ICD-10: C83.37

Diffuse large B-cell lymphoma, spleen

Additional Information

Description

Clinical Description of ICD-10 Code C83.37: Diffuse Large B-Cell Lymphoma, Spleen

ICD-10 Code C83.37 specifically refers to Diffuse Large B-Cell Lymphoma (DLBCL) that is primarily located in the spleen. DLBCL is a type of non-Hodgkin lymphoma characterized by the rapid proliferation of large B-lymphoid cells. This aggressive form of lymphoma can arise in various anatomical sites, including lymph nodes, extranodal tissues, and the spleen.

Key Characteristics of Diffuse Large B-Cell Lymphoma

  1. Pathophysiology:
    - DLBCL is a heterogeneous group of lymphomas that can arise from different stages of B-cell development. The malignancy is marked by the presence of large atypical lymphoid cells that infiltrate lymphoid tissues and can spread to other organs.

  2. Clinical Presentation:
    - Patients with DLBCL may present with symptoms such as:

    • Lymphadenopathy: Swollen lymph nodes, which may be painless.
    • Splenomegaly: Enlargement of the spleen, which can lead to abdominal discomfort or fullness.
    • B-symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement.
    • Fatigue: Generalized weakness and fatigue are common due to the disease's impact on the body.
  3. Diagnosis:
    - Diagnosis typically involves a combination of clinical evaluation, imaging studies (such as CT scans), and histopathological examination of biopsy samples. Immunophenotyping and genetic studies may also be performed to classify the specific subtype of DLBCL and guide treatment.

  4. Staging:
    - The Ann Arbor staging system is commonly used to determine the extent of the disease. Staging is crucial for treatment planning and prognosis. DLBCL can be localized (stage I) or disseminated (stages II-IV), with splenic involvement often indicating a more advanced stage.

  5. Treatment:
    - The standard treatment for DLBCL includes:

    • Chemotherapy: The most common regimen is R-CHOP, which combines rituximab with cyclophosphamide, doxorubicin, vincristine, and prednisone.
    • Radiation Therapy: May be used in localized cases or as a consolidation treatment.
    • Stem Cell Transplantation: Considered for relapsed or refractory cases.
  6. Prognosis:
    - The prognosis for patients with DLBCL varies based on several factors, including the International Prognostic Index (IPI) score, which considers age, stage, performance status, and lactate dehydrogenase (LDH) levels. Early diagnosis and treatment are associated with better outcomes.

Conclusion

ICD-10 code C83.37 captures the clinical essence of Diffuse Large B-Cell Lymphoma affecting the spleen, highlighting its aggressive nature and the importance of timely diagnosis and treatment. Understanding the clinical features, diagnostic criteria, and treatment options is essential for healthcare providers managing patients with this condition. Early intervention can significantly improve patient outcomes, making awareness and education about DLBCL critical in clinical practice.

Clinical Information

Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin lymphoma characterized by the rapid proliferation of large B-cells. When it specifically involves the spleen, it is classified under the ICD-10 code C83.37. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for diagnosis and management.

Clinical Presentation

Signs and Symptoms

  1. Lymphadenopathy: Patients may present with enlarged lymph nodes, which can be localized or generalized. In cases where the spleen is involved, splenomegaly (enlargement of the spleen) is often noted.

  2. Abdominal Symptoms: Due to splenomegaly, patients may experience abdominal discomfort, pain, or a feeling of fullness. This can lead to early satiety, where patients feel full after eating only a small amount of food.

  3. Constitutional Symptoms: Common systemic symptoms include:
    - Fever: Often low-grade but can be persistent.
    - Night Sweats: Profuse sweating during the night.
    - Weight Loss: Unintentional weight loss is frequently reported.
    - Fatigue: A general sense of tiredness or lack of energy is common.

  4. Hematological Symptoms: Patients may exhibit signs of anemia, thrombocytopenia (low platelet count), or leukopenia (low white blood cell count), which can lead to increased susceptibility to infections and bleeding.

  5. B Symptoms: The presence of fever, drenching night sweats, and weight loss exceeding 10% of body weight in six months is referred to as "B symptoms" and is significant in the staging and prognosis of lymphoma.

Patient Characteristics

  1. Age: DLBCL can occur at any age but is more common in older adults, typically affecting individuals over 60 years old. However, it can also present in younger adults and children.

  2. Gender: There is a slight male predominance in the incidence of DLBCL, with men being more frequently diagnosed than women.

  3. Comorbidities: Patients with a history of autoimmune diseases, immunosuppression (such as HIV/AIDS), or prior chemotherapy may have an increased risk of developing DLBCL. Additionally, conditions like diabetes mellitus have been associated with inferior survival outcomes in DLBCL patients[8].

  4. Ethnicity: The incidence of DLBCL can vary by ethnicity, with some studies indicating higher rates in certain populations, although the reasons for these disparities are not fully understood.

  5. Genetic Factors: Certain genetic mutations and chromosomal abnormalities may predispose individuals to DLBCL, influencing both the clinical presentation and response to treatment.

Conclusion

Diffuse large B-cell lymphoma of the spleen (ICD-10 code C83.37) presents with a range of clinical signs and symptoms, including lymphadenopathy, abdominal discomfort, and systemic "B symptoms." Patient characteristics such as age, gender, and comorbidities play a significant role in the disease's manifestation and prognosis. Early recognition and diagnosis are essential for effective management and treatment of this aggressive lymphoma type. Understanding these aspects can aid healthcare professionals in providing timely and appropriate care for affected patients.

Approximate Synonyms

Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin lymphoma characterized by the rapid growth of large B-cells. The specific ICD-10-CM code for DLBCL affecting the spleen is C83.37. Below are alternative names and related terms associated with this condition.

Alternative Names for Diffuse Large B-Cell Lymphoma

  1. Large Cell Lymphoma: This term is often used interchangeably with DLBCL, emphasizing the size of the cancerous cells.
  2. B-cell Lymphoma: A broader category that includes various types of lymphomas originating from B-cells, including DLBCL.
  3. Non-Hodgkin Lymphoma (NHL): DLBCL is a subtype of non-Hodgkin lymphoma, which encompasses a wide range of lymphatic cancers.
  4. Aggressive B-cell Lymphoma: This term highlights the aggressive nature of DLBCL, which typically requires prompt treatment.
  5. Diffuse B-cell Lymphoma: A variation of the name that emphasizes the diffuse nature of the tumor spread.
  1. ICD-10 Code: C83.37 specifically refers to DLBCL of the spleen, while other codes exist for different sites and types of lymphomas.
  2. Histological Subtypes: DLBCL can have various subtypes based on histological features, such as:
    - Germinal Center B-cell-like (GCB) DLBCL
    - Activated B-cell-like (ABC) DLBCL
  3. Lymphoma Staging: Related terms include staging classifications such as Ann Arbor staging, which is used to determine the extent of lymphoma spread.
  4. Chemotherapy Regimens: Common treatment regimens for DLBCL include R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone).
  5. Immunotherapy: Treatments like CAR T-cell therapy and monoclonal antibodies (e.g., Rituximab) are also relevant in the context of DLBCL.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C83.37 is crucial for healthcare professionals involved in the diagnosis and treatment of diffuse large B-cell lymphoma. This knowledge aids in accurate coding, effective communication among medical teams, and appropriate treatment planning. If you need further details on treatment options or specific coding guidelines, feel free to ask!

Diagnostic Criteria

Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin lymphoma characterized by the rapid proliferation of large B-cells. The ICD-10 code C83.37 specifically refers to DLBCL located in the spleen. Diagnosing DLBCL, including its splenic variant, involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below are the key criteria and steps typically used in the diagnosis of DLBCL, particularly for the ICD-10 code C83.37.

Clinical Presentation

Symptoms

Patients with DLBCL may present with a variety of symptoms, including:
- Lymphadenopathy: Swelling of lymph nodes, which may be localized or generalized.
- Splenomegaly: Enlargement of the spleen, which is particularly relevant for C83.37.
- B-symptoms: These include fever, night sweats, and unexplained weight loss, which can indicate more aggressive disease.

Medical History

A thorough medical history is essential, including:
- Previous history of lymphoma or other malignancies.
- Family history of lymphoproliferative disorders.
- Exposure to certain risk factors, such as immunosuppression or infections (e.g., HIV, Epstein-Barr virus).

Diagnostic Imaging

Imaging Studies

Imaging techniques are crucial for assessing the extent of the disease:
- CT Scans: Computed tomography scans of the abdomen and pelvis can reveal splenic involvement and assess lymph node enlargement.
- PET Scans: Positron emission tomography scans may be used to evaluate metabolic activity in suspected lymphoma sites.

Histopathological Examination

Biopsy

A definitive diagnosis of DLBCL requires a tissue biopsy:
- Excisional Biopsy: Removal of an entire lymph node or a portion of the spleen for examination.
- Core Needle Biopsy: A less invasive option that can also provide sufficient tissue for diagnosis.

Pathological Criteria

The histopathological evaluation typically includes:
- Morphological Assessment: Identification of large atypical B-cells in the tissue sample.
- Immunophenotyping: Use of immunohistochemical stains to confirm the B-cell lineage (e.g., CD19, CD20, CD22) and to rule out other types of lymphoma.
- Genetic Studies: Molecular tests, such as fluorescence in situ hybridization (FISH), may be performed to detect specific genetic abnormalities associated with DLBCL, such as the MYC gene rearrangement.

Staging and Classification

Ann Arbor Staging System

Once diagnosed, the disease is staged using the Ann Arbor classification, which considers:
- The number of involved lymph node regions.
- The presence of extranodal disease (e.g., splenic involvement).
- The presence of B-symptoms.

WHO Classification

The World Health Organization (WHO) classification of lymphoid neoplasms provides further categorization, which is essential for treatment planning and prognosis.

Conclusion

The diagnosis of diffuse large B-cell lymphoma, particularly with splenic involvement (ICD-10 code C83.37), is a multifaceted process that combines clinical evaluation, imaging studies, and histopathological analysis. Accurate diagnosis is crucial for determining the appropriate treatment strategy and improving patient outcomes. If you have further questions or need more specific information about treatment options or prognosis, feel free to ask!

Treatment Guidelines

Diffuse large B-cell lymphoma (DLBCL), particularly when it involves the spleen, is a type of non-Hodgkin lymphoma characterized by aggressive behavior and a need for prompt treatment. The standard treatment approaches for DLBCL, including cases coded under ICD-10 code C83.37, typically involve a combination of chemotherapy, immunotherapy, and sometimes radiation therapy. Below is a detailed overview of the standard treatment modalities.

1. Chemotherapy

R-CHOP Regimen

The most common first-line treatment for DLBCL is the R-CHOP regimen, which includes:
- Rituximab: A monoclonal antibody targeting CD20 on B-cells.
- Cyclophosphamide: An alkylating agent that interferes with DNA replication.
- Doxorubicin (Adriamycin): An anthracycline that disrupts DNA and RNA synthesis.
- Vincristine (Oncovin): A vinca alkaloid that inhibits cell division.
- Prednisone: A corticosteroid that helps reduce inflammation and suppress the immune response.

This combination is typically administered in cycles over several months, with the goal of achieving complete remission[1][2].

2. Immunotherapy

Targeted Therapies

In addition to chemotherapy, targeted therapies such as Polatuzumab vedotin (Polivy) may be used, especially in cases where the disease is refractory or relapsed. This drug is an antibody-drug conjugate that delivers a cytotoxic agent directly to CD79b-expressing B-cells, enhancing the effectiveness of treatment[3][4].

CAR-T Cell Therapy

For patients with relapsed or refractory DLBCL, CAR-T cell therapy (Chimeric Antigen Receptor T-cell therapy) has emerged as a promising option. This involves modifying a patient’s T-cells to better recognize and attack lymphoma cells. CAR-T therapy has shown significant efficacy in patients who do not respond to standard treatments[5][6].

3. Radiation Therapy

While chemotherapy and immunotherapy are the mainstays of treatment, radiation therapy may be considered in specific scenarios, such as:
- Localized disease: If the lymphoma is confined to the spleen and surrounding areas, radiation can help eliminate residual disease.
- Palliative care: To relieve symptoms in advanced cases where the disease is causing discomfort or complications[7].

4. Supportive Care

Supportive care is crucial throughout the treatment process. This includes:
- Management of side effects: Addressing nausea, fatigue, and immunosuppression.
- Infection prophylaxis: Given the immunocompromised state of patients undergoing chemotherapy, prophylactic antibiotics and vaccinations may be necessary[8].

Conclusion

The treatment of diffuse large B-cell lymphoma, particularly when it involves the spleen (ICD-10 code C83.37), is multifaceted and tailored to the individual patient’s condition, including factors such as age, overall health, and specific disease characteristics. The R-CHOP regimen remains the cornerstone of initial therapy, with additional options like targeted therapies and CAR-T cell therapy available for more complex cases. Ongoing research continues to refine these approaches, aiming to improve outcomes for patients diagnosed with this aggressive form of lymphoma.

For patients and healthcare providers, staying informed about the latest treatment protocols and clinical trials is essential for optimizing care and improving survival rates.

Related Information

Description

  • Diffuse Large B-Cell Lymphoma
  • Primarily located in the spleen
  • Type of non-Hodgkin lymphoma
  • Rapid proliferation of large B-lymphoid cells
  • Aggressive form of lymphoma
  • Lymphadenopathy and splenomegaly symptoms
  • B-symptoms include fever and fatigue
  • Diagnosis by biopsy and histopathology
  • Staging with Ann Arbor system
  • Treatment includes chemotherapy and radiation

Clinical Information

  • Lymphadenopathy is a common presentation
  • Splenomegaly leads to abdominal discomfort
  • Fever is often low-grade but persistent
  • Night sweats are profuse and frequent
  • Weight loss is unintentional and significant
  • Fatigue is a general sense of tiredness
  • Anemia and thrombocytopenia occur frequently
  • DLBCL affects older adults predominantly
  • Male gender has a slight predominance
  • Comorbidities increase disease risk

Approximate Synonyms

  • Large Cell Lymphoma
  • B-cell Lymphoma
  • Non-Hodgkin Lymphoma (NHL)
  • Aggressive B-cell Lymphoma
  • Diffuse B-cell Lymphoma

Diagnostic Criteria

  • Lymphadenopathy with swelling of lymph nodes
  • Splenomegaly with enlargement of spleen
  • B-symptoms including fever, night sweats, and weight loss
  • Previous history of lymphoma or malignancies
  • Family history of lymphoproliferative disorders
  • Exposure to immunosuppression or infections
  • CT scans for assessing disease extent
  • PET scans for evaluating metabolic activity
  • Excisional biopsy for definitive diagnosis
  • Core needle biopsy as less invasive option
  • Morphological assessment of atypical B-cells
  • Immunophenotyping to confirm B-cell lineage
  • Genetic studies including FISH and MYC gene rearrangement
  • Ann Arbor staging system considering lymph node regions
  • Extranodal disease presence, including splenic involvement
  • WHO classification for treatment planning and prognosis

Treatment Guidelines

  • Chemotherapy R-CHOP regimen standard
  • Rituximab monoclonal antibody therapy
  • Cyclophosphamide alkylating agent treatment
  • Doxorubicin anthracycline disrupts DNA synthesis
  • Vincristine vinca alkaloid inhibits cell division
  • Prednisone corticosteroid reduces inflammation
  • Immunotherapy targeted therapies available
  • Polatuzumab vedotin antibody-drug conjugate
  • CAR-T cell therapy modifies T-cells
  • Radiation therapy localized disease treatment
  • Palliative care symptom relief in advanced cases
  • Supportive care management of side effects

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