ICD-10: C81.30

Lymphocyte depleted Hodgkin lymphoma, unspecified site

Additional Information

Description

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 condition is classified under the ICD-10-CM code C81.30, which specifically denotes "Lymphocyte depleted Hodgkin lymphoma, unspecified site." Below is a detailed overview of this diagnosis, including its clinical description, characteristics, and relevant coding information.

Clinical Description

Overview of Hodgkin Lymphoma

Hodgkin lymphoma (HL) is a type of lymphatic system cancer that originates from B lymphocytes. It is distinguished from non-Hodgkin lymphoma by the presence of Reed-Sternberg cells, which are large, abnormal lymphocytes. Hodgkin lymphoma is further classified into several subtypes, with lymphocyte depleted Hodgkin lymphoma being one of the less common forms.

Characteristics of Lymphocyte Depleted Hodgkin Lymphoma

  • Cellular Composition: LDHL is characterized by a marked reduction in lymphocytes, which are typically present in other forms of Hodgkin lymphoma. The tumor microenvironment is often dominated by Reed-Sternberg cells and a fibrotic background, leading to a distinct histological appearance.
  • Clinical Presentation: Patients may present with symptoms such as lymphadenopathy (swollen lymph nodes), fever, night sweats, and weight loss. Due to the aggressive nature of this subtype, patients may experience more severe symptoms compared to other types of Hodgkin lymphoma.
  • Demographics: LDHL is more frequently diagnosed in older adults and is associated with a poorer prognosis compared to other subtypes of Hodgkin lymphoma.

Diagnosis

Diagnosis typically involves a combination of clinical evaluation, imaging studies (such as CT scans), and histopathological examination of lymph node biopsies. Immunohistochemistry is often used to confirm the presence of Reed-Sternberg cells and to assess the lymphocyte composition.

Coding Information

ICD-10-CM Code C81.30

  • Code Description: The ICD-10-CM code C81.30 specifically refers to "Lymphocyte depleted Hodgkin lymphoma, unspecified site." This code is used when the lymphoma is diagnosed but the specific site of involvement is not specified.
  • Usage: This code is essential for accurate medical billing and coding, ensuring that healthcare providers can document the diagnosis appropriately for treatment planning and insurance reimbursement.
  • C81.31: Lymphocyte depleted Hodgkin lymphoma, cervical lymph nodes
  • C81.32: Lymphocyte depleted Hodgkin lymphoma, mediastinal lymph nodes
  • C81.33: Lymphocyte depleted Hodgkin lymphoma, axillary lymph nodes
  • C81.34: Lymphocyte depleted Hodgkin lymphoma, inguinal lymph nodes
  • C81.39: Lymphocyte depleted Hodgkin lymphoma, other sites

Conclusion

Lymphocyte Depleted Hodgkin Lymphoma, classified under ICD-10 code C81.30, represents a unique and aggressive form of Hodgkin lymphoma with distinct clinical and pathological features. Understanding this diagnosis is crucial for healthcare providers in terms of treatment planning and coding for insurance purposes. Accurate documentation and coding are essential for effective patient management and resource allocation in healthcare settings.

Clinical Information

Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.30, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for diagnosis and management.

Clinical Presentation

General Overview

Lymphocyte depleted Hodgkin lymphoma typically presents in a more aggressive manner compared to other subtypes of Hodgkin lymphoma. It is often diagnosed at an advanced stage, which can complicate treatment and prognosis. The disease is more prevalent in older adults and is associated with a poorer prognosis than other forms of Hodgkin lymphoma[1][2].

Signs and Symptoms

Patients with LDHL may exhibit a variety of signs and symptoms, which can include:

  • Lymphadenopathy: Swelling of lymph nodes, particularly in the cervical, axillary, or inguinal regions. This is often one of the first noticeable signs[3].
  • B Symptoms: These systemic symptoms include:
  • Fever: Unexplained fevers that may be intermittent.
  • Night Sweats: Profuse sweating during the night, often soaking through clothing.
  • Weight Loss: Unintentional weight loss of more than 10% of body weight over six months[4].
  • Fatigue: Persistent tiredness that does not improve with rest, often reported by patients[5].
  • Pruritus: Itching of the skin, which can be generalized or localized[6].
  • Abdominal Symptoms: In cases where lymph nodes in the abdomen are affected, patients may experience abdominal pain or discomfort, and in some cases, splenomegaly (enlarged spleen) may be noted[7].

Patient Characteristics

Certain demographic and clinical characteristics are associated with lymphocyte depleted Hodgkin lymphoma:

  • Age: LDHL is more commonly diagnosed in older adults, particularly those over the age of 60, although it can occur at any age[8].
  • Gender: There is a slight male predominance in the incidence of Hodgkin lymphoma, including the lymphocyte depleted subtype[9].
  • Associated Conditions: Patients with LDHL may have a history of immunosuppression, such as those with HIV/AIDS or those who have undergone organ transplantation, which can increase the risk of developing this aggressive form of lymphoma[10].
  • Histological Features: The diagnosis is confirmed through histological examination, which reveals a predominance of Reed-Sternberg cells and a marked reduction in lymphocytes, distinguishing it from other subtypes of Hodgkin lymphoma[11].

Conclusion

Lymphocyte depleted Hodgkin lymphoma, coded as C81.30 in the ICD-10 classification, presents with distinct clinical features and symptoms that are critical for diagnosis and treatment planning. Recognizing the signs, such as lymphadenopathy and B symptoms, along with understanding patient demographics, can aid healthcare providers in managing this aggressive lymphoma subtype effectively. Early diagnosis and intervention are essential for improving patient outcomes, particularly given the challenges associated with its advanced presentation.

For further information or specific case management strategies, consulting oncology specialists or referring to updated clinical guidelines is recommended.

Approximate Synonyms

Lymphocyte depleted Hodgkin lymphoma (LDHL) is a subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes. The ICD-10 code C81.30 specifically refers to this condition when the site of the lymphoma is unspecified. 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.

  1. Hodgkin Lymphoma: A broader category that includes all subtypes of Hodgkin lymphoma, including lymphocyte depleted, mixed cellularity, and nodular sclerosis.

  2. Reed-Sternberg Cells: The hallmark cells of Hodgkin lymphoma, which are often present in lymphocyte depleted cases.

  3. Non-Hodgkin Lymphoma: While distinct from Hodgkin lymphoma, this term is often mentioned in discussions about lymphomas in general.

  4. Stage IV Hodgkin Lymphoma: Lymphocyte depleted Hodgkin lymphoma can often present at advanced stages, including stage IV, which may be relevant in clinical discussions.

  5. Hodgkin's Lymphoma, Unspecified Site: This term may be used in clinical settings when the specific site of the lymphoma is not identified.

Clinical Context

Lymphocyte depleted Hodgkin lymphoma is relatively rare compared to other subtypes and is often associated with poorer prognosis. It is essential for healthcare providers to recognize the specific characteristics and terminology associated with this condition for accurate diagnosis and treatment planning.

In summary, understanding the alternative names and related terms for ICD-10 code C81.30 can enhance communication among healthcare professionals and improve patient care by ensuring clarity in diagnosis and treatment discussions.

Diagnostic Criteria

Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.30, 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 involves several criteria, which can be categorized into clinical, histopathological, and imaging assessments.

Clinical Criteria

  1. Symptoms: Patients may present with classic symptoms of lymphoma, including:
    - Painless lymphadenopathy (swollen lymph nodes)
    - Fever
    - Night sweats
    - Weight loss
    - Fatigue

  2. Physical Examination: A thorough physical examination is essential to identify lymphadenopathy and splenomegaly, which are common in Hodgkin lymphoma.

Histopathological Criteria

  1. Biopsy: A definitive diagnosis of LDHL requires a biopsy of the affected lymph node or tissue. The histological examination should reveal:
    - A predominance of Reed-Sternberg cells.
    - A marked depletion of small lymphocytes, which is a distinguishing feature of this subtype.
    - The presence of a fibrotic background and inflammatory cells, which may include eosinophils and plasma cells.

  2. Immunohistochemistry: Immunophenotyping is crucial for confirming the diagnosis. Reed-Sternberg cells typically express:
    - CD30 (a hallmark of Hodgkin lymphoma)
    - CD15
    - PAX5
    - They are usually negative for B-cell markers (e.g., CD20) and T-cell markers.

Imaging Studies

  1. CT or PET Scans: Imaging studies are used to assess the extent of disease and to identify any additional sites of involvement. These scans help in staging the disease, which is critical for treatment planning.

  2. Bone Marrow Biopsy: In some cases, a bone marrow biopsy may be performed to evaluate for bone marrow involvement, which can occur in advanced stages of the disease.

Differential Diagnosis

It is important to differentiate LDHL from other subtypes of Hodgkin lymphoma and non-Hodgkin lymphomas. The presence of significant lymphocyte depletion and the specific histological features are key in distinguishing LDHL from other lymphomas.

Conclusion

The diagnosis of lymphocyte depleted Hodgkin lymphoma (ICD-10 code C81.30) is based on a combination of clinical presentation, histopathological findings, and imaging studies. Accurate diagnosis is essential for appropriate management and treatment planning, as LDHL can have different prognostic implications compared to other subtypes of Hodgkin lymphoma. If you have further questions or need more detailed information, feel free to ask!

Treatment Guidelines

Lymphocyte Depleted Hodgkin Lymphoma (LD-HL), classified under ICD-10 code C81.30, 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 at diagnosis and requires a tailored treatment approach. Below, we explore the standard treatment strategies for LD-HL.

Overview of Lymphocyte Depleted Hodgkin Lymphoma

LD-HL is known for its aggressive nature and is less common than other subtypes of Hodgkin lymphoma. Patients typically present with symptoms such as lymphadenopathy, fever, night sweats, and weight loss. Due to its rarity, treatment protocols may vary, but they generally align with those used for other forms of Hodgkin lymphoma, albeit with some modifications to address the unique challenges posed by this subtype.

Standard Treatment Approaches

1. Chemotherapy

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

  • ABVD Regimen: This includes Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. While ABVD is standard for many Hodgkin lymphoma patients, its efficacy in LD-HL may be limited due to the disease's aggressive nature.

  • BEACOPP Regimen: This regimen (Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, and Prednisone) is often considered for patients with advanced-stage disease or those who do not respond adequately to ABVD. BEACOPP has shown improved outcomes in some studies for patients with LD-HL, particularly in terms of progression-free survival[1].

2. Radiation Therapy

Radiation therapy may be employed as part of the treatment plan, especially in localized disease or as consolidation therapy following chemotherapy. The use of involved-field radiation therapy (IFRT) can help target residual disease and reduce the risk of relapse. However, the decision to use radiation therapy is carefully considered, particularly in younger patients, due to the potential long-term side effects[2].

3. Stem Cell Transplantation

For patients with relapsed or refractory LD-HL, high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be indicated. This approach is typically reserved for those who have not responded to initial treatment or have experienced a relapse after first-line therapy. ASCT can provide a chance for long-term remission in select patients[3].

4. Targeted Therapies and Immunotherapy

Recent advancements in targeted therapies and immunotherapy have opened new avenues for treating Hodgkin lymphoma, including LD-HL. Agents such as:

  • Brentuximab Vedotin (Adcetris): This is an antibody-drug conjugate that targets CD30, a marker present on Reed-Sternberg cells. It is used in cases of relapsed or refractory Hodgkin lymphoma and may be considered in the treatment of LD-HL, particularly in combination with chemotherapy or as a bridge to stem cell transplantation[4].

  • Checkpoint Inhibitors: Drugs like Pembrolizumab and Nivolumab, which target PD-1, have shown promise in treating relapsed Hodgkin lymphoma and may be considered for LD-HL patients who do not respond to conventional therapies[5].

Conclusion

The treatment of Lymphocyte Depleted Hodgkin Lymphoma is complex and 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, in some cases, stem cell transplantation or targeted therapies. Given the aggressive nature of LD-HL, ongoing clinical trials and research are essential to optimize treatment strategies and improve patient outcomes. As always, treatment decisions should be made in consultation with a healthcare team experienced in managing this rare lymphoma subtype.


References

  1. Chemotherapy regimens for Hodgkin lymphoma.
  2. Radiation therapy in Hodgkin lymphoma.
  3. Stem cell transplantation for relapsed Hodgkin lymphoma.
  4. Brentuximab Vedotin in Hodgkin lymphoma.
  5. Immunotherapy for Hodgkin lymphoma.

Related Information

Description

  • Rarest subtype of Hodgkin lymphoma
  • Predominance of Reed-Sternberg cells
  • Significant depletion of lymphocytes
  • Aggressive nature of this subtype
  • Poorer prognosis compared to others
  • Unspecified site involvement

Clinical Information

  • Lymphocyte depleted Hodgkin lymphoma
  • Rare subtype of Hodgkin lymphoma
  • Aggressive manner compared to other subtypes
  • Advanced stage at diagnosis
  • Poorer prognosis than other forms
  • Common in older adults over 60 years old
  • Slight male predominance in incidence
  • Immunosuppression increases risk
  • Lymphadenopathy and B symptoms common signs
  • Fever, night sweats, weight loss typical symptoms
  • Fatigue, pruritus, abdominal symptoms possible

Approximate Synonyms

  • Lymphocyte Depleted Hodgkin's Disease
  • Hodgkin Lymphoma, Lymphocyte Depleted Type
  • Depleted Lymphocyte Hodgkin Lymphoma
  • Hodgkin Lymphoma
  • Reed-Sternberg Cells
  • Non-Hodgkin Lymphoma
  • Stage IV Hodgkin Lymphoma

Diagnostic Criteria

  • Painless lymphadenopathy
  • Fever and night sweats present
  • Weight loss and fatigue occur
  • Thorough physical examination required
  • Biopsy of affected lymph node or tissue
  • Reed-Sternberg cells in histological examination
  • Marked depletion of small lymphocytes
  • Fibrotic background with inflammatory cells
  • CD30, CD15, and PAX5 expression confirmed
  • Negative for B-cell and T-cell markers

Treatment Guidelines

  • Chemotherapy with ABVD regimen
  • BEACOPP regimen for advanced disease
  • Involved-field radiation therapy for localized disease
  • Autologous stem cell transplantation for relapsed/refractory patients
  • Brentuximab Vedotin for CD30+ cells
  • Checkpoint inhibitors for refractory patients

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