• Unexplained weight loss
• Fatigue: A common complaint among patients, often due to the disease's systemic effects.
• Pruritus: Itching without an apparent rash, which can be a symptom associated with Hodgkin lymphoma[1].

Signs

Upon physical examination, clinicians may observe:

• Palpable lymph nodes: Enlarged lymph nodes in various regions of the body.
• Abdominal mass: Due to splenomegaly or lymphadenopathy in the abdominal cavity.
• Signs of anemia: Such as pallor or fatigue, which may be evident in advanced cases[1].

Patient Characteristics

Demographics

• Age: Most commonly diagnosed in individuals aged 30 to 50 years, but can occur in older adults as well.
• Gender: More prevalent in males, with a higher incidence in those with a history of immunosuppression or HIV infection[1].

Risk Factors

• Immunosuppression: Patients with compromised immune systems, such as those with HIV/AIDS or those on immunosuppressive therapy, are at increased risk for developing LDHL.
• Previous Epstein-Barr Virus (EBV) infection: There is a noted association between EBV and Hodgkin lymphoma, including the lymphocyte-depleted subtype[1].

Prognosis

The prognosis for patients with Lymphocyte Depleted Hodgkin Lymphoma tends to be poorer compared to other subtypes of Hodgkin lymphoma. This is primarily due to its aggressive nature and the likelihood of presenting at an advanced stage. Treatment often involves a combination of chemotherapy and radiation therapy, tailored to the individual patient's disease stage and overall health status[1].

Conclusion

Lymphocyte Depleted Hodgkin Lymphoma, particularly when involving the spleen, presents with a unique set of clinical features and patient characteristics. Understanding these aspects is crucial for timely diagnosis and effective management. Given its aggressive nature and association with advanced disease at diagnosis, early recognition of symptoms and appropriate referral for further evaluation are essential for improving patient outcomes.

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 ICD-10 code for this condition is C81.37, specifically indicating the involvement of the spleen. Below are alternative names and related terms associated with this diagnosis.

Alternative Names for Lymphocyte Depleted Hodgkin Lymphoma

1. Lymphocyte Depleted Hodgkin's Disease: This term is often used interchangeably with lymphocyte depleted Hodgkin lymphoma, emphasizing the disease aspect.
2. Hodgkin Lymphoma, Lymphocyte Depleted Type: This name highlights the specific subtype of Hodgkin lymphoma.
3. Depleted Lymphocyte Hodgkin Lymphoma: A variation that maintains the focus on the lymphocyte depletion characteristic of this subtype.

Related Terms

1. Reed-Sternberg Cells: These are the hallmark cells found in Hodgkin lymphoma, including the lymphocyte depleted subtype. Their presence is critical for diagnosis.
2. Hodgkin Lymphoma: A broader category that includes various subtypes, including lymphocyte depleted, mixed cellularity, and nodular sclerosis.
3. Spleen Involvement: Refers to the specific anatomical involvement in this case, which is significant for staging and treatment considerations.
4. Non-Classical Hodgkin Lymphoma: While not a direct synonym, this term can sometimes encompass various atypical presentations of Hodgkin lymphoma, including lymphocyte depleted forms.

Clinical Context

Lymphocyte depleted Hodgkin lymphoma is often associated with poorer prognosis compared to other subtypes of Hodgkin lymphoma. It is more prevalent in older adults and can present with advanced disease at diagnosis. Understanding the terminology and related terms is crucial for accurate diagnosis, treatment planning, and coding in medical records.

In summary, while C81.37 specifically refers to lymphocyte depleted Hodgkin lymphoma with splenic involvement, the alternative names and related terms provide a broader context for understanding this condition within the spectrum of Hodgkin 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 the spleen, is guided by specific clinical, histological, and imaging criteria. Below is a detailed overview of the criteria used for diagnosis, particularly in relation to the ICD-10 code C81.37.

Clinical Presentation

Symptoms

Patients with lymphocyte depleted Hodgkin lymphoma may present with a variety of symptoms, including:
- Lymphadenopathy: Swollen lymph nodes, often painless.
- Splenomegaly: Enlargement of the spleen, which may be detected during a physical examination or imaging studies.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many types of lymphoma.

Patient History

A thorough patient history is essential, including:
- Previous history of lymphoma or other malignancies.
- Family history of hematological malignancies.
- Exposure to risk factors such as certain infections (e.g., Epstein-Barr virus).

Diagnostic Imaging

Imaging Studies

Imaging plays a crucial role in the diagnosis and staging of LDHL:
- CT Scans: Computed tomography scans of the chest, abdomen, and pelvis are used to identify lymphadenopathy and splenic involvement.
- PET Scans: Positron emission tomography can help assess metabolic activity in lymph nodes and the spleen, aiding in the diagnosis and staging.

Histopathological Examination

Biopsy

A definitive diagnosis of lymphocyte depleted Hodgkin lymphoma requires a biopsy of affected tissue:
- Excisional Biopsy: This is the preferred method, allowing for the examination of the architecture of lymph nodes or spleen.
- Histological Features: Pathological examination reveals:
- A predominance of Reed-Sternberg cells.
- A marked depletion of lymphocytes, which distinguishes LDHL from other subtypes of Hodgkin lymphoma.
- The presence of a fibrotic background and inflammatory cells.

Immunohistochemistry

Immunohistochemical staining is performed to confirm the diagnosis:
- Positive Markers: Reed-Sternberg cells typically express CD30 and CD15.
- Negative Markers: The absence of lymphocyte markers (e.g., CD3, CD20) in the majority of the cellular population supports the diagnosis of lymphocyte depletion.

Laboratory Tests

Blood Tests

Routine blood tests may reveal:
- Anemia or thrombocytopenia, which can occur due to bone marrow involvement.
- Elevated lactate dehydrogenase (LDH) levels, which may indicate tumor burden.

Conclusion

The diagnosis of lymphocyte depleted Hodgkin lymphoma, particularly with splenic involvement (ICD-10 code C81.37), relies on a combination of clinical evaluation, imaging studies, histopathological examination, and laboratory tests. The integration of these diagnostic criteria is essential for accurate diagnosis and appropriate management of the disease. If you have further questions or need more specific information, feel free to ask!

Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.37, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. This form of lymphoma often presents with advanced disease and can involve the spleen, necessitating a comprehensive treatment approach. Below, we explore the standard treatment modalities for this condition.

Treatment Overview

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 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 patients with LDHL due to its more intensive nature[1].

2. Radiation Therapy

Radiation therapy can be utilized in conjunction with chemotherapy, particularly in cases where localized disease is present or after chemotherapy to consolidate the response. The use of involved-field radiation therapy (IFRT) is common, targeting the spleen and any other affected lymph nodes. However, the role of radiation therapy is less clear in advanced cases of LDHL, where systemic treatment is prioritized[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 is typically reserved for those who have achieved a partial or complete response to initial therapy but have a high risk of relapse. Allogeneic stem cell transplantation may also be an option in select cases, particularly for younger patients or those with significant comorbidities[3].

4. Targeted Therapy and Novel Agents

Recent advancements in targeted therapies have opened new avenues for treatment. Agents such as brentuximab vedotin, an antibody-drug conjugate targeting CD30, have shown promise in treating relapsed Hodgkin lymphoma and may be considered in the treatment of LDHL, especially in cases where traditional therapies have failed[4]. Additionally, clinical trials exploring the efficacy of immune checkpoint inhibitors, such as pembrolizumab and nivolumab, are ongoing and may provide new options for patients with refractory disease[5].

Conclusion

The management of lymphocyte depleted Hodgkin lymphoma, particularly when involving the spleen, requires a multidisciplinary approach tailored to the individual patient's disease characteristics and overall health. Standard treatment typically involves a combination of chemotherapy, radiation therapy, and potentially stem cell transplantation, with ongoing research into targeted therapies offering hope for improved outcomes. As treatment protocols continue to evolve, it is essential for patients to discuss their options with a healthcare provider specializing in hematologic malignancies to determine the most appropriate course of action.

References

1. Article - Billing and Coding: Radiation Therapies (A59350).
2. Billing and Coding: Intensity Modulated Radiation Therapy.
3. Hematopoietic Cell Transplantation for Hodgkin's Disease.
4. Adcetris® (brentuximab vedotin).
5. Donor Lymphocyte Infusion for Hematologic Malignancies.