ICD-10: C81.17

Nodular sclerosis Hodgkin lymphoma (NSHL) is a subtype of Hodgkin lymphoma characterized by specific histological features and clinical presentations. The ICD-10 code C81.17 specifically refers to cases of nodular sclerosis Hodgkin lymphoma that involve the spleen. Below is a detailed overview of this condition, including its clinical description, diagnostic criteria, and relevant coding information.

Clinical Description of Nodular Sclerosis Hodgkin Lymphoma

Definition and Characteristics

Nodular sclerosis Hodgkin lymphoma is the most common subtype of Hodgkin lymphoma, accounting for approximately 60-80% of all cases. It is characterized by the presence of Reed-Sternberg cells, which are large, abnormal lymphocytes that are typically found in lymphoid tissue. The nodular sclerosis variant is distinguished by the presence of fibrous bands that create nodular structures within the lymph nodes.

Symptoms

Patients with NSHL may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, often in the neck, axilla, or mediastinum.
- Splenomegaly: Enlargement of the spleen, which can be detected during a physical examination or imaging studies.
- B symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement.

Diagnosis

The diagnosis of nodular sclerosis Hodgkin lymphoma typically involves:
- Histopathological examination: A biopsy of affected lymph nodes or spleen tissue is essential for identifying Reed-Sternberg cells and the characteristic nodular architecture.
- Imaging studies: CT scans or PET scans may be used to assess the extent of disease and involvement of the spleen and other organs.
- Staging: The Ann Arbor staging system is commonly used to determine the extent of the disease, which is crucial for treatment planning.

ICD-10 Code C81.17: Specifics

Code Definition

The ICD-10 code C81.17 specifically denotes "Nodular sclerosis Hodgkin lymphoma, spleen." This code is used when the lymphoma is confirmed to involve the spleen, indicating a more advanced stage of the disease.

Coding Guidelines

• Use of C81.17: This code should be applied when documenting cases where nodular sclerosis Hodgkin lymphoma has been confirmed to affect the spleen, either through imaging or histological evidence.
• Related Codes: Other related codes for Hodgkin lymphoma include C81.10 (Nodular sclerosis Hodgkin lymphoma, unspecified site) and C81.11 (Nodular sclerosis Hodgkin lymphoma, lymph nodes of head, face, and neck), which may be relevant for comprehensive documentation.

Treatment Implications

The involvement of the spleen in nodular sclerosis Hodgkin lymphoma may influence treatment decisions. Common treatment modalities include:
- Chemotherapy: Often the first line of treatment, typically involving a combination of drugs.
- Radiation therapy: May be used in conjunction with chemotherapy, especially if there is localized disease.
- Surgical intervention: In some cases, splenectomy (removal of the spleen) may be considered, particularly if the spleen is significantly enlarged or symptomatic.

Conclusion

Nodular sclerosis Hodgkin lymphoma involving the spleen, classified under ICD-10 code C81.17, represents a significant clinical entity within hematological malignancies. Accurate coding and documentation are essential for effective treatment planning and management of the disease. Understanding the clinical features, diagnostic criteria, and treatment options is crucial for healthcare providers involved in the care of patients with this condition.

Nodular sclerosis Hodgkin lymphoma (NSHL) is a subtype of Hodgkin lymphoma characterized by specific clinical presentations, signs, symptoms, and patient demographics. This form of lymphoma is often associated with the spleen, leading to the ICD-10 code C81.17. Below is a detailed overview of its clinical presentation and associated characteristics.

Clinical Presentation

Signs and Symptoms

1. Lymphadenopathy:
   - The most common initial symptom is painless swelling of lymph nodes, particularly in the cervical (neck) and supraclavicular regions. Patients may also experience lymphadenopathy in the axillary and inguinal areas[1][2].

2. Splenomegaly:
   - Enlargement of the spleen is frequently observed in patients with NSHL. This can lead to discomfort or a feeling of fullness in the abdomen[3][4].

3. B Symptoms:
   - Patients may present with systemic symptoms known as "B symptoms," which include:

   • Unexplained fever
   • Night sweats
   • Significant weight loss (more than 10% of body weight over six months) [5][6].

4. Fatigue:
   - A common complaint among patients, often attributed to the disease process and its systemic effects[7].

5. Pruritus:
   - Some patients report itching, which can be generalized and is often unrelated to any rash or skin condition[8].

6. Respiratory Symptoms:
   - In cases where lymph nodes in the mediastinum are involved, patients may experience cough, chest pain, or difficulty breathing due to compression of the airways[9].

Patient Characteristics

1. Demographics:
   - NSHL typically affects young adults, with a peak incidence in individuals aged 15 to 30 years. There is a second peak in older adults, particularly those over 55 years[10][11].

2. Gender:
   - The disease has a slight male predominance, although it can affect individuals of any gender[12].

3. Histological Features:
   - Histologically, NSHL is characterized by the presence of Reed-Sternberg cells within a background of fibrous tissue and inflammatory cells. This distinctive histological pattern is crucial for diagnosis[13][14].

4. Staging:
   - The clinical stage at diagnosis can vary, with many patients presenting with early-stage disease (Stage I or II). However, advanced stages (Stage III or IV) are also common, particularly in those with splenic involvement[15].

5. Prognosis:
   - The prognosis for patients with NSHL is generally favorable, especially when diagnosed at an early stage. Treatment options typically include chemotherapy, radiation therapy, or a combination of both, depending on the stage and individual patient factors[16][17].

Conclusion

Nodular sclerosis Hodgkin lymphoma, particularly with splenic involvement (ICD-10 code C81.17), presents with a range of clinical signs and symptoms, including lymphadenopathy, splenomegaly, and systemic B symptoms. Understanding the patient demographics and histological characteristics is essential for accurate diagnosis and effective treatment planning. Early detection and appropriate management can lead to favorable outcomes for patients diagnosed with this subtype of Hodgkin lymphoma.

For further information or specific case studies, consulting oncology resources or clinical guidelines may provide additional insights into the management of NSHL.

Nodular sclerosis Hodgkin lymphoma (NSHL) is a subtype of Hodgkin lymphoma characterized by specific histological features. The ICD-10 code C81.17 specifically refers to this condition when it involves the spleen. Below are alternative names and related terms associated with this diagnosis.

Alternative Names for Nodular Sclerosis Hodgkin Lymphoma

1. Nodular Sclerosis Lymphoma: This term is often used interchangeably with nodular sclerosis Hodgkin lymphoma, emphasizing the nodular pattern observed in the lymphatic tissue.

2. Nodular Sclerosis Type of Hodgkin Lymphoma: This phrase highlights that it is a specific type of Hodgkin lymphoma, distinguishing it from other subtypes.

3. Hodgkin's Disease, Nodular Sclerosis Variant: This term reflects the historical nomenclature of Hodgkin lymphoma, where it was commonly referred to as Hodgkin's disease.

4. Sclerosing Hodgkin Lymphoma: This name emphasizes the sclerotic (hardened) nature of the lymphoid tissue in this subtype.

Related Terms

1. Hodgkin Lymphoma: A broader term that encompasses all subtypes of Hodgkin lymphoma, including nodular sclerosis.

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

3. Splenic Involvement: This term refers to the presence of lymphoma in the spleen, which is specifically noted in the context of C81.17.

4. Stage II Hodgkin Lymphoma: If the nodular sclerosis Hodgkin lymphoma is localized to the spleen and nearby lymph nodes, it may be classified under this staging.

5. Histological Subtype: Refers to the specific microscopic characteristics of the lymphoma, which in the case of NSHL, includes the presence of Reed-Sternberg cells and fibrous tissue.

6. Reed-Sternberg Cells: These are the characteristic cells found in Hodgkin lymphoma, including nodular sclerosis, and are crucial for diagnosis.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C81.17 is essential for accurate diagnosis, coding, and communication among healthcare professionals. This knowledge aids in ensuring proper treatment and management of patients with nodular sclerosis Hodgkin lymphoma, particularly when it involves the spleen. If you need further details or specific coding guidelines, feel free to ask!

Nodular sclerosis Hodgkin lymphoma (NSHL) is a subtype of Hodgkin lymphoma characterized by specific histological features and clinical presentations. The ICD-10 code C81.17 specifically refers to nodular sclerosis Hodgkin lymphoma that involves the spleen. Understanding the diagnostic criteria for this condition is crucial for accurate coding and treatment planning.

Diagnostic Criteria for Nodular Sclerosis Hodgkin Lymphoma

1. Clinical Presentation

• Symptoms: Patients may present with symptoms such as painless lymphadenopathy, fever, night sweats, and weight loss, commonly referred to as "B symptoms." Splenic involvement may lead to splenomegaly, which can be detected during a physical examination or imaging studies[1].
• Age and Gender: NSHL typically occurs in young adults, with a slight predominance in females. The age of onset is often between 15 and 35 years[2].

2. Histopathological Examination

• Biopsy: A definitive diagnosis requires a biopsy of the affected lymph node or spleen. The histological examination will reveal characteristic features of NSHL, including:
  • Reed-Sternberg Cells: These are large, atypical lymphoid cells that are a hallmark of Hodgkin lymphoma.
  • Nodular Architecture: The presence of fibrous bands creating nodular structures is typical in NSHL.
  • Lymphocyte Depletion: There is often a background of varying degrees of lymphocyte depletion, which can be assessed through immunohistochemical staining[3].

3. Imaging Studies

• CT or PET Scans: Imaging studies such as computed tomography (CT) or positron emission tomography (PET) scans are utilized to assess the extent of disease, including splenic involvement. These imaging modalities help in staging the disease and determining the presence of lymphadenopathy or organ involvement[4].

4. Staging

• Ann Arbor Staging System: The Ann Arbor classification is used to stage Hodgkin lymphoma, which is essential for treatment planning. The presence of splenic involvement typically indicates at least stage II disease, depending on the extent of lymph node involvement and other factors[5].

5. Laboratory Tests

• Blood Tests: Routine blood tests may show anemia, elevated erythrocyte sedimentation rate (ESR), and other nonspecific findings. However, these tests are not diagnostic but can provide supportive information regarding the patient's overall health status[6].

Conclusion

The diagnosis of nodular sclerosis Hodgkin lymphoma with splenic involvement (ICD-10 code C81.17) relies on a combination of clinical evaluation, histopathological findings, imaging studies, and staging criteria. Accurate diagnosis is essential for appropriate treatment and management of the disease. If you have further questions or need more detailed information on treatment options or management strategies, feel free to ask!

Nodular sclerosis Hodgkin lymphoma (NSHL), particularly when it involves the spleen, is a subtype of Hodgkin lymphoma characterized by specific histological features. The treatment approaches for this condition are multifaceted and depend on various factors, including the stage of the disease, the patient's overall health, and specific clinical guidelines. Below is a detailed overview of standard treatment approaches for NSHL, particularly focusing on cases coded under ICD-10 C81.17.

Overview of Nodular Sclerosis Hodgkin Lymphoma

Nodular sclerosis Hodgkin lymphoma is the most common subtype of Hodgkin lymphoma, accounting for approximately 60-80% of all cases. It typically presents with lymphadenopathy, splenomegaly, and systemic symptoms such as fever, night sweats, and weight loss. The presence of the disease in the spleen can indicate more advanced disease and may influence treatment decisions.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy is the cornerstone of treatment for NSHL. The most commonly used regimens include:

• ABVD Regimen: This includes Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. ABVD is often the first-line treatment for early-stage and advanced-stage Hodgkin lymphoma due to its efficacy and relatively favorable side effect profile[1].

• BEACOPP Regimen: This regimen (Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, and Prednisone) is more intensive and may be used in certain cases, particularly for advanced-stage disease or in patients with high-risk features[2].

2. Radiation Therapy

Radiation therapy may be employed in conjunction with chemotherapy, especially for localized disease. It is particularly effective in early-stage NSHL and can be used to target specific areas, such as the spleen, if involved. The use of involved-field radiation therapy (IFRT) is common, which focuses on the areas of known disease while minimizing exposure to surrounding healthy tissues[3].

3. Stem Cell Transplantation

For patients with relapsed or refractory NSHL, autologous stem cell transplantation (ASCT) may be considered. This approach is typically reserved for those who have not responded to initial treatments or have experienced a relapse after chemotherapy[4]. 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.

4. Targeted Therapy and Immunotherapy

Recent advancements in treatment have introduced targeted therapies and immunotherapies:

• Brentuximab Vedotin (Adcetris): This is an antibody-drug conjugate that targets CD30, a marker expressed on Hodgkin lymphoma cells. It is often used in cases of relapsed or refractory disease and can be combined with chemotherapy or used as a single agent[5].

• Checkpoint Inhibitors: Drugs such as Pembrolizumab and Nivolumab, which target PD-1, have shown promise in treating relapsed or refractory Hodgkin lymphoma. These agents work by enhancing the immune response against cancer cells[6].

Conclusion

The treatment of nodular sclerosis Hodgkin lymphoma, particularly when involving the spleen, is a dynamic process that typically involves a combination of chemotherapy, radiation therapy, and potentially stem cell transplantation or targeted therapies. The choice of treatment is tailored to the individual patient based on disease characteristics and overall health. Ongoing research continues to refine these approaches, aiming to improve outcomes and reduce long-term side effects for patients with this condition.

For patients diagnosed with NSHL, it is crucial to discuss treatment options with a healthcare provider specializing in oncology to determine the most appropriate and effective treatment plan based on the latest clinical guidelines and research findings.

References

1. [Chemotherapy regimens for Hodgkin lymphoma]
2. [BEACOPP regimen details]
3. [Radiation therapy in Hodgkin lymphoma]
4. [Stem cell transplantation in Hodgkin lymphoma]
5. [Brentuximab Vedotin in Hodgkin lymphoma]
6. [Checkpoint inhibitors in Hodgkin lymphoma]