Screening and diagnosis

Classification and risk stratification

Perioperative care

Quality improvement

Patient education

Nonpharmacologic interventions

Therapeutic procedures

Respiratory support

Follow-up and surveillance

Specific circumstances

Preventative measures

Medical management

Surgical interventions

Inpatient care

Diagnostic procedures

Diagnostic investigations

Insufficient evidence to make a recommendation.

Strongly favor this test or intervention.

The Modified Ann Arbor classification for non-Hodgkin's lymphoma is a tool to determine the stage of lymphoma by analyzing three critical aspects of the patient's condition:

- Lymph Node Involvement
- Extralymphatic Organ/Site Involvement
- Spleen Involvement

Each aspect offers a range of conditions:

1. Lymph Node Involvement can range from no involvement to distant (nonregional) nodes.
2. Extralymphatic Organ/Site Involvement can vary from none to multifocal involvement of one or more organs/sites.
3. Spleen Involvement is identified simply as either present (Yes) or absent (No).

Based on these inputs, the classification process identifies the stage of the lymphoma through specific combinations:

- Stage I is assigned when a single lymph node region is involved, without any extralymphatic organ involvement or spleen involvement.
- Stage IE occurs with no lymph node involvement but a single organ/site is affected, and the spleen is not involved.
- Stage II indicates involvement of multiple lymph node regions on the same side of the diaphragm, without extralymphatic organ or spleen involvement.
- Stage IIE results from regional lymph nodes of a single extralymphatic organ or site (potentially combined with other lymph nodes on the same diaphragm side), provided the spleen is not involved.
- Stage III classification is for lymph node regions on both sides of the diaphragm with neither extralymphatic nor spleen involvement.
- Stage IIIE is determined when both sides of the diaphragm have lymph node involvement along with a single organ/site, but without spleen involvement.
- Stage IIIS emerges when both diaphragm sides have lymph node involvement with spleen involvement, but no extralymphatic organ involvement.
- Stage IIIE+S results from the same conditions as IIIS, but with the addition of a single organ/site being involved.
- Stage IV can be determined when there is any lymph node involvement (even single or multiple on the same diaphragm side) alongside multifocal organ involvement, regardless of spleen status.
- Stage IVE occurs when distant (nonregional) lymph nodes are involved along with a single organ, and spleen involvement is inconsequential to this determination.

This meticulous classification framework ensures precise categorization of lymphomas based on the combination and extent of lymph node and organ involvement, supplemented by spleen status, enabling tailored treatment and management approaches.

The Modified Ann Arbor Classification is a staging system specifically used for non-Hodgkin's lymphoma (NHL). This classification system is used to determine the extent of the disease and to guide treatment decisions. It is based on the number of lymph node regions involved, the presence of extranodal disease, and whether the disease is localized or disseminated. 

The patient population for this calculator includes individuals diagnosed with non-Hodgkin's lymphoma. The clinical utility of the Modified Ann Arbor Classification is to provide a standardized method for staging NHL, which is crucial for prognosis and treatment planning. 

There are no specific exclusion criteria for its use, but it is less applicable to patients with certain types of lymphoma that do not typically present with nodal disease, such as primary cutaneous lymphomas. It is also less useful in the pediatric population, where lymphomas often have different characteristics and behavior compared to adults.

Modified Ann Arbor classification for non-Hodgkin's lymphoma

The Central Nervous System International Prognostic Index (CNS-IPI) is a tool designed to estimate the risk of central nervous system (CNS) relapse in individuals based on six specific clinical factors. Each of these factors is evaluated, potentially contributing to a cumulative score ranging from 0 to 6. The higher the score, the greater the risk of CNS relapse.

Here's how the scoring is determined for each factor:

- Age: 
  - If the individual is older than 60 years, they receive 1 point.
  - Individuals 60 years or younger receive 0 points.

- Ann Arbor stages III or IV:
  - Presence of these stages results in 1 point.
  - Absence scores 0 points.

- Extranodal involvement of more than 1 site:
  - If more than one extranodal site is involved, 1 point is added.
  - If not, 0 points are added.

- Kidney and/or adrenal gland involvement:
  - Presence of involvement adds 1 point.
  - Absence results in 0 points.

- Lactate dehydrogenase (LDH) elevation:
  - Elevated levels contribute 1 point.
  - Normal levels contribute 0 points.

- Eastern Cooperative Oncology Group (ECOG) Performance Status greater than 1:
  - A performance status > 1 adds 1 point.
  - A status of 1 or less adds 0 points.

The total score is the sum of all points based on the individual’s responses. Depending on this total, the risk of CNS relapse is classified as follows:

- Scores of 0 or 1 indicate a low risk of CNS relapse, with an estimated 2-year risk of approximately 0.6%.
  
- Scores of 2 or 3 suggest an intermediate risk, with a 2-year CNS relapse risk of around 3.4%.
  
- Scores of 4 or higher signify a high risk, correlating to a 2-year CNS relapse risk of about 10.2%.

This assessment helps tailor risk evaluation for CNS relapse, offering a nuanced understanding based on these clinical factors.

The Central Nervous System International Prognostic Index (CNS-IPI) is a clinical calculator used in the field of oncology, specifically for patients with diffuse large B-cell lymphoma. This scoring system is used to predict the risk of central nervous system relapse in patients with DLBCL. The CNS-IPI takes into account several factors including age, performance status, lactate dehydrogenase  levels, stage of disease, extranodal sites, and the involvement of specific organs such as the kidney or adrenal gland. 

The clinical utility of the CNS-IPI is to aid clinicians in identifying high-risk patients who may benefit from prophylactic CNS-directed therapy. It can also be used to stratify patients in clinical trials. 

The CNS-IPI is not applicable to patients with other types of lymphoma or to patients with DLBCL who have already had a CNS relapse. It should also be used with caution in patients with significant comorbidities or those who are not candidates for intensive chemotherapy.

Central Nervous System International Prognostic Index (CNS-IPI)

The Eastern Cooperative Oncology Group (ECOG) performance status calculator is designed to evaluate a patient's functional ability and categorize it into a specific score from 0 to 5. This quantitative measure helps clinicians make informed decisions about treatment based on the patient's capacity for daily activities and self-care. Here's how it operates:

The user provides input by selecting the description that best matches the patient's current condition from a list of predefined options. These options encapsulate various levels of activity and ability:

- A patient who is "Fully active, able to carry on all pre-disease performance without restriction" is assigned a score of ECOG PS 0. This indicates that the patient is not limited in any capacity by their disease.
- If a patient is "Restricted in physically strenuous activity but ambulatory and able to carry out work of a light or sedentary nature, such as light housework, office work," they receive an ECOG PS 1. This means the patient can perform some self-care and work activities but faces restrictions with strenuous tasks.
- "Ambulatory and capable of all self-care but unable to carry out any work activities; up and about more than 50% of waking hours" corresponds to an ECOG PS 2. This reflects moderate impairment, where the patient is active for the majority of the day but not capable of work activities.
- A patient who is "Capable of only limited self-care, confined to bed or chair more than 50% of waking hours" gets an ECOG PS 3. This score denotes significant limitation, as the patient is mobile for less than 50% of the time and requires assistance for most activities.
- With "Completely disabled, cannot carry on any self-care, totally confined to bed or chair," the patient is categorized as ECOG PS 4, indicating severe impairment and complete dependency.
- Finally, if the patient is "Dead," they are assigned an ECOG PS 5.

These score assignments help standardize assessments, providing an objective measure of a patient's status which can be pivotal in guiding medical decisions and evaluating treatment options.

The Eastern Cooperative Oncology Group (ECOG) performance status, also known as the ECOG score, is a widely used tool in oncology to assess a patient's level of functioning in terms of their ability to care for themselves, daily activity, and physical ability (walking, working, etc.). It is applicable to patients with cancer of any type and at any stage.

The ECOG score ranges from 0 to 5, with 0 indicating fully active and able to carry on all pre-disease activities without restriction, and 5 indicating death. The score is used to determine a patient's prognosis, assess how a disease is progressing, and to determine appropriate treatment strategies. It is also often used in clinical trials to assess eligibility and to monitor patient progress.

There are no specific exclusion criteria for the use of the ECOG score, but it is important to note that it is a subjective measure and may not fully capture a patient's functional status. It is also less useful in patients with fluctuating or rapidly changing conditions.

Eastern Cooperative Oncology Group performance status (ECOG score)

The Karnofsky Performance Scale (KPS) provides a practical method to evaluate a patient's functional ability and the extent of care required based on their health status. The scale ranges from 0% to 100%, where higher percentages signify better functional capacity and health.

This tool involves selecting a descriptive statement that best reflects a patient's current condition from a predefined list. Each statement is connected to a specific KPS percentage that indicates the patient’s ability to perform daily activities and their dependency levels. Here’s how the assessment works:

- When a patient is described as "Normal, no complaints, no evidence of disease," this is assessed as KPS 100%. At this level, the individual can engage in all regular activities, including work, without needing special care.

- If a patient can "carry on normal activity" but exhibits "minor signs or symptoms of disease," they are scored at KPS 90%, indicating the capability to maintain routine activities without requiring special care.

- A description of "Normal activity with effort, some signs or symptoms of disease" aligns with KPS 80%. This suggests the patient can still carry out normal activities but might require some extra effort.

- When the condition reflects "Cares for self, unable to carry on normal activity or to do active work," it corresponds to KPS 70%. The patient can handle most personal needs independently but may need occasional assistance.

- "Requires occasional assistance but is able to care for most personal needs" is rated as KPS 60%, implying increased dependency on assistance though the patient remains mostly self-sufficient.

- At KPS 50%, for conditions described as "Requires considerable assistance and frequent medical care," the patient can live at home but needs considerable help with personal care.

- As the need for care intensifies, with descriptions like "Disabled, requires special care and assistance," the KPS drops to 40%. This marks a significant deterioration in self-care ability.

- "Severely disabled, hospital admission is indicated, death not imminent" corresponds to KPS 30%, highlighting a need for hospitalization without immediate life-threatening conditions.

- For those "Very sick," necessitating hospital admission and "active supportive treatment," the KPS is 20%, indicating critical care requirements.

- At KPS 10%, "Moribund" denotes an inability to care for oneself, with urgent need for hospital-level care and a poor prognosis.

- Lastly, "Dead" is considered as KPS 0%.

This scale is instrumental in providing a quick overview of a patient's ability to function independently and their need for medical support, thereby facilitating better care planning and resource allocation.

The Karnofsky Performance Status Scale (KPSS) is a clinical calculator used primarily in patients with cancer. It is a simple, numerical scale that measures a patient's functional status, ability to perform daily activities, and overall quality of life. The KPSS ranges from 0 (indicating death) to 100 (indicating perfect health). 

The clinical utility of the KPSS is to assess a patient's prognosis, determine appropriate treatment strategies, and monitor changes in a patient's condition over time. It is particularly useful in oncology for determining eligibility for certain treatments, such as chemotherapy or surgery, and for predicting survival outcomes. 

There are no specific exclusion criteria for the use of the KPSS. However, it is less useful in patients with non-cancer illnesses or in those with cognitive impairment who may not be able to accurately self-report their functional status.

Karnofsky Performance Scale

The Lugano classification system is a structured method for staging lymphoma based on key clinical features. The system evaluates three major components: nodal involvement, spleen involvement, and extranodal involvement. By interpreting these factors, clinicians can determine the lymphoma stage, which informs treatment decisions and prognosis.

1. Nodal involvement:
   - Single lymph node region
   - Two or more regions on the same side of the diaphragm
   - Two or more regions on both sides of the diaphragm

2. Spleen involvement:
   - Yes
   - No

3. Extranodal involvement:
   - Single lesion
   - Noncontiguous (disseminated) involvement of one or more sites

The combination of these inputs determines the staging:

- Stage I is identified by the involvement of a single lymph node region. 
- Stage IE occurs if, on top of Stage I criteria, there is a single extranodal lesion.
- Stage II corresponds to multiple regions affected on the same side of the diaphragm.
- Stage IIE is like Stage II but with an additional single extranodal lesion.
- Stage III arises when multiple regions are affected on both sides of the diaphragm. 
- Stage IIIE happens if Stage III conditions include a single extranodal lesion.
- Stage IIIS indicates multiple regions on both sides of the diaphragm with spleen involvement.
- Stage IIIE+S reflects the combination of multiple regions on both sides, spleen involvement, and a single extranodal lesion.
- Stage IV represents noncontiguous extranodal involvement of one or more sites, regardless of other regions or spleen involvement.

This classification helps illuminate the extent of lymphoma, guiding clinical approaches and facilitating communication among healthcare providers.

The Lugano Classification is a clinical calculator used for staging lymphoma, specifically Hodgkin and non-Hodgkin lymphomas. This classification system is based on the Ann Arbor staging system with Cotswolds modifications, but includes additional imaging modalities like PET/CT scans. The Lugano Classification provides a standardized method to assess the extent of lymphoma, which is crucial for determining the appropriate treatment strategy and predicting patient prognosis. It takes into account the number of lymph nodes involved, the presence of extranodal disease, and the involvement of organs such as the spleen or liver. The classification does not apply to patients with lymphomas that have a unique staging system, such as primary cutaneous lymphomas, primary CNS lymphomas, and chronic lymphocytic leukemia/small lymphocytic lymphoma.

Lugano classification for staging lymphoma

The Modified Glasgow Prognostic Score (mGPS) is a key tool in predicting cancer outcomes, relying on the assessment of two biochemical markers: C-reactive protein (CRP) and albumin. This score helps to categorize the prognosis into three levels: good, intermediate, and poor.

To compute the mGPS:

- C-reactive protein is evaluated first. Two options are considered:  
  - If the CRP is less than or equal to 10 mg/L, it indicates lower systemic inflammation.
  - If the CRP is greater than 10 mg/L, it signifies higher inflammation levels.

- Albumin is the second marker, with two possibilities:  
  - An albumin level of less than 3.5 g/dL [35 g/L] suggests malnutrition or potential inflammatory conditions.
  - An albumin level equal to or greater than 3.5 g/dL [35 g/L] signifies a normal nutritional status and protein reserves.

The mGPS score is derived based on the following systematic interpretation:

- If CRP is <= 10 mg/L, regardless of the albumin level, the score is mGPS 0, corresponding to a good prognosis. This combination indicates a low level of inflammation, which is a favorable signal in cancer outcomes.
 
- If CRP is > 10 mg/L and albumin is >= 3.5 g/dL [35 g/L], the score is mGPS 1, associated with an intermediate prognosis. This combination suggests elevated inflammation yet with sufficient nutritional status, resulting in a moderate outlook.

- If CRP is > 10 mg/L and albumin is < 3.5 g/dL [35 g/L], the result is mGPS 2, indicating a poor prognosis. This reflects high inflammation levels coupled with low albumin, suggesting a more severe condition.

Here's a concise summary table for reference:

| C-reactive Protein | Albumin               | mGPS Score | Interpretation          |
|---------------------|-----------------------|-------------|-----------------------|
| <= 10 mg/L          | < 3.5 or >= 3.5 g/dL  | 0           | Good prognosis         |
| > 10 mg/L           | >= 3.5 g/dL           | 1           | Intermediate prognosis |
| > 10 mg/L           | < 3.5 g/dL            | 2           | Poor prognosis         |

This systematic approach enables healthcare providers to improve risk stratification in cancer patients, allowing for more tailored treatment planning.

The Modified Glasgow Prognostic Score (mGPS) is a clinical calculator used in the field of oncology to predict the prognosis of patients with cancer. It is applicable to a wide range of cancer types and stages, making it a versatile tool for clinicians. The mGPS is based on two simple, routinely measured inflammatory markers: C-reactive protein (CRP) and albumin levels. 

The clinical utility of the mGPS lies in its ability to stratify patients into different risk groups based on their inflammatory response to cancer. This can guide clinicians in making decisions about treatment strategies and follow-up plans. It can also provide valuable prognostic information to patients and their families.

There are no specific exclusion criteria for the use of the mGPS. However, it should be noted that the score may not be as accurate in patients with concurrent inflammatory or infectious conditions, as these can also affect CRP and albumin levels. Additionally, the mGPS may not be applicable to patients with rare types of cancer or those with very early or very advanced disease, as it has not been extensively validated in these groups.

Modified Glasgow Prognostic Score for cancer outcomes (mGPS)

Diffuse large B-cell lymphoma

Induction and consolidation therapy

Induction and consolidation therapy, young patients, advanced/high-risk disease

Prognostic scores

Staging

Patients with primary mediastinal diffuse large B-cell lymphoma

Patients with primary mediastinal DLBCL (bone marrow biopsy)

General principles

Patients with primary mediastinal DLBCL (baseline imaging)

Patients with intravascular large B-cell lymphoma

Central nervous system prophylaxis

CNS prophylaxis

Ancillary testing

Patients with gastric diffuse large B-cell lymphoma

Patients with gastric DLBCL

Biopsy and histopathology

General counseling

Repeat biopsy

Patients with primary mediastinal DLBCL (mediastinal biopsy)

Patients with central nervous system diffuse large B-cell lymphoma

Patients with CNS DLBCL (cognitive assessment)

Infection prophylaxis

Patients with primary mediastinal DLBCL, salvage therapy

History and physical examination

Patients with CNS DLBCL, diagnosis

Induction and consolidation therapy, elderly/frail patients

Patients with hepatitis B virus infection

Patients with HBV infection

Clinical follow-up

Febrile neutropenia prophylaxis

Lumbar puncture

Patients with plasmablastic lymphoma

Imaging for staging

Management of non-donor-specific anti-HLA antibodies

Patients with CNS DLBCL (management of intraocular lymphoma)

Laboratory evaluation

Patients with CNS DLBCL, salvage therapy

Management of donor-specific anti-HLA antibodies

Management of donor-specific anti-HLA antibodies (desensitization therapy)

Induction and consolidation therapy, young patients, early/low-risk disease

Patients with primary mediastinal DLBCL (assessment of treatment response)

Patients with primary mediastinal DLBCL (consolidation therapy)

Patients with testicular diffuse large B-cell lymphoma

Patients with testicular DLBCL

Patients with human immunodeficiency virus-associated diffuse large B-cell lymphoma

Patients with HIV-associated DLBCL

Patients with CNS DLBCL, induction chemotherapy

Patients with CNS DLBCL (consolidation therapy)

Patients with breast diffuse large B-cell lymphoma

Patients with breast DLBCL

Management of donor-specific anti-HLA antibodies (follow-up)

Bone marrow biopsy

Patients with primary mediastinal DLBCL (fertility counseling)

Patients with bone diffuse large B-cell lymphoma

Patients with bone DLBCL

Patients with primary extranodal diffuse large B-cell lymphoma

Patients with primary extranodal DLBCL

Imaging follow-up

Cardiac evaluation

Patients with CNS DLBCL (assessment of treatment response)

Laboratory follow-up

Patients with CNS DLBCL (baseline imaging)

Patients with primary mediastinal DLBCL, induction therapy

Patients with CNS DLBCL (ophthalmological assessment)

Patients with primary mediastinal DLBCL (pregnant patients)

Management of donor-specific anti-HLA antibodies (prevention)

Patients with cutaneous diffuse large B-cell lymphoma

Patients with cutaneous DLBCL

Management of osteoporosis

Management of donor-specific anti-HLA antibodies (testing)

Updated 2024 BSH guidelines for the diagnosis and management of diffuse large B-cell lymphoma


Diffuse large B-cell lymphoma

Background

Overview

Guidelines

Key sources