The International Prognostic Index (IPI) is a clinical tool developed by oncologists to aid in predicting the prognosis of patients with aggressive non-Hodgkin's lymphoma. Previous to IPI's development, the primary consideration in assessing prognosis was the Ann Arbor stage alone, but this was increasingly found to be an inadequate means of predicting survival outcomes, and so other factors were studied.[ citation needed ]
In 1984, the first prognostic indicator for advanced non-Hodkin's lymphoma was developed. An information theory guided, computer search and evaluation procedure entropy minimax was employed to discover the largest sub-groupings for which survival is as extreme as possible [1] In the clinical trials analyzed retrospectively and containing a large fraction of patients not matching the 'good' - of 'good' (Karnofsky status >80 and 'A" Symptoms and SGOT <36 U/L), 'poor' (Karnofsky status <70 or Night sweats) and 'intermediate' (All Remaining) prognosis patterns identified, a significant difference was found between the survival of patients with and those without a complete response to therapy. The authors concluded that trials using a patient mix weighted toward good prognosis will not find such a difference.
In 1993, a retrospective analysis was performed on 2031 patients with aggressive non-Hodgkin's lymphoma, of all ages, treated with a doxorubicin-based chemotherapy regimen such as CHOP between 1982 and 1987. [2] Several patient characteristics were analyzed to determine whether they were associated with differences in survival, and the factors that emerged as significant were, in addition to the Ann Arbor stage: age, elevated serum lactate dehydrogenase (LDH), performance status, and number of extranodal sites of disease.
One point is assigned for each of the following risk factors:[ citation needed ]
The sum of the points allotted correlates with the following risk groups:
A simplified index can be used when comparing patients within an age group (i.e. 60 or younger, or over 60) and includes only 3 of the above factors:[ citation needed ]
The sum of the points allotted correlates with the following risk groups:
Although the IPI has shown itself to be a useful clinical tool, widely used by oncologists and a mainstay of risk stratification in clinical trials for lymphoma, it should be kept in mind that it was developed prior to the use of rituximab, which is now included with anthracycline-based combination chemotherapy as of the standard of care in B-cell lymphomas (the majority of non-Hodgkin's lymphomas). Rituximab has significantly improved the outcomes of lymphoma patients; and its effect on the prognostic value of the IPI is uncertain. Future development of a more rigorous prognostic index may thus be useful.
Given the success of the IPI for intermediate grade lymphomas, an effort was undertaken to develop a similar prognostic index for the most common low-grade lymphoma, follicular lymphoma. The prognostic factors that emerged from this were: age, stage, number of lymph node areas involved, serum hemoglobin level, and serum LDH. [3]
One point is assigned for each of the following adverse prognostic factors:[ citation needed ]
The sum of the points allotted correlates with the following risk groups:
An effort was more recently undertaken to identify a similar prognostic index predictive of outcome in advanced mantle cell lymphoma. There were four factors found to have independent prognostic relevance: age, performance status, LDH, and white blood cell count (WBC). [4]
The point values are assigned as follows:
The sum of the allotted points correlates with the following risk groups:
Non-Hodgkin lymphoma (NHL), also known as non-Hodgkin's lymphoma, is a group of blood cancers that includes all types of lymphomas except Hodgkin lymphomas. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tiredness. Other symptoms may include bone pain, chest pain, or itchiness. Some forms are slow-growing while others are fast-growing.
Lymphoma is a group of blood and lymph tumors that develop from lymphocytes. The name typically refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlarged lymph nodes, fever, drenching sweats, unintended weight loss, itching, and constantly feeling tired. The enlarged lymph nodes are usually painless. The sweats are most common at night.
Prognosis is a medical term for predicting the likelihood or expected development of a disease, including whether the signs and symptoms will improve or worsen or remain stable over time; expectations of quality of life, such as the ability to carry out daily activities; the potential for complications and associated health issues; and the likelihood of survival. A prognosis is made on the basis of the normal course of the diagnosed disease, the individual's physical and mental condition, the available treatments, and additional factors. A complete prognosis includes the expected duration, function, and description of the course of the disease, such as progressive decline, intermittent crisis, or sudden, unpredictable crisis.
Waldenström macroglobulinemia is a type of cancer affecting two types of B cells: lymphoplasmacytoid cells and plasma cells. Both cell types are white blood cells. It is characterized by having high levels of a circulating antibody, immunoglobulin M (IgM), which is made and secreted by the cells involved in the disease. Waldenström macroglobulinemia is an "indolent lymphoma" and a type of lymphoproliferative disease which shares clinical characteristics with the indolent non-Hodgkin lymphomas. It is commonly classified as a form of plasma cell dyscrasia, similar to other plasma cell dyscrasias that, for example, lead to multiple myeloma. Waldenström macroglobulinemia is commonly preceded by two clinically asymptomatic but progressively more pre-malignant phases, IgM monoclonal gammopathy of undetermined significance and smoldering Waldenström macroglobulinemia. The Waldenström macroglobulinemia spectrum of dysplasias differs from other spectrums of plasma cell dyscrasias in that it involves not only aberrant plasma cells but also aberrant lymphoplasmacytoid cells and that it involves IgM while other plasma dyscrasias involve other antibody isoforms.
CHOP is the acronym for a chemotherapy regimen used in the treatment of non-Hodgkin lymphoma. CHOP consists of:
Follicular lymphoma (FL) is a cancer that involves certain types of white blood cells known as lymphocytes. The cancer originates from the uncontrolled division of specific types of B-cells known as centrocytes and centroblasts. These cells normally occupy the follicles in the germinal centers of lymphoid tissues such as lymph nodes. The cancerous cells in FL typically form follicular or follicle-like structures in the tissues they invade. These structures are usually the dominant histological feature of this cancer.
Lymphoma (lymphosarcoma) in animals is a type of cancer defined by a proliferation of malignant lymphocytes within solid organs such as the lymph nodes, bone marrow, liver and spleen. The disease also may occur in the eye, skin, and gastrointestinal tract.
T-cell lymphoma is a rare form of cancerous lymphoma affecting T-cells. Lymphoma arises mainly from the uncontrolled proliferation of T-cells and can become cancerous.
Primary gastric lymphoma is an uncommon condition, accounting for less than 15% of gastric malignancies and about 2% of all lymphomas. However, the stomach is a very common extranodal site for lymphomas. It is also the most common source of lymphomas in the gastrointestinal tract.
The B-cell lymphomas are types of lymphoma affecting B cells. Lymphomas are "blood cancers" in the lymph nodes. They develop more frequently in older adults and in immunocompromised individuals.
Diffuse large B-cell lymphoma (DLBCL) is a cancer of B cells, a type of lymphocyte that is responsible for producing antibodies. It is the most common form of non-Hodgkin lymphoma among adults, with an annual incidence of 7–8 cases per 100,000 people per year in the US and UK. This cancer occurs primarily in older individuals, with a median age of diagnosis at ~70 years, although it can occur in young adults and, in rare cases, children. DLBCL can arise in virtually any part of the body and, depending on various factors, is often a very aggressive malignancy. The first sign of this illness is typically the observation of a rapidly growing mass or tissue infiltration that is sometimes associated with systemic B symptoms, e.g. fever, weight loss, and night sweats.
Manchester score is an indicator of prognosis in small cell lung cancer. It is calculated from a number of physical and biochemical markers.
The Ranson criteria form a clinical prediction rule for predicting the prognosis and mortality risk of acute pancreatitis. They were introduced in 1974 by the English-American pancreatic expert and surgeon Dr. John Ranson (1938–1995).
Mantle cell lymphoma (MCL) is a type of non-Hodgkin's lymphoma, comprising about 6% of cases. It is named for the mantle zone of the lymph nodes where it develops. The term 'mantle cell lymphoma' was first adopted by Raffeld and Jaffe in 1991.
Aggressive lymphoma, also known as high-grade lymphoma, is a group of fast growing non-Hodgkin lymphoma.
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a slow-growing CD20 positive form of Hodgkin lymphoma, a cancer of the immune system's B cells.
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a rare type of lymphoma that commonly involves midline areas of the nasal cavity, oral cavity, and/or pharynx At these sites, the disease often takes the form of massive, necrotic, and extremely disfiguring lesions. However, ENKTCL-NT can also involve the eye, larynx, lung, gastrointestinal tract, skin, and various other tissues. ENKTCL-NT mainly affects adults; it is relatively common in Asia and to lesser extents Mexico, Central America, and South America but is rare in Europe and North America. In Korea, ENKTCL-NT often involves the skin and is reported to be the most common form of cutaneous lymphoma after mycosis fungoides.
Hodgkin lymphoma (HL) is a type of lymphoma in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells are present in the patient's lymph nodes. The condition was named after the English physician Thomas Hodgkin, who first described it in 1832. Symptoms may include fever, night sweats, and weight loss. Often, nonpainful enlarged lymph nodes occur in the neck, under the arm, or in the groin. Persons affected may feel tired or be itchy.
Primary cutaneous follicle center lymphoma is a type of lymphoma. It was recognized as a distinct disease entity in the 2008 WHO classification. PCFCL had been previously conceived as a variant of follicular lymphoma (FL).
Indolent lymphoma, also known as low-grade lymphoma, is a group of slow-growing non-Hodgkin lymphomas (NHLs). Because they spread slowly, they tend to have fewer signs and symptoms when first diagnosed and may not require immediate treatment. Symptoms can include swollen but painless lymph nodes, unexplained fever, and unintended weight loss.