ICD-10: C81.33
Lymphocyte depleted Hodgkin lymphoma, intra-abdominal lymph nodes
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 specific subtype is associated with a more aggressive clinical course compared to other forms of Hodgkin lymphoma. The ICD-10 code C81.33 specifically refers to cases of lymphocyte depleted Hodgkin lymphoma that involve intra-abdominal lymph nodes.
Clinical Description of Lymphocyte Depleted Hodgkin Lymphoma
Pathophysiology
Lymphocyte depleted Hodgkin lymphoma is marked by the presence of large Reed-Sternberg cells, which are derived from B lymphocytes. In this subtype, there is a notable reduction in the number of normal lymphocytes, which contributes to the unique clinical and histological features of the disease. The exact cause of LDHL remains unclear, but it is often associated with immunocompromised states, such as HIV infection or other conditions that affect the immune system.
Symptoms
Patients with lymphocyte depleted Hodgkin lymphoma may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the abdomen, which is a hallmark of this condition.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many types of lymphoma.
- Abdominal Pain or Discomfort: Due to the involvement of intra-abdominal lymph nodes, patients may experience pain or a feeling of fullness in the abdomen.
- Fatigue: Generalized fatigue is often reported due to the systemic effects of the disease.
Diagnosis
Diagnosis of LDHL typically involves:
- Histopathological Examination: A biopsy of affected lymph nodes is essential for confirming the diagnosis. The presence of Reed-Sternberg cells amidst a background of few lymphocytes is indicative of this subtype.
- Imaging Studies: CT scans or PET scans are often utilized to assess the extent of disease involvement, particularly in the abdomen and other lymphatic regions.
- Laboratory Tests: Blood tests may reveal anemia or other abnormalities consistent with malignancy.
ICD-10 Code C81.33: Specifics
Code Definition
The ICD-10 code C81.33 specifically denotes "Lymphocyte depleted Hodgkin lymphoma, intra-abdominal lymph nodes." This classification is crucial for accurate billing and coding in medical records, ensuring that healthcare providers can track and manage cases of this specific lymphoma subtype effectively.
Clinical Implications
The designation of C81.33 has significant implications for treatment and management:
- Treatment Options: Patients with LDHL may require aggressive treatment regimens, including chemotherapy and possibly radiation therapy, depending on the stage of the disease and overall health of the patient.
- Prognosis: The prognosis for lymphocyte depleted Hodgkin lymphoma is generally poorer than for other subtypes, necessitating close monitoring and potentially more intensive therapeutic approaches.
Conclusion
Lymphocyte depleted Hodgkin lymphoma, particularly when involving intra-abdominal lymph nodes, presents unique challenges in diagnosis and treatment. The ICD-10 code C81.33 is essential for healthcare providers to ensure accurate documentation and appropriate management of this aggressive lymphoma subtype. Understanding the clinical features, diagnostic criteria, and treatment options is vital for improving patient outcomes in those affected by this condition.
Clinical Information
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. The ICD-10 code C81.33 specifically refers to cases where the lymphoma is primarily located in the intra-abdominal lymph nodes. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for accurate diagnosis and management.
Clinical Presentation
Signs and Symptoms
Patients with lymphocyte depleted Hodgkin lymphoma may present with a variety of symptoms, which can be broadly categorized into local and systemic manifestations:
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Local Symptoms:
- Abdominal Pain: Patients may experience discomfort or pain in the abdominal region due to the enlargement of intra-abdominal lymph nodes.
- Abdominal Mass: A palpable mass may be detected during physical examination, often associated with lymphadenopathy. -
Systemic Symptoms:
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in many types of lymphoma, including Hodgkin lymphoma.
- Fatigue: Patients often report significant fatigue, which can be debilitating.
- Anemia: Due to bone marrow involvement or chronic disease, patients may present with anemia, leading to pallor and weakness. -
Other Symptoms:
- Pruritus: Some patients may experience itching, which is not uncommon in lymphomas.
- Splenomegaly: Enlargement of the spleen may occur, contributing to abdominal discomfort.
Patient Characteristics
Lymphocyte depleted Hodgkin lymphoma typically presents in specific demographic groups:
- Age: This subtype is more commonly diagnosed in older adults, particularly those over the age of 50. However, it can occur in younger individuals as well.
- Gender: There is a slight male predominance in the incidence of Hodgkin lymphoma, including the lymphocyte depleted subtype.
- Comorbidities: Patients may have a history of immunosuppression or other underlying health conditions, which can influence the presentation and progression of the disease.
Diagnosis and Evaluation
Diagnosis of lymphocyte depleted Hodgkin lymphoma involves a combination of clinical evaluation, imaging studies, and histopathological examination:
- Imaging: CT scans of the abdomen and pelvis are often utilized to assess the extent of lymphadenopathy and to identify any masses.
- Biopsy: A definitive diagnosis is made through a biopsy of the affected lymph nodes, where histological examination reveals the characteristic Reed-Sternberg cells amidst a background of sparse lymphocytes.
Conclusion
Lymphocyte depleted Hodgkin lymphoma, particularly when involving intra-abdominal lymph nodes, presents with a unique set of clinical features. Recognizing the signs and symptoms, along with understanding patient demographics, is essential for timely diagnosis and effective management. Given its rarity and the potential for aggressive behavior, early intervention and a multidisciplinary approach to treatment are critical for improving patient outcomes.
Approximate Synonyms
ICD-10 code C81.33 refers specifically to "Lymphocyte depleted Hodgkin lymphoma, intra-abdominal lymph nodes." This classification is part of the broader category of Hodgkin lymphoma, which is a type of cancer that originates in the lymphatic system. Below are alternative names and related terms associated with this specific diagnosis.
Alternative Names
- Lymphocyte Depleted Hodgkin's Lymphoma: This is the full name of the condition, emphasizing the specific subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes.
- Depleted Lymphocyte Hodgkin Lymphoma: A variation in phrasing that conveys the same meaning.
- Hodgkin Lymphoma, Lymphocyte Depletion Type: This term highlights the specific histological characteristics of the lymphoma.
Related Terms
- Hodgkin Lymphoma: The broader category under which C81.33 falls, encompassing various subtypes, including lymphocyte depleted, mixed cellularity, and nodular sclerosis.
- Non-Hodgkin Lymphoma: While distinct from Hodgkin lymphoma, this term is often used in contrast to Hodgkin lymphoma and may be relevant in discussions about lymphatic cancers.
- Reed-Sternberg Cells: These are the abnormal cells found in Hodgkin lymphoma, including the lymphocyte depleted subtype, and are a key diagnostic feature.
- Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in Hodgkin lymphoma.
- Intra-abdominal Lymph Nodes: This specifies the location of the affected lymph nodes in the context of C81.33, indicating that the lymphoma is present in the abdominal region.
Clinical Context
Lymphocyte depleted Hodgkin lymphoma is a rare and aggressive form of Hodgkin lymphoma, often associated with a poorer prognosis compared to other subtypes. It typically presents with advanced disease and may require aggressive treatment strategies, including chemotherapy and possibly radiation therapy.
Understanding these alternative names and related terms can aid healthcare professionals in accurately diagnosing and coding for this specific type of lymphoma, ensuring appropriate treatment and management strategies are employed.
Diagnostic Criteria
Lymphocyte depleted Hodgkin lymphoma (LDHL) is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes. The diagnosis of LDHL, particularly when it involves intra-abdominal lymph nodes, follows specific clinical, histological, and imaging criteria. Below is a detailed overview of the criteria used for diagnosis, particularly in relation to the ICD-10 code C81.33.
Clinical Presentation
Symptoms
Patients with lymphocyte depleted Hodgkin lymphoma may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, particularly in the abdomen.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are common in Hodgkin lymphoma.
- Abdominal Symptoms: Due to intra-abdominal lymph node involvement, patients may experience abdominal pain, discomfort, or fullness.
Patient History
A thorough medical history is essential, including:
- Previous history of lymphoma or other malignancies.
- Family history of lymphoproliferative disorders.
- Any prior treatments that may affect lymphatic function.
Diagnostic Imaging
Imaging Studies
Imaging plays a crucial role in the diagnosis and staging of LDHL:
- CT Scans: A computed tomography (CT) scan of the abdomen and pelvis is typically performed to identify enlarged lymph nodes and assess the extent of disease.
- PET Scans: Positron emission tomography (PET) scans may be used to evaluate metabolic activity in lymph nodes and help differentiate between active disease and scar tissue.
Histopathological Examination
Biopsy
A definitive diagnosis of lymphocyte depleted Hodgkin lymphoma requires a biopsy of the affected lymph nodes. The following histological features are critical:
- Reed-Sternberg Cells: The presence of these characteristic cells is essential for the diagnosis of Hodgkin lymphoma.
- Lymphocyte Depletion: A marked reduction in lymphocytes surrounding the Reed-Sternberg cells is a hallmark of this subtype.
- Background Inflammatory Cells: The histological examination may reveal a background of inflammatory cells, but they are typically fewer in number compared to other subtypes of Hodgkin lymphoma.
Immunohistochemistry
Immunohistochemical staining is often performed to confirm the diagnosis:
- CD30 and CD15: Reed-Sternberg cells typically express CD30 and CD15 markers.
- Lack of Lymphocyte Markers: A lack of T-cell and B-cell markers in the background lymphocytes supports the diagnosis of lymphocyte depletion.
Staging
Ann Arbor Staging System
The Ann Arbor staging system is used to classify the extent of Hodgkin lymphoma:
- Stage I: Involvement of a single lymph node region.
- Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm.
- Stage III: Involvement of lymph nodes on both sides of the diaphragm, which may include the spleen.
- Stage IV: Disseminated involvement of one or more extralymphatic organs.
In the case of C81.33, the diagnosis specifically pertains to intra-abdominal lymph nodes, which would typically be classified under Stage II or III, depending on the extent of lymph node involvement.
Conclusion
The diagnosis of lymphocyte depleted Hodgkin lymphoma, particularly involving intra-abdominal lymph nodes (ICD-10 code C81.33), relies on a combination of clinical presentation, imaging studies, histopathological examination, and staging criteria. Accurate diagnosis is crucial for determining the appropriate treatment plan 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 Depleted Hodgkin Lymphoma (LDHL), classified under ICD-10 code C81.33, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes. This specific type often presents with advanced disease and can involve intra-abdominal lymph nodes, necessitating a tailored treatment approach. Below, we explore the standard treatment modalities for this condition.
Overview of Lymphocyte Depleted Hodgkin Lymphoma
LDHL is known for its aggressive nature and is often diagnosed at a later stage compared to other Hodgkin lymphoma subtypes. Patients may present with symptoms such as fever, night sweats, weight loss, and lymphadenopathy, particularly in the abdominal region. The prognosis for LDHL is generally poorer than for other forms of Hodgkin lymphoma, making effective treatment crucial.
Standard Treatment Approaches
1. Chemotherapy
Chemotherapy remains the cornerstone of treatment for LDHL. The most commonly used regimens include:
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ABVD Regimen: This includes Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. While ABVD is standard for many Hodgkin lymphoma patients, its efficacy in LDHL 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 advanced-stage patients or those with poor prognostic factors. BEACOPP has shown improved outcomes in some studies for patients with LDHL, particularly in those with extensive disease.
2. Radiation Therapy
Radiation therapy may be employed in conjunction with chemotherapy, especially for localized disease or residual masses after chemotherapy. In cases where intra-abdominal lymph nodes are involved, targeted radiation can help reduce tumor burden and improve local control.
3. Stem Cell Transplantation
For patients with relapsed or refractory LDHL, high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be considered. This approach is particularly relevant for younger patients or those with good performance status, as it can offer a chance for long-term remission.
4. Targeted Therapy
While traditional chemotherapy remains the primary treatment, targeted therapies are being explored. Agents such as brentuximab vedotin (Adcetris®), an antibody-drug conjugate targeting CD30, have shown promise in treating relapsed Hodgkin lymphoma and may be considered in specific cases of LDHL.
5. Clinical Trials
Given the rarity of LDHL, participation in clinical trials may be an option for patients. These trials often explore novel therapies or combinations that could provide better outcomes than standard treatments.
Conclusion
The treatment of Lymphocyte Depleted Hodgkin Lymphoma, particularly when involving intra-abdominal lymph nodes, requires a comprehensive approach that may include chemotherapy, radiation therapy, stem cell transplantation, and potentially targeted therapies. Due to the aggressive nature of this lymphoma subtype, early diagnosis and a multidisciplinary treatment strategy are essential for improving patient outcomes. As research continues, new therapies and combinations may emerge, offering hope for better management of this challenging condition.
For patients diagnosed with LDHL, it is crucial to discuss all available treatment options with a healthcare provider specializing in hematologic malignancies to tailor the approach to individual needs and circumstances.
Related Information
Description
- Rare subtype of Hodgkin lymphoma
- Predominance of Reed-Sternberg cells
- Significant depletion of lymphocytes
- More aggressive clinical course
- Involves intra-abdominal lymph nodes
- Associated with immunocompromised states
- Typically presents with lymphadenopathy
Clinical Information
- Abdominal pain due to lymph node enlargement
- Palpable abdominal mass associated with lymphadenopathy
- Fever is common in B symptoms
- Night sweats are typical of B symptoms
- Unexplained weight loss is a symptom
- Significant fatigue is reported by patients
- Anemia can lead to pallor and weakness
- Pruritus may occur due to lymphoma
- Splenomegaly contributes to abdominal discomfort
- Typically diagnosed in older adults over 50 years
- Male predominance is observed in incidence
- Immunosuppression or comorbidities can influence disease
Approximate Synonyms
- Lymphocyte Depleted Hodgkin's Lymphoma
- Depleted Lymphocyte Hodgkin Lymphoma
- Hodgkin Lymphoma, Lymphocyte Depletion Type
- Lymphadenopathy Intra-abdominal
- Hodgkin Lymphoma Rare Aggressive Form
- Reed-Sternberg Cells Abnormality Present
Diagnostic Criteria
Treatment Guidelines
- Chemotherapy remains cornerstone of treatment
- ABVD regimen may not be effective enough
- BEACOPP regimen considered for advanced-stage patients
- Radiation therapy used with chemotherapy or alone
- Stem cell transplantation for relapsed/refractory cases
- Brentuximab vedotin targeted therapy explored
- Clinical trials offer novel therapies and combinations
Related Diseases
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