ICD-10: C81.4

Lymphocyte-rich Hodgkin lymphoma

Clinical Information

Inclusion Terms

  • Lymphocyte-rich classical Hodgkin lymphoma

Additional Information

Description

Lymphocyte-rich Hodgkin lymphoma (LRHL) is a subtype of Hodgkin lymphoma characterized by a predominance of lymphocytes in the tumor microenvironment. This specific type is classified under the ICD-10-CM code C81.4, which is used for diagnostic coding in clinical settings.

Clinical Description

Definition and Characteristics

Lymphocyte-rich Hodgkin lymphoma is defined by the presence of a significant number of lymphocytes, with fewer Reed-Sternberg cells compared to other subtypes of Hodgkin lymphoma. This subtype is often associated with a better prognosis than other forms of Hodgkin lymphoma, such as mixed cellularity or lymphocyte-depleted types. The histological features typically include:

  • Lymphocyte Predominance: A high ratio of lymphocytes to Reed-Sternberg cells.
  • Reed-Sternberg Cells: These are large, abnormal lymphocytes that are a hallmark of Hodgkin lymphoma, but they are less prevalent in this subtype.
  • Fibrosis: There may be varying degrees of fibrosis in the tumor microenvironment.

Epidemiology

LRHL is relatively rare compared to other subtypes of Hodgkin lymphoma. It tends to occur more frequently in males and is often diagnosed in younger adults, typically between the ages of 15 and 35. The incidence of this subtype is lower than that of classic Hodgkin lymphoma, which includes mixed cellularity and nodular sclerosis types.

Symptoms

Patients with lymphocyte-rich Hodgkin lymphoma may present with symptoms similar to other forms of Hodgkin lymphoma, including:

  • Lymphadenopathy: Swelling of lymph nodes, often in the neck, armpits, or groin.
  • Fever: Unexplained fevers that may come and go.
  • 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.

Diagnosis

Diagnosis of LRHL typically involves a combination of clinical evaluation, imaging studies (such as CT scans), and histopathological examination of lymph node biopsies. The biopsy is crucial for identifying the characteristic histological features of the lymphoma.

Treatment

The treatment for lymphocyte-rich Hodgkin lymphoma may include:

  • Chemotherapy: Often the first line of treatment, using regimens such as ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine).
  • Radiation Therapy: May be used in conjunction with chemotherapy, especially in localized cases.
  • Targeted Therapy: In some cases, targeted therapies may be considered based on the individual patient's characteristics and disease progression.

Prognosis

The prognosis for patients with lymphocyte-rich Hodgkin lymphoma is generally favorable, with higher survival rates compared to other subtypes. Factors influencing prognosis include the stage of the disease at diagnosis, the patient's age, and overall health.

Conclusion

ICD-10 code C81.4 is specifically designated for lymphocyte-rich Hodgkin lymphoma, a subtype that is characterized by a predominance of lymphocytes and a relatively favorable prognosis. Understanding the clinical features, diagnostic criteria, and treatment options is essential for effective management of this condition. As with all cancers, early detection and tailored treatment strategies are critical for improving patient outcomes.

Clinical Information

Lymphocyte-rich Hodgkin lymphoma (LR-cHL), classified under ICD-10 code C81.4, is a subtype of Hodgkin lymphoma characterized by a predominance of lymphocytes. This form of lymphoma presents unique clinical features, signs, symptoms, and patient demographics that distinguish it from other types of Hodgkin lymphoma.

Clinical Presentation

Signs and Symptoms

Patients with lymphocyte-rich Hodgkin lymphoma typically exhibit a range of signs and symptoms, which may include:

  • Lymphadenopathy: The most common presentation is painless swelling of lymph nodes, particularly in the cervical (neck) region, axillary (armpit), or mediastinal (chest) areas. This lymph node enlargement can be firm and rubbery in texture[1].
  • B Symptoms: These systemic symptoms include:
  • Fever: Often low-grade and intermittent.
  • Night Sweats: Profuse sweating during the night that may soak clothing and bedding.
  • Weight Loss: Unintentional weight loss exceeding 10% of body weight over six months[2].
  • Fatigue: Patients frequently report a general sense of tiredness or lack of energy, which can be debilitating.
  • Pruritus: Some patients may experience itching, which can be generalized or localized[3].

Patient Characteristics

Lymphocyte-rich Hodgkin lymphoma tends to have specific demographic characteristics:

  • Age: This subtype is more commonly diagnosed in younger adults, particularly those in their 20s and 30s, although it can occur at any age[4].
  • Gender: There is a slight male predominance in the incidence of LR-cHL, similar to other forms of Hodgkin lymphoma[5].
  • Geographic Variability: The prevalence of Hodgkin lymphoma, including LR-cHL, can vary by region, with higher rates reported in certain areas, such as Central and South America[6].

Diagnosis and Characterization

The diagnosis of lymphocyte-rich Hodgkin lymphoma typically involves:

  • Histopathological Examination: A biopsy of the affected lymph node is essential for diagnosis. The histological features include a predominance of lymphocytes with few Reed-Sternberg cells, which are characteristic of Hodgkin lymphoma[7].
  • Imaging Studies: CT scans or PET scans may be utilized to assess the extent of disease and to identify any additional lymph node involvement or extranodal disease[8].

Prognosis and Survival

The prognosis for patients with lymphocyte-rich Hodgkin lymphoma is generally favorable, with studies indicating that this subtype may have a better overall survival rate compared to other types of Hodgkin lymphoma. Factors influencing prognosis include the stage of the disease at diagnosis and the presence of B symptoms[9].

Conclusion

Lymphocyte-rich Hodgkin lymphoma (ICD-10 code C81.4) is characterized by specific clinical presentations, including lymphadenopathy and systemic B symptoms, alongside distinct demographic features. Understanding these aspects is crucial for timely diagnosis and effective management of the disease. As research continues, further insights into the biological behavior and treatment responses of this lymphoma subtype will enhance patient care and outcomes.

Approximate Synonyms

Lymphocyte-rich Hodgkin lymphoma (LRHL), classified under ICD-10 code C81.4, is a subtype of classical Hodgkin lymphoma. This specific type is characterized by a predominance of lymphocytes and is known for its unique histological features. Below are alternative names and related terms associated with this condition.

Alternative Names for Lymphocyte-rich Hodgkin Lymphoma

  1. Lymphocyte-rich Classical Hodgkin Lymphoma: This term emphasizes its classification as a classical subtype of Hodgkin lymphoma while highlighting the lymphocyte-rich nature of the tumor.

  2. Lymphocyte-predominant Hodgkin Lymphoma: Although this term can sometimes refer to a different entity (lymphocyte-predominant Hodgkin lymphoma, which is a distinct subtype), it is occasionally used interchangeably in clinical discussions regarding lymphocyte-rich variants.

  3. Classic Hodgkin Lymphoma, Lymphocyte-rich: This is a descriptive term that specifies the classical type of Hodgkin lymphoma with a lymphocyte-rich histology.

  1. Hodgkin Lymphoma: A broader term that encompasses all types of Hodgkin lymphoma, including lymphocyte-rich Hodgkin lymphoma.

  2. Classical Hodgkin Lymphoma (cHL): This term refers to the main category of Hodgkin lymphoma, which includes several subtypes, including lymphocyte-rich Hodgkin lymphoma.

  3. C81.4: The specific ICD-10 code for lymphocyte-rich Hodgkin lymphoma, which is used for medical billing and coding purposes.

  4. Histological Subtypes of Hodgkin Lymphoma: This term refers to the various histological classifications of Hodgkin lymphoma, including lymphocyte-rich, mixed cellularity, and nodular sclerosis, among others.

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

Understanding these alternative names and related terms is crucial for accurate diagnosis, treatment planning, and communication among healthcare professionals. Each term may carry specific implications regarding the disease's characteristics and management strategies.

Diagnostic Criteria

Lymphocyte-rich Hodgkin lymphoma (LRHL), classified under ICD-10 code C81.4, is a subtype of Hodgkin lymphoma characterized by a predominance of lymphocytes. The diagnosis of LRHL involves a combination of clinical evaluation, histopathological examination, and imaging studies. Below are the key criteria used for its diagnosis:

Clinical Evaluation

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

  2. Physical Examination: A thorough physical examination is conducted to assess lymph node involvement and any other systemic signs.

Histopathological Examination

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

  2. Microscopic Analysis: The histopathological evaluation focuses on:
    - Cellularity: LRHL is characterized by a high number of lymphocytes, with few Reed-Sternberg cells (the hallmark cells of Hodgkin lymphoma).
    - Reed-Sternberg Cells: These cells are typically present in smaller numbers compared to other subtypes of Hodgkin lymphoma.
    - Background: The background of the tissue should show a rich lymphocytic infiltrate, which is a distinguishing feature of LRHL.

  3. Immunohistochemistry: Immunophenotyping is performed to confirm the diagnosis. Key markers include:
    - CD15 and CD30: Typically positive in Reed-Sternberg cells.
    - Lymphocyte markers: Such as CD20 and CD45, which are positive in the lymphocyte-rich background.

Imaging Studies

  1. CT Scans: Imaging studies, particularly computed tomography (CT) scans, are used to assess the extent of disease and to identify any additional lymph node involvement or extranodal disease.

  2. PET Scans: Positron emission tomography (PET) scans may be utilized to evaluate metabolic activity in lymph nodes and to help stage the disease.

Staging

The Ann Arbor staging system is commonly used to determine the extent of Hodgkin lymphoma, including LRHL. Staging involves assessing the number of lymph node regions involved and whether the disease has spread to other organs.

Conclusion

The diagnosis of lymphocyte-rich Hodgkin lymphoma (ICD-10 code C81.4) is a multifaceted process that combines clinical assessment, histopathological findings, and imaging studies. Accurate diagnosis is crucial for determining the appropriate treatment plan and prognosis for the patient. If you have further questions or need more detailed information on specific aspects of LRHL, feel free to ask!

Treatment Guidelines

Lymphocyte-rich Hodgkin lymphoma (LR-cHL), classified under ICD-10 code C81.4, is a subtype of classic Hodgkin lymphoma characterized by a predominance of lymphocytes. This specific type of lymphoma has distinct clinical and pathological features, which influence its treatment approach. Below, we explore the standard treatment strategies for LR-cHL, including chemotherapy, radiation therapy, and emerging therapies.

Overview of Lymphocyte-Rich Hodgkin Lymphoma

LR-cHL is known for its relatively favorable prognosis compared to other subtypes of Hodgkin lymphoma. It typically presents with fewer symptoms and may be diagnosed at an earlier stage. The treatment regimen is often tailored based on the stage of the disease, the patient's overall health, and specific characteristics of the lymphoma.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains the cornerstone of treatment for LR-cHL. The most commonly used regimens include:

  • ABVD Regimen: This includes Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. The ABVD regimen is often the first-line treatment for early-stage and advanced-stage Hodgkin lymphoma, including LR-cHL. Studies have shown that it is effective in achieving remission in many patients[1][2].

  • Escalated BEACOPP: For patients with advanced disease or those at high risk of treatment failure, the escalated BEACOPP regimen (Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, and Prednisone) may be considered. This regimen is more intensive and has been associated with improved outcomes in certain high-risk populations[3].

2. Radiation Therapy

Radiation therapy is often used in conjunction with chemotherapy, particularly for early-stage LR-cHL. The following approaches are common:

  • Involved Field Radiation Therapy (IFRT): This technique targets only the lymph nodes involved in the disease, minimizing exposure to surrounding healthy tissues. IFRT is typically administered after chemotherapy to consolidate the response and reduce the risk of relapse[4].

  • Total Body Irradiation (TBI): In specific cases, particularly when preparing for stem cell transplantation, TBI may be utilized. However, this is less common for LR-cHL compared to other types of Hodgkin lymphoma[5].

3. Emerging Therapies

Research is ongoing to improve treatment outcomes for LR-cHL. Some promising areas include:

  • Targeted Therapies: Agents such as brentuximab vedotin, an antibody-drug conjugate, have shown efficacy in relapsed or refractory cases of Hodgkin lymphoma. This therapy targets CD30, a protein expressed on the surface of Hodgkin lymphoma cells[6].

  • Immunotherapy: Checkpoint inhibitors like pembrolizumab and nivolumab are being investigated for their potential in treating Hodgkin lymphoma, including LR-cHL, especially in cases that do not respond to standard therapies[7].

Conclusion

The treatment of lymphocyte-rich Hodgkin lymphoma (ICD-10 code C81.4) typically involves a combination of chemotherapy and radiation therapy, tailored to the individual patient's disease stage and overall health. While standard regimens like ABVD and IFRT are effective, ongoing research into targeted therapies and immunotherapy holds promise for improving outcomes in this patient population. 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 best course of action.


References

  1. Classic Hodgkin lymphoma, lymphocyte-rich (LR-cHL) [1].
  2. Hodgkin lymphoma burden in Central and South America [9].
  3. Reporting Hodgkin's Lymphoma Using ICD-10 Codes [12].
  4. Article - Billing and Coding: Radiation Therapies [5].
  5. ICD-10-CM Code for Lymphocyte-rich Hodgkin lymphoma C81.4 [10].
  6. ICD 10 CM code c81.4 and healthcare outcomes [11].
  7. 2025 ICD-10-CM Codes C81*: Hodgkin lymphoma [13].

Related Information

Description

  • Characterized by high ratio of lymphocytes
  • Predominance of lymphocytes in tumor microenvironment
  • Fewer Reed-Sternberg cells compared to other subtypes
  • Typically associated with better prognosis
  • Lymphocyte Predominance: hallmark feature
  • Reed-Sternberg Cells: less prevalent in this subtype
  • Fibrosis: varying degrees present in tumor microenvironment

Clinical Information

  • Painless swelling of lymph nodes
  • Lymphadenopathy particularly cervical axillary mediastinal areas
  • Low-grade fever often intermittent
  • Profuse night sweats
  • Unintentional weight loss exceeding 10%
  • General sense of tiredness or lack of energy
  • Pruritus some patients may experience itching
  • Youthful age predominantly diagnosed in 20s and 30s
  • Male predominance similar to other Hodgkin lymphomas
  • Geographic variability with higher rates reported
  • Predominance of lymphocytes few Reed-Sternberg cells
  • CT scans or PET scans used for imaging studies

Approximate Synonyms

  • Lymphocyte-rich Classical Hodgkin Lymphoma
  • Lymphocyte-predominant Hodgkin Lymphoma
  • Classic Hodgkin Lymphoma, Lymphocyte-rich
  • Hodgkin Lymphoma
  • Classical Hodgkin Lymphoma (cHL)

Diagnostic Criteria

  • Painless lymphadenopathy present
  • Fever as a common symptom
  • Night sweats may occur
  • Weight loss possible
  • Fatigue is a common complaint
  • Biopsy required for diagnosis
  • High cellularity with few Reed-Sternberg cells
  • Immunophenotyping confirms diagnosis
  • CD15 and CD30 typically positive
  • Lymphocyte markers CD20 and CD45 positive

Treatment Guidelines

  • Chemotherapy is cornerstone of treatment
  • ABVD regimen used for early-stage disease
  • Escalated BEACOPP for advanced or high-risk disease
  • Radiation therapy used in conjunction with chemotherapy
  • Involved Field Radiation Therapy (IFRT) used to minimize exposure
  • Total Body Irradiation (TBI) used in specific cases
  • Targeted therapies like brentuximab vedotin effective in relapsed cases
  • Immunotherapy with checkpoint inhibitors being investigated

Coding Guidelines

Excludes 1

  • nodular lymphocyte predominant Hodgkin lymphoma (C81.0-)

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