ICD-10: C81.07

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a distinct subtype of Hodgkin lymphoma characterized by the presence of large atypical lymphoid cells known as "popcorn cells." This type of lymphoma is notable for its relatively indolent behavior compared to other forms of Hodgkin lymphoma, and it often presents with localized disease.

Clinical Description of ICD-10 Code C81.07

Definition and Characteristics

ICD-10 code C81.07 specifically refers to Nodular lymphocyte predominant Hodgkin lymphoma that is primarily located in the spleen. This subtype is recognized for its unique histological features, which include:

• Popcorn Cells: These are large, atypical Reed-Sternberg-like cells that are often surrounded by a background of small lymphocytes.
• Lymphoid Architecture: The lymphoid tissue architecture is typically preserved, with nodular patterns that can be mistaken for reactive lymphoid hyperplasia.

Epidemiology

NLPHL is relatively rare, accounting for approximately 5% of all Hodgkin lymphoma cases. It predominantly affects younger adults, with a median age of diagnosis in the mid-30s. There is a slight male predominance in the incidence of this lymphoma.

Symptoms

Patients with NLPHL may present with:

• Lymphadenopathy: Swelling of lymph nodes, which may be localized or generalized.
• Splenomegaly: Enlargement of the spleen, which is specifically noted in cases coded as C81.07.
• B Symptoms: These include fever, night sweats, and unexplained weight loss, although they are less common in NLPHL compared to other types of Hodgkin lymphoma.

Diagnosis

Diagnosis of NLPHL typically involves:

• Histopathological Examination: A biopsy of the affected lymphoid tissue, often revealing the characteristic popcorn cells.
• Imaging Studies: CT scans or PET scans may be used to assess the extent of disease, particularly in the spleen and other lymphatic regions.

Treatment

The treatment approach for NLPHL may include:

• Watchful Waiting: In cases where the disease is asymptomatic and localized.
• Chemotherapy: Regimens may include ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine) or other combinations, depending on the stage and symptoms.
• Radiation Therapy: Often used in localized disease, particularly if the spleen is involved.

Prognosis

The prognosis for patients with NLPHL is generally favorable, especially when diagnosed early. The five-year survival rate is high, and the disease tends to have a more indolent course compared to classical Hodgkin lymphoma.

Conclusion

ICD-10 code C81.07 encapsulates the clinical details of nodular lymphocyte predominant Hodgkin lymphoma affecting the spleen. Understanding the characteristics, symptoms, and treatment options for this specific subtype is crucial for effective management and improved patient outcomes. As with all lymphomas, early diagnosis and tailored treatment strategies are key to achieving the best possible prognosis.

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a distinct subtype of Hodgkin lymphoma characterized by specific clinical presentations, signs, symptoms, and patient demographics. The ICD-10 code C81.07 specifically refers to NLPHL involving the spleen. 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, axillary, or inguinal regions. In NLPHL, lymphadenopathy may be more localized compared to other types of Hodgkin lymphoma[1][2].

2. Splenomegaly:
   - As indicated by the ICD-10 code C81.07, involvement of the spleen is a hallmark of this subtype. Patients may present with an enlarged spleen, which can be detected during a physical examination or imaging studies[3].

3. B Symptoms:
   - Some patients may experience systemic symptoms known as "B symptoms," which include:

   • Unexplained fever
   • Night sweats
   • Significant weight loss (more than 10% of body weight over six months) [4].

4. Fatigue:
   - Patients often report a general sense of fatigue or malaise, which can be attributed to the disease process or anemia associated with lymphoma[5].

5. Pruritus:
   - Itching without an apparent rash can occur in some patients, although it is less common in NLPHL compared to other types of Hodgkin lymphoma[6].

Patient Characteristics

1. Demographics:
   - NLPHL typically affects younger adults, with a median age of diagnosis in the late 20s to early 30s. However, it can occur in older adults as well[7].

2. Gender:
   - There is a slight male predominance in NLPHL cases, although the difference is not as pronounced as in other types of Hodgkin lymphoma[8].

3. Histological Features:
   - The disease is characterized by the presence of large, atypical Reed-Sternberg-like cells, which are often surrounded by a background of small lymphocytes. This histological pattern is crucial for diagnosis and differentiates NLPHL from classical Hodgkin lymphoma[9].

4. Prognosis:
   - NLPHL generally has a favorable prognosis compared to classical Hodgkin lymphoma, with a higher overall survival rate. However, the presence of splenic involvement may indicate a more advanced stage of disease, which can influence treatment decisions and outcomes[10].

Conclusion

Nodular lymphocyte predominant Hodgkin lymphoma, particularly when involving the spleen (ICD-10 code C81.07), presents with specific clinical features such as lymphadenopathy, splenomegaly, and potential B symptoms. Understanding these characteristics is essential for accurate diagnosis and effective management. Given its generally favorable prognosis, early detection and appropriate treatment are crucial for improving patient outcomes. If you have further questions or need additional information, feel free to ask!

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a specific subtype of Hodgkin lymphoma characterized by the predominance of nodular lymphocytes. The ICD-10 code C81.07 specifically refers to NLPHL that is located in the spleen. Below are alternative names and related terms associated with this condition.

Alternative Names for NLPHL

1. Nodular Lymphocyte Predominant Hodgkin Disease: This term is often used interchangeably with NLPHL and emphasizes the disease aspect.
2. Nodular Lymphocyte Predominant Lymphoma: This name highlights the lymphomatous nature of the condition.
3. NLPHL: The acronym for Nodular Lymphocyte Predominant Hodgkin Lymphoma is commonly used in clinical settings.

Related Terms

1. Hodgkin Lymphoma: A broader category that includes NLPHL as one of its subtypes, alongside classic Hodgkin lymphoma.
2. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.
3. Splenic Lymphoma: While not specific to NLPHL, this term refers to lymphomas that primarily affect the spleen, which is relevant given the specific ICD-10 code C81.07.
4. Lymphocyte Predominant Hodgkin Lymphoma: This term is sometimes used to describe the same condition, focusing on the lymphocyte predominance.

Clinical Context

NLPHL is distinct from classic Hodgkin lymphoma due to its unique histological features and clinical behavior. It typically presents with a more indolent course and may require different management strategies. Understanding these alternative names and related terms is crucial for accurate diagnosis, treatment planning, and coding in medical records.

In summary, the ICD-10 code C81.07 for Nodular lymphocyte predominant Hodgkin lymphoma of the spleen is associated with various alternative names and related terms that reflect its classification within the broader category of lymphomas. These terms are essential for healthcare professionals in ensuring precise communication and documentation.

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a distinct subtype of Hodgkin lymphoma characterized by specific clinical and pathological features. The diagnosis of NLPHL, particularly when it involves the spleen, follows established criteria that integrate clinical evaluation, imaging studies, and histopathological examination. Below is a detailed overview of the criteria used for diagnosing NLPHL, specifically for the ICD-10 code C81.07.

Clinical Presentation

Symptoms

Patients with NLPHL may present with:
- Lymphadenopathy: Painless swelling of lymph nodes, often in the neck, axilla, or groin.
- 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, although they are less common in NLPHL compared to other types of Hodgkin lymphoma.

Patient History

A thorough medical history is essential, including:
- Duration and progression of symptoms.
- Any previous history of lymphoproliferative disorders.
- Family history of lymphomas or related conditions.

Imaging Studies

Radiological Evaluation

Imaging studies play a crucial role in the diagnosis and staging of NLPHL:
- CT Scans: Chest, abdomen, and pelvis CT scans are typically performed to assess lymphadenopathy and splenic involvement.
- PET Scans: Positron emission tomography (PET) scans may be utilized to evaluate metabolic activity in lymph nodes and the spleen, helping to distinguish between active disease and residual masses.

Histopathological Examination

Biopsy

A definitive diagnosis of NLPHL requires a biopsy of affected lymph nodes or spleen tissue. The following histological features are critical:
- Lymphocyte Predominance: The presence of a predominance of lymphocytes, particularly small, mature lymphocytes, is a hallmark of NLPHL.
- Reed-Sternberg-like Cells: The presence of atypical cells resembling Reed-Sternberg cells, but with a distinct morphology, is characteristic.
- Background: A mixed inflammatory background with a predominance of small lymphocytes and occasional eosinophils is often observed.

Immunohistochemistry

Immunophenotyping is essential for confirming the diagnosis:
- CD20 Positive: NLPHL cells typically express CD20, a B-cell marker.
- CD15 and CD30 Negative: Unlike classical Hodgkin lymphoma, NLPHL cells are usually negative for CD15 and CD30, which helps differentiate it from other subtypes.

Staging and Classification

Ann Arbor Staging System

Once diagnosed, NLPHL is staged using the Ann Arbor classification, which considers the number of affected lymph node regions and the presence of systemic symptoms. The involvement of the spleen would classify it as stage II or higher, depending on the extent of disease.

Conclusion

The diagnosis of nodular lymphocyte predominant Hodgkin lymphoma, particularly with splenic involvement (ICD-10 code C81.07), relies on a combination of clinical evaluation, imaging studies, and histopathological findings. The integration of these criteria ensures accurate diagnosis and appropriate management of this unique lymphoma subtype. For further information or specific case discussions, consultation with a hematologist or oncologist specializing in lymphoproliferative disorders is recommended.

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL), classified under ICD-10 code C81.07, is a rare subtype of Hodgkin lymphoma characterized by the presence of large, atypical Reed-Sternberg cells and a predominance of lymphocytes. The treatment approaches for NLPHL can vary based on the stage of the disease, the patient's overall health, and specific clinical factors. Below is a detailed overview of standard treatment strategies for NLPHL.

Treatment Overview

1. Initial Assessment and Staging

Before initiating treatment, a thorough assessment is essential. This includes:
- Imaging Studies: CT scans or PET scans to determine the extent of the disease.
- Biopsy: Confirmation of diagnosis through lymph node biopsy.
- Laboratory Tests: Blood tests to evaluate overall health and organ function.

2. Treatment Modalities

The treatment for NLPHL typically involves a combination of the following approaches:

A. Chemotherapy

Chemotherapy is often the first-line treatment for NLPHL, especially in advanced stages. Common regimens include:
- ABVD Regimen: This includes Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. It is frequently used for various types of Hodgkin lymphoma.
- Other Regimens: Depending on the patient's specific situation, alternative regimens may be considered.

B. Radiation Therapy

Radiation therapy may be employed in conjunction with chemotherapy or as a standalone treatment, particularly for localized disease. It is effective in targeting specific areas where the lymphoma is present, such as the spleen in cases of C81.07.

C. Surgery

While surgery is not a primary treatment for NLPHL, it may be indicated in certain cases, particularly for diagnostic purposes or to remove localized disease. Splenectomy (removal of the spleen) may be considered if the spleen is significantly involved.

3. Targeted Therapy and Immunotherapy

Recent advancements in treatment have introduced targeted therapies and immunotherapies:
- Rituximab: This monoclonal antibody targets CD20 on B-cells and may be used in combination with chemotherapy for NLPHL.
- Brentuximab Vedotin: This is an antibody-drug conjugate that targets CD30, which may be considered in relapsed or refractory cases.

4. Follow-Up Care

Post-treatment monitoring is crucial to assess for recurrence and manage any long-term side effects of treatment. Regular follow-up appointments typically include:
- Physical Examinations: To check for signs of recurrence.
- Imaging Studies: Periodic scans to monitor for any new developments.

Conclusion

The management of nodular lymphocyte predominant Hodgkin lymphoma (ICD-10 code C81.07) involves a multidisciplinary approach tailored to the individual patient. Treatment typically includes chemotherapy, radiation therapy, and may incorporate targeted therapies as needed. Continuous follow-up is essential to ensure the best outcomes and to address any potential complications arising from treatment. As research progresses, new therapies may emerge, offering additional options for patients with this rare lymphoma subtype.