ICD-10: C81.47

Lymphocyte-rich Hodgkin lymphoma, spleen

Additional Information

Diagnostic Criteria

Lymphocyte-rich Hodgkin lymphoma (LRHL) is a subtype of Hodgkin lymphoma characterized by a predominance of lymphocytes and is classified under the ICD-10 code C81.47, specifically indicating its involvement in the spleen. The diagnosis of LRHL involves several criteria, including clinical evaluation, histopathological examination, and imaging studies. Below is a detailed overview of the diagnostic criteria and considerations for this condition.

Clinical Evaluation

  1. Symptoms: Patients may present with common symptoms associated with Hodgkin lymphoma, such as:
    - Painless lymphadenopathy (swelling of lymph nodes)
    - Fever
    - Night sweats
    - Weight loss
    - Fatigue

  2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy and splenomegaly (enlargement of the spleen), which are indicative of lymphatic involvement.

Histopathological Examination

  1. Biopsy: A definitive diagnosis of LRHL requires a biopsy of the affected lymph node or spleen tissue. The biopsy can be performed through:
    - Excisional biopsy (removal of an entire lymph node)
    - Core needle biopsy (removal of a small cylinder of tissue)

  2. Microscopic Analysis: The histopathological examination will reveal:
    - A predominance of lymphocytes, particularly small, mature lymphocytes.
    - Reed-Sternberg cells, which are characteristic of Hodgkin lymphoma, may be present but are typically fewer in LRHL compared to other subtypes.
    - A mixed cellularity background with varying degrees of fibrosis.

  3. Immunohistochemistry: Immunophenotyping is performed to confirm the diagnosis. Key markers include:
    - CD30 positive (a hallmark of Hodgkin lymphoma)
    - CD15 positive
    - CD45 positive (indicating lymphocyte origin)
    - Other markers may be assessed to differentiate LRHL from other lymphomas.

Imaging Studies

  1. CT Scans: Computed tomography (CT) scans of the chest, abdomen, and pelvis are utilized to assess the extent of disease and to identify any lymphadenopathy or splenic involvement.

  2. PET Scans: Positron emission tomography (PET) scans may be used to evaluate metabolic activity in lymph nodes and the spleen, helping to determine the stage of the disease.

Staging

The Ann Arbor staging system is commonly used to classify the extent of Hodgkin lymphoma, including LRHL. Staging involves:
- Stage I: Involvement of a single lymph node region or a single extralymphatic site.
- Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm.
- Stage III: Involvement of lymph node regions on both sides of the diaphragm.
- Stage IV: Disseminated involvement of one or more extralymphatic organs.

Conclusion

The diagnosis of lymphocyte-rich Hodgkin lymphoma, particularly when involving the spleen, is a multifaceted process that requires careful clinical assessment, histopathological confirmation, and imaging studies to determine the extent of the disease. Accurate diagnosis is crucial for guiding treatment decisions and improving patient outcomes. If you have further questions or need additional information on treatment options or management strategies, feel free to ask!

Treatment Guidelines

Lymphocyte-rich Hodgkin lymphoma (LRHL), classified under ICD-10 code C81.47, is a rare subtype of Hodgkin lymphoma characterized by a predominance of lymphocytes and a relatively low number of Reed-Sternberg cells. The treatment approaches for this specific type of lymphoma typically align with the general strategies used for Hodgkin lymphoma but may be tailored based on the unique characteristics of LRHL.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone of treatment for Hodgkin lymphoma, including LRHL. 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 effectiveness and manageable side effects[1].

  • BEACOPP Regimen: This regimen (Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, and Prednisone) is more intensive and may be considered for patients with advanced disease or those at high risk of treatment failure[2].

2. Radiation Therapy

Radiation therapy can be an effective treatment modality, particularly for localized disease. It is often used in conjunction with chemotherapy, especially in early-stage cases where the disease is confined to a specific area, such as the spleen. The use of involved-field radiation therapy (IFRT) targets only the areas affected by the lymphoma, minimizing exposure to surrounding healthy tissues[3].

3. Stem Cell Transplantation

For patients with relapsed or refractory LRHL, high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be considered. This approach allows for the administration of higher doses of chemotherapy than would be tolerable without the support of stem cell rescue[4].

4. Targeted Therapy

Brentuximab vedotin (Adcetris®) is a targeted therapy that has shown efficacy in treating Hodgkin lymphoma, particularly in cases that are refractory to standard chemotherapy. It is an antibody-drug conjugate that targets CD30, a protein expressed on Reed-Sternberg cells. This treatment may be considered for patients with advanced or relapsed disease[5].

5. Clinical Trials

Given the rarity of lymphocyte-rich Hodgkin lymphoma, participation in clinical trials may be an option for patients. These trials often explore new treatment combinations or novel therapies that could provide additional benefits over standard approaches[6].

Conclusion

The treatment of lymphocyte-rich Hodgkin lymphoma, particularly when involving the spleen, typically involves a combination of chemotherapy, radiation therapy, and potentially stem cell transplantation or targeted therapies. The choice of treatment is influenced by various factors, including the stage of the disease, the patient's overall health, and the presence of any comorbid conditions. As with all cancers, a multidisciplinary approach involving oncologists, radiologists, and other healthcare professionals is essential to optimize outcomes for patients with this rare lymphoma subtype.

For the most current treatment options and clinical trials, consulting with a healthcare provider or a specialist in hematologic malignancies is recommended.

Description

Lymphocyte-rich Hodgkin lymphoma (LRHL) is a subtype of Hodgkin lymphoma characterized by a predominance of lymphocytes in the tumor microenvironment. The ICD-10 code C81.47 specifically refers to cases of lymphocyte-rich Hodgkin lymphoma that are localized to the spleen. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Lymphocyte-Rich Hodgkin Lymphoma

Definition and Characteristics

Lymphocyte-rich Hodgkin lymphoma is a rare variant of Hodgkin lymphoma, which is a type of cancer that originates in the lymphatic system. This subtype is distinguished by the following features:

  • Histological Features: The histological examination reveals a high number of lymphocytes, with fewer Reed-Sternberg cells compared to other subtypes of Hodgkin lymphoma. The presence of a rich lymphocytic background is a hallmark of this variant[1].
  • Clinical Presentation: Patients may present with symptoms typical of Hodgkin lymphoma, including lymphadenopathy (swollen lymph nodes), fever, night sweats, and weight loss. However, lymphocyte-rich Hodgkin lymphoma may also present with splenomegaly (enlargement of the spleen) as a prominent feature[1][2].

Epidemiology

Lymphocyte-rich Hodgkin lymphoma is relatively uncommon, accounting for a small percentage of all Hodgkin lymphoma cases. It tends to occur more frequently in males and is often diagnosed in younger adults, although it can occur at any age[2].

Diagnosis

Diagnosis of lymphocyte-rich Hodgkin lymphoma involves:

  • Imaging Studies: CT scans or PET scans may be utilized to assess the extent of disease, particularly in the spleen and surrounding lymphatic tissues.
  • Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph node or spleen tissue, followed by histopathological examination to identify the characteristic lymphocyte-rich composition[1][3].

Treatment

The treatment for lymphocyte-rich Hodgkin lymphoma typically involves:

  • Chemotherapy: The standard treatment regimen may include chemotherapy, often using a combination of drugs such as ABVD (Adriamycin, Bleomycin, Vinblastine, and Dacarbazine).
  • Radiation Therapy: In some cases, radiation therapy may be employed, particularly if the disease is localized to the spleen or if there is residual disease after chemotherapy[2][3].

Prognosis

The prognosis for patients with lymphocyte-rich Hodgkin lymphoma is generally favorable, with many patients achieving remission. Factors influencing prognosis include the stage of the disease at diagnosis and the patient's overall health[2].

Conclusion

ICD-10 code C81.47 designates lymphocyte-rich Hodgkin lymphoma localized to the spleen, a subtype characterized by a predominance of lymphocytes and a favorable prognosis with appropriate treatment. Understanding the clinical features, diagnostic criteria, and treatment options is essential for effective management of this condition. For further information, healthcare providers may refer to clinical guidelines and local coverage determinations related to Hodgkin lymphoma treatment[1][2][3].

Clinical Information

Lymphocyte-rich Hodgkin lymphoma (LRHL), classified under ICD-10 code C81.47, is a subtype of Hodgkin lymphoma characterized by a predominance of lymphocytes. This specific type of lymphoma can involve the spleen, leading to a range of clinical presentations, signs, symptoms, and patient characteristics. Below is a detailed overview of these aspects.

Clinical Presentation

General Overview

Lymphocyte-rich Hodgkin lymphoma is known for its relatively favorable prognosis compared to other subtypes of Hodgkin lymphoma. It typically presents in younger adults, although it can occur at any age. The disease often manifests with lymphadenopathy, splenomegaly, and systemic symptoms.

Signs and Symptoms

  1. Lymphadenopathy:
    - The most common initial presentation is painless swelling of lymph nodes, particularly in the cervical, axillary, or inguinal regions. In cases involving the spleen, splenic enlargement may also be noted.

  2. Splenomegaly:
    - Patients may experience an enlarged spleen, which can be detected during a physical examination or imaging studies. This enlargement can lead to discomfort or a feeling of fullness in the abdomen.

  3. Systemic Symptoms:
    - Common systemic symptoms include:

    • Fever: Often low-grade and intermittent.
    • Night Sweats: Profuse sweating during the night.
    • Weight Loss: Unintentional weight loss over a short period.
    • Fatigue: Persistent tiredness that does not improve with rest.
  4. Other Symptoms:
    - Patients may also report pruritus (itching), especially in the absence of a rash, and may experience pain in lymph nodes after alcohol consumption, a phenomenon known as "alcohol-induced pain."

Patient Characteristics

Demographics

  • Age: LRHL typically affects younger adults, with a peak incidence in individuals aged 15 to 35 years. However, it can also occur in older adults.
  • Gender: There is a slight male predominance in the incidence of Hodgkin lymphoma, including the lymphocyte-rich subtype.

Risk Factors

  • Family History: A family history of Hodgkin lymphoma or other lymphoproliferative disorders may increase risk.
  • Immune System Status: Patients with compromised immune systems, such as those with HIV/AIDS or those on immunosuppressive therapy, may be at higher risk for developing Hodgkin lymphoma.

Histological Features

  • Lymphocyte Predominance: Histologically, LRHL is characterized by a high number of lymphocytes, with Reed-Sternberg cells being less prominent compared to other subtypes of Hodgkin lymphoma. This histological feature is crucial for diagnosis and differentiating LRHL from other types of lymphoma.

Conclusion

Lymphocyte-rich Hodgkin lymphoma, particularly when involving the spleen, presents with a combination of lymphadenopathy, splenomegaly, and systemic symptoms such as fever, night sweats, and weight loss. Understanding the clinical presentation and patient characteristics is essential for timely diagnosis and management. If you suspect symptoms consistent with LRHL, it is crucial to seek medical evaluation for appropriate diagnostic testing and treatment options.

Approximate Synonyms

Lymphocyte-rich Hodgkin lymphoma (LRHL) is a subtype of Hodgkin lymphoma characterized by a predominance of lymphocytes. The ICD-10 code for this specific condition is C81.47, which denotes lymphocyte-rich Hodgkin lymphoma located in the spleen. Below are alternative names and related terms associated with this diagnosis.

Alternative Names for Lymphocyte-rich Hodgkin Lymphoma

  1. Lymphocyte-predominant Hodgkin Lymphoma (LPHL): This term is often used interchangeably with lymphocyte-rich Hodgkin lymphoma, although LPHL is a broader category that includes various presentations of the disease.

  2. Nodular Lymphocyte-Predominant Hodgkin Lymphoma (NLPHL): This is a specific variant of lymphocyte-predominant Hodgkin lymphoma that is characterized by nodular growth patterns.

  3. Hodgkin's Disease, Lymphocyte-rich Type: This is a more general term that may be used in clinical settings to describe the same condition.

  1. Hodgkin Lymphoma: This is the overarching term for a group of blood cancers that originate from lymphatic cells, including various subtypes such as lymphocyte-rich Hodgkin lymphoma.

  2. Splenic Involvement: This term refers to the presence of lymphoma in the spleen, which is specifically noted in the ICD-10 code C81.47.

  3. Lymphoma: A general term for cancers that affect the lymphatic system, which includes Hodgkin lymphoma and its subtypes.

  4. B-cell Lymphoma: Since Hodgkin lymphoma is derived from B-lymphocytes, this term is relevant in the context of its cellular origin.

  5. Stage IV Hodgkin Lymphoma: If lymphocyte-rich Hodgkin lymphoma has spread to the spleen, it may be classified as stage IV, indicating advanced disease.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C81.47 is essential for accurate diagnosis, treatment planning, and communication among healthcare professionals. These terms help clarify the specific subtype of Hodgkin lymphoma and its implications for patient care. If you need further information or specific details about treatment options or prognosis, feel free to ask!

Related Information

Diagnostic Criteria

  • Painless lymphadenopathy
  • Fever and night sweats
  • Weight loss and fatigue
  • Lymphadenopathy and splenomegaly
  • Predominance of small mature lymphocytes
  • Reed-Sternberg cells present but fewer
  • Mixed cellularity background with fibrosis
  • CD30 positive, CD15 positive
  • CD45 positive for lymphocyte origin

Treatment Guidelines

  • ABVD Regimen standard first-line chemotherapy
  • BEACOPP Regimen for advanced high-risk disease
  • Radiation Therapy localized involved-field
  • Stem Cell Transplantation relapsed refractory cases
  • Brentuximab vedotin targeted therapy for advanced
  • Clinical Trials explore new treatment combinations

Description

Clinical Information

  • Painless lymph node swelling
  • Enlarged spleen detected by exam or imaging
  • Low-grade intermittent fever
  • Profuse night sweats
  • Unintentional weight loss
  • Persistent fatigue
  • Pruritus without rash
  • Alcohol-induced pain in lymph nodes
  • Younger adults typically affected
  • Male predominance in incidence
  • Family history of lymphoma increases risk
  • Compromised immune system may increase risk

Approximate Synonyms

  • Lymphocyte-predominant Hodgkin Lymphoma
  • Nodular Lymphocyte-Predominant Hodgkin Lymphoma
  • Hodgkin's Disease, Lymphocyte-rich Type
  • Hodgkin Lymphoma
  • Splenic Involvement
  • Lymphoma
  • B-cell Lymphoma
  • Stage IV Hodgkin Lymphoma

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