Disease Description
Non-Hodgkin lymphoma (NHL) is a group of malignant neoplasms originating in lymph nodes and other lymphoid tissues, and is a major type of lymphoma (other than Hodgkin lymphoma) that ranges from the most inert to the most aggressive human malignancies. the new WH0 classification identifies each lymphoma type as a separate disease, with different types of lymphoma combining tumor The different types of lymphoma are considered as separate diseases from each other and different therapeutic strategies are recommended, combining tumor involvement at the primary site, specific etiologic features, morphology, immunohistochemical phenotype, cytogenetic abnormalities, and specific clinical features. The etiological, cytogenetic and molecular genetic basis of certain types is well established. The results of multicenter international clinical randomized trials are a good guide for clinical selection of appropriate regimens. Cen Xinan, Department of Hematology, Peking University First Hospital
Disease classification
The classification of lymphatic malignancies has been steadily developed in the twentieth century. In the early twentieth century non-Hodgkin’s lymphoma (NHL) was separated from Hodgkin’s lymphoma (HL) because of the recognition of R-S cells. In the 1950s Gall and Mallory proposed the first systematic classification of NHL into giant follicular lymphoma, lymphosarcoma, and reticulocytic sarcoma, a rather simple classification that proved to be inaccurate and only cursorily applicable in clinical practice. Subsequently, Henry Rappaport et al. recognized the importance of growth type in the classification of NHL, and the use of cell size and morphology as the basis for classification proved to be more clinically useful. In the 1970s, the recognition that NHL was a malignancy of lymphocytes and derived from T and B cells led to the recognition that NHL was a malignancy of lymphocytes. This led to immunologically based classifications of lymphomas such as the Lukes-Collins classification in the United States and the Kiel classification proposed by Lennert et al. in Europe. In order to standardize terminology and improve effective communication between pathologists and clinicians, the Working Formulation classification was proposed in 1982. The Kiel classification dominated European clinical practice for the next two decades, while the Working Formulation was the dominant classification system in North America.
Over the past two decades, further studies of the immune system and genetic abnormalities in NHL have identified many previously unrecognized types of lymphoma. In recognition of these new clinically relevant lymphomas, in 1994, the International Lymphoma Study Group proposed the “European and American Modified Lymphoma Classification”, referred to as the REAL classification, which is based on morphologic, immunophenotypic, genetic features and clinical characteristics, and has gradually demonstrated its scientific accuracy in clinical practice. In 2001, based on the REAL classification, WHO published the classification of “lymphoid tissues tumors”, which is referred to as WHO classification. The new WHO classification of lymphocytic malignancies takes into account morphologic, clinical, immunologic, and genetic abnormalities, and attempts to include clinical and pathologic components in the classification of NHL and other lymphoid malignancies to provide clinical and therapeutic reference guidance. Clinical studies have shown that this new classification has good clinical relevance and higher diagnostic accuracy than the previously applied classification. The classification identifies each lymphoma type as an independent disease, and the different types of lymphoma are considered to be independent of each other by combining the primary site of tumor involvement, specific etiologic features, morphology, immunohistochemical phenotype, cytogenetic abnormalities, and specific clinical features.
The WHO lymphoma classification was widely used in the world in 2001 and was revised for the 4th time in 2008. The main principle of the new WHO classification is that morphologic, immunohistochemical phenotypes, genetic, molecular features and clinical characteristics are combined to form a solid basis for disease classification, and new knowledge and ideas are incorporated into it. The following is a list of the 2008 WHO lymphoma classification.
Progenitor lymphocytic neoplasms
B lymphoblastic leukemia/lymphoma
T-lymphoblast leukemia/lymphoma
Mature B-cell, T-cell, NK-cell lymphoma
Mature B-cell lymphoma
Chronic lymphocytic leukemia/small lymphocytic lymphoma
Pro-B lymphocytic leukemia
Splenic marginal zone lymphoma
Hairy cell leukemia
Splenic lymphoma/leukemia, not classifiable*
Diffuse erythrocytic small B-cell lymphoma of the spleen*
Hairy cell lymphoma Variant*
Lymphoplasmacytic lymphoma
Walden’s macroglobulinemia
Heavy chain disease
alpha heavy chain disease
gamma heavy chain disease
μ heavy chain disease
Plasma cell myeloma
Isolated plasmacytoma of bone
Extramedullary plasmacytoma
Extraconjunctival mucosa-associated lymphoid tissue marginal zone lymphoma (MALT lymphoma)
Intranodal marginal zone lymphoma
Lymphoma of the marginal zone of the lymph nodes in children*
Follicular lymphoma
Follicular lymphoma* in children
Primary cutaneous follicular center lymphoma
Stromal cell lymphoma
Diffuse large B-cell lymphoma, non-specific (DLBCL, NOS)
T-cell/histiocyte-rich large B-cell lymphoma
Primary DLBCL of the central nervous system
Primary cutaneous DLBCL, leg type
Aged EBV-positive DLBCL*
Chronic inflammation-associated DLBCL
Lymphomatoid granuloma
Primary mediastinal (thymic) large B-cell lymphoma
Intravascular large B-cell lymphoma
ALK-positive large B-cell lymphoma
Plasmacytoid lymphoma
Large B-cell lymphoma arising from HHV8-positive multicentric Castleman disease
Primary exudative lymphoma
Burkitt’s lymphoma
Undifferentiated large B-cell lymphoma between diffuse large B-cell lymphoma and Burkitt’s lymphoma
B-cell lymphoma
Unclassifiable B-cell lymphoma between diffuse large B-cell lymphoma and classical Hodgkin’s lymphoma
Unclassifiable B-cell lymphoma between diffuse large B-cell lymphoma and classical Hodgkin lymphoma
Mature T/NK cell lymphoma
Pro-T lymphocytic leukemia
T large granular lymphocytic leukemia
Chronic NK-cell lymphoproliferative disorders*
Aggressive NK-cell leukemia
Childhood systemic EBV-positive T-cell lymphoproliferative disorders
Seedpox blister-like lymphoma
Adult T-cell leukemia/lymphoma
Extranodal NK/T-cell lymphoma, nasal type
Enteropathy-associated T-cell lymphoma
Hepatosplenic T-cell lymphoma
Subcutaneous lipofuscinosis-like T-cell lymphoma
Myxoid granuloma
Sezary syndrome
Primary cutaneous CD30-positive T-cell lymphoproliferative disorder
Lymphomatoid papulosis
Primary cutaneous mesenchymal large cell lymphoma
Primary cutaneous γδ T-cell lymphoma
Primary cutaneous aggressive pro-epidermal CD8-positive cytotoxic T-cell lymphoma*
Primary cutaneous small/medium CD4-positive T-cell lymphoma*
Peripheral T-cell lymphoma, non-specific
Angioimmunoblast T-cell lymphoma
Mesenchymal large cell lymphoma, ALK-positive
Mesenchymal large cell lymphoma, ALK-negative*
Hodgkin’s lymphoma
Nodular lymphocyte-dominant Hodgkin’s lymphoma
Classical Hodgkin’s lymphoma
Nodular sclerosing classical Hodgkin’s lymphoma
Lymphocyte-rich classical Hodgkin’s lymphoma
Mixed cell classical Hodgkin’s lymphoma
Lymphocyte-ablative classical Hodgkin’s lymphoma
Post-transplant lymphoproliferative disease (PTLD)
Early stage lesions
Plasma cell hyperplasia
Infectious mononucleosis-like PTLD
Polymorphic PTLD
Monomorphic PTLD (B-cell and T/NK-cell type)#
Classical Hodgkin’s lymphoma-like PTLD
* Italics indicate that the classification is tentative. The WHO Working Group has not yet had sufficient basis to recognize this type as a separate disease.
Epidemiology
The annual incidence of NHL in Western countries is 14/100,000-19/100,000, accounting for 4% of all tumors, and has been increasing at an annual rate of 3%-4% since 1970. The incidence of NHL increases with age, and the ratio of male to female incidence is 3:2.
The incidence and subtypes of NHL vary with geographic distribution, with T-cell lymphoma being more common in Asia than in Western countries, while some subtypes of B-cell lymphoma, such as follicular lymphoma, are more common in Western countries. Another subtype of NHL is associated with infection with human T-lymphotropic virus I (HTLV-1) and is particularly common in southern Japan and the Caribbean.
Causes and pathogenesis
Patients with both primary and secondary immunodeficiencies are susceptible to NHL, including patients with HIV infection; organ transplant patients, congenital immunodeficiencies, dry syndrome, and rheumatoid arthritis patients.
A number of environmental factors have been associated with the development of NHL, including infections, chemicals, etc. A number of studies have demonstrated that exposure to pesticides is associated with an increased incidence of NHL. Whether NHL may occur in treated patients with Hodgkin’s lymphoma (HL) as a consequence of HL or as a consequence of treatment for HL remains unclear. However, infectious factors have been responsible for the rapid spread of NHL epidemics in some regions in recent years. Table 1 shows the relationship between these infectious factors and the development of NHL. HTLV-1 infection of T cells directly leads to the development of adult T-cell lymphoma in a small proportion of infected patients. The cumulative lifetime risk of NHL in patients who have been infected with HTLV-1, which is transmitted by infected lymphocytes through maternal and infant blood-borne transmission, or sexually transmitted, is 2.5%. The median age of adult T-cell lymphoma patients is approximately 56 years, indicating a long latency period.
EBV has been associated with the development of Burkitt’s lymphoma in Central Africa and with the development of aggressive NHL in immunosuppressed patients in Western countries. In Asia and South America EBV infection is strongly associated with the development of extranodal NK/T-cell lymphoma. In contrast, HIV-infected patients are more likely to develop aggressive B-cell NHL, which may be caused by overexpression of IL6 by infected macrophages.
Helicobacter pylori infection in the stomach causes gastric mucosa-associated lymphoid tissue lymphoma (MALT). The bacteria do not directly convert lymphocytes to lymphoma; rather, the intense immune response and chronic antigenic stimulation induced by the bacteria lead to tumors.
In addition to infectious factors, a range of other diseases or exposures can cause lymphoma.
Previous chemotherapy and radiotherapy
Genetics: Lymphocytic malignancies are associated with genetic abnormalities. Genetic abnormalities can be identified at various levels, including chromosomal alterations (e.g., translocations, additions, or deletions), rearrangements of specific genes, and overexpression, underexpression, or mutations of specific oncogenes. Many lymphomas contain chromosomal translocations involving antigen receptor genes. In diffuse large B-cell lymphoma, translocation t(14;18) occurs in 30% of patients and results in overexpression of the bcl-2 gene on chromosome 18. Some other patients without this translocation also overexpress the bcl-2 protein. This protein is involved in the inhibition of apoptosis – the mechanism of cell death most often induced by cytotoxic chemotherapeutic agents. Higher recurrence rates are seen in patients with tumors that overexpress the bcl-2 protein. In some types, such as t(14; 18) seen in follicular lymphoma; t(8; 14) seen in Burkitt lymphoma; and t(11; 14) seen in condyloma; most patients diagnosed with these types have these abnormalities. These defects may have prognostic significance.
Clinical presentation
Non-Hodgkin’s lymphoma can occur at any age.
The most common clinical presentation is painless, progressive superficial lymph node enlargement, most commonly in the neck, followed by the axillae, groin, and supratentorial.
Some patients present with deep lymph node enlargement, with mediastinal lymph node invasion being the most common, and most of them have no obvious symptoms at the beginning, but only show up on imaging. The symptoms are aggravated when lying down or bending over. The lesion may also invade retroperitoneal or mesenteric lymph nodes, causing abdominal pain, abdominal masses, intestinal obstruction, abdominal organ compression, or prolonged unexplained fever.
The lesions also often start outside the nodes and can invade almost any organ or tissue.
Primary involvement of the gastrointestinal tract is most common, with the stomach being the most common site, and may manifest as epigastric pain, abdominal masses, loss of appetite, and gastrointestinal bleeding.
The nasopharynx is easily involved, with symptoms such as sore throat and nasal congestion, and the local tonsils may be enlarged on one side.
Lymphoma also often invades the liver and spleen, manifesting as hepatosplenomegaly and jaundice.
Skin manifestations of lymphoma are more common and may include skin lumps, nodules, infiltrative plaques, ulcers, papules, etc.
The skeleton, central nervous system, thyroid, lung, breast, and kidney can be involved, resulting in symptoms.
Patients often have systemic symptoms such as fever, night sweats and emaciation.
Diagnosis and differentiation
(A) Diagnostic basis
1.Symptoms and signs
(1) Non-Hodgkin’s lymphoma (NHL) mostly has painless lymph node enlargement.
(2) The lesion often starts outside the node, and can invade almost any organ and tissue, including digestive tract, skin, Wechsler’s ring, thyroid, bone, bone marrow and nervous system. The corresponding symptoms such as mass, pressure, infiltration or bleeding are manifested respectively.
(3) Systemic symptoms:fever, night sweats, weight loss.
2.Pathological examination
The diagnosis of NHL must rely on pathological diagnosis, and lymph node biopsy is the most commonly used means. In case of painless lymph node enlargement, lymph node biopsy should be performed as soon as possible. When NHL is highly suspected clinically, multiple biopsies from different sites can be performed if necessary if the pathological diagnosis is reactive hyperplasia. Surgical excision of lesions involved outside the nodes of NHL is also commonly used.
A complete lymph node biopsy is the basis for a correct pathologic diagnosis, and surgical excision rather than fine needle aspiration is favored for the initial biopsy. In terms of site selection, tough lymph nodes are considered first, and sites that are less affected by inflammatory factors, such as supraclavicular, supraclavicular, cervical, and axillary, while submandibular and inguinal sites are susceptible to local inflammation. For deep lymph nodes, pulmonary nodes or retroperitoneal masses mediastinoscopy, thoracoscopy, CT or ultrasound-guided puncture (these relatively less invasive means) can be used to obtain pathology as early as possible; for extra-nodal special sites such as the stomach, intestine, and center, endoscopic biopsy and open lesion biopsy are required to obtain pathology.
To grasp the new WHO classification of lymphoma in 2008, as each subclass is an independent disease with its own unique pathogenesis, immunophenotype, cytogenetic features and clinical characteristics, clinicians need to fully cooperate with experienced pathologists to obtain an accurate diagnosis from a comprehensive analysis of clinical manifestations, cell morphology and immunophenotype.
3.Blood biochemistry
In addition to assessing the function of the patient’s vital organs, the level of serum lactate dehydrogenase is related to the prognosis of the primary disease, and the elevated level of uric acid should alert the risk of renal damage in tumor lysis syndrome after chemotherapy.
4.Bone marrow aspiration and biopsy
to determine whether there is bone marrow involvement of lymphoma.
5. CT or PET-CT examination
PET-CT examination can not only determine the lesion site but also distinguish the metabolic activity of the lesion site, which is especially helpful for the analysis of the efficacy.
6.Other
Gastroscopy, various colonoscopies, and gastrointestinal imaging can help detect lymphoma GI tract involvement. Cranial magnetic resonance examination and lumbar puncture cerebrospinal fluid examination can help to detect neurological involvement.
(ii) Diagnosis
Painless progressive lymph node enlargement and/or extra-nodal organ tissue involvement, with or without fever, night sweats and wasting. The diagnosis can be confirmed based on the biopsy of lymph nodes or involved organ tissues.
The pathological features of NHL are: destruction of normal structures of lymph nodes or involved tissues by tumor cells; heterogeneous morphology of malignantly proliferating lymphocytes; and invasion of the lymph node envelope.
(C) Staging
The common clinical staging is still Ann Arbor staging, but the correlation between this staging and clinical prognosis is not as good as that of Hodgkin’s lymphoma, and since NHL is a systemic disease with a “jumpy” pathogenesis, the International Prognostic Index (IPI) is now preferred to determine the extent of the disease. The advantage of the IPI is that it integrates the patient’s overall condition and has a stronger correlation with clinical prognosis.
(iv) Differential diagnosis
The differential diagnosis of NHL is mainly distinguished from reactive hyperplasia, tuberculosis, chronic lymphadenitis, viral infection, Hodgkin’s lymphoma, nodal disease, metastatic cancer, etc. All of the above diseases have different corresponding clinical manifestations, and the typical ones are easy to distinguish.
Treatment of non-Hodgkin’s lymphoma
(A) Treatment principles
Because NHL is a systemic disease, most patients should be treated mainly with combination chemotherapy.
The intensity of combination chemotherapy should be decided after integrating the patient’s condition, pathological characteristics, disease stage, and International Prognostic Index (IPI). The ability of patients to receive treatment generally depends on age, general condition, and comorbidities. the new WH0 classification identifies each lymphoma type as a separate disease, and different types of lymphomas combine tumor involvement at the primary site, specific etiologic features, morphology, immunohistochemical phenotype, cytogenetic abnormalities, and specific clinical features. different types of lymphomas are viewed as independent of each other and different treatment strategies. The results of multicenter international clinical randomized trials are a good guide for clinical selection of appropriate regimens.
(II) Selection of chemotherapy regimens for different types of NHL
The treatment of NHL differs in the regimens and regimens used in various different types of NHL. For example, the first-line treatment regimen for diffuse large B-cell lymphoma is CHOP + rituximab or EPOCH + rituximab; the first-line treatment regimen for set cell lymphoma is HyperCVAD/MTX-AraC + rituximab or EPOCH + rituximab or cladribine + rituximab, etc.; the first-line treatment regimen for peripheral T-cell lymphoma is preferred to clinical trials or CHOP or HyperCVAD alternating with high-dose methotrexate and cytarabine; first-line treatment options for follicular lymphoma can be fludarabine + rituximab or CHOP + rituximab or bendamustine + rituximab or CVP + rituximab or FND + rituximab or rituximab or radioimmunotherapy, etc.
(iii) Immunotherapy
Immunotherapy is a rapidly developing treatment in recent years: it includes interferon, various cytokines, and various monoclonal antibodies. Among them, anti-CD20 monoclonal antibodies (rituximab) have achieved significant clinical efficacy. Although anti-CD20 monoclonal antibodies also have certain efficacy when applied alone, the improvement of efficacy is more significant when combined with chemotherapy. Therefore, at present, in clinical application, except for patients with poor general condition, unable to tolerate chemotherapy or rituximab maintenance therapy after disease remission, combination application with chemotherapy is generally advocated.
(iv) Hematopoietic stem cell transplantation
In patients who fail conventional treatment or relapse after remission, autologous hematopoietic stem cell transplantation should be considered. At present, it is advocated that in some aggressive NHL with high IPI (≥2 points), autologous HSCT can be consolidated in the first line in order to obtain better disease-free survival and overall survival time. In a small number of patients, allogeneic HSCT may even be considered.
(v) Radiotherapy
In general, radiotherapy can be used as a supplement to chemotherapy, mostly for the adjuvant treatment of large tumor sites after chemotherapy, as well as for some residual lesions. However, for limited extranodal NK/T-cell lymphoma of the upper aerodigestive tract, early radiotherapy is of great value to improve overall survival and disease-free survival, with a recommended dose of ≥50 Gy.
(vi) Surgery
The status of surgical treatment is more helpful in removing the lesion for definitive diagnosis and is rarely used in the treatment of NHL.
Disease prognosis
Non-Hodgkin’s lymphoma ranges from the most inert to the most aggressive human malignancies. The biological behavior of NHL varies by pathologic type, and the clinical prognosis is inconsistent.
Many clinical and molecular biological features of pretreatment NHL are closely related to patient survival, such as age at diagnosis, systemic (B) symptoms, general physical status, serum LDH, serum β2 microglobulin, number of extra-nodal involvement sites, disease stage, giant lesions, immunohistochemical Ki67, bcl-2 protein expression, P53 mutations, etc.
In 1993, Shipp et al. proposed the International Prognostic Index (IPI) for NHL, which classified the prognosis into four categories: low-risk, low-medium-risk, high-medium-risk, and high-risk, with expected 5-year overall survival rates of 73%, 51%, 43%, and 26%, respectively. Age older than 60 years, stage III or IV, one or more extra-nodal lesions, bed-ridden or requiring care by others, and elevated serum LDH are the five IPIs with poor prognosis, and the prognosis of NHL can be judged based on the number of IPIs the patient has.
Disease prevention
Since the cause of lymphoma patients is not very clear, prevention methods include (1) minimizing infections, avoiding exposure to radiation and other toxic substances, especially drugs that have a suppressive effect on immune function; (2) exercising properly, strengthening physical fitness, and improving one’s resistance to disease.
Clinical features, treatment and prognosis of several common types of non-Hodgkin’s lymphoma
Diffuse large B-cell lymphoma: Diffuse large B-cell lymphoma is the most common type of NHL, accounting for almost one-third of all cases. this type of lymphoma accounts for the majority of cases of previously clinically “aggressive” or “intermediate to highly malignant” lymphoma.
The correct diagnosis of diffuse large B-cell lymphoma requires a hematopathologist based on appropriate biopsy and evidence of B-cell immunophenotyping. Patients with prominent mediastinal invasion are sometimes diagnosed with a separate subtype called primary mediastinal diffuse large B-cell lymphoma, which arises in the anterior mediastinum and may originate in the thymus, usually with distinct mesenchymal fibrous septa, at a younger median age (37 years) and more frequently in women (66%).
Diffuse large B-cell lymphoma may have a primary lymph node or a primary extra-nodal lesion. Over 50% of patients have extra-nodal invasion at the time of diagnosis. The most common extra-nodal lesions are the gastrointestinal tract and bone marrow, each accounting for 15-20% of patients. Any organ can be involved and a diagnostic biopsy is necessary. For example, diffuse large B-cell lymphoma of the pancreas has a much better prognosis than pancreatic cancer, but will be a missed opportunity if a biopsy is not performed. The incidence of primary diffuse large B-cell lymphoma of the brain has increased in recent years.
Gene expression profiling results: Gene expression profiling of over 1000 genes by gene microarray technology at Stanford University in collaboration with the American Institute for Cancer Research showed that DLBCL contains three different molecular subtypes: germinal center B-cell-like (GCB-like) type, which expresses genes characteristic of normal germinal center B cells and has a better prognosis; activated B-cell-like (ABC -like) type, expressing genes characteristic of activated peripheral blood B cells and plasma cells, with a poorer prognosis; and type III expression profile, other heterozygous types without clear features, but with the same prognosis as ABC. pathologists, after further determination of the clinicopathological type, studied the classification of DLBCL by immunohistochemical methods, using a few markers representative of the differentiation spectrum, into GCB-like and NON-GCB types, the latter including ABC and type III classified by gene expression profiles, and these markers include: CD10 and bcl-6 as GCB markers and MUM1 as a NON-GCB marker. rosenwald et al. reported 5-year overall survival rates of GCB type 76% and NON-GCB type 5-year overall survival rate of 34%.
The standard first-line treatment regimen should be rituximab (R) + CHOP regimen, and better efficacy can be achieved by increasing the dose density of the regimen and shortening the time between courses, such as the R-CHOP14 regimen. R-EPOCH can also be used as the first-line treatment regimen. Second-line treatment options include DHAP, ESHAP, GDP, ICE, miniBEAM, and MINE. For primary patients with significant adverse prognostic factors (high-risk high-risk group in the International Prognostic Index IPI), induction chemotherapy to CR followed by high-dose chemoradiotherapy combined with autologous peripheral blood stem cell transplantation can significantly improve their long-term disease-free survival and overall survival. In patients with relapsed disease, transplantation rescue will achieve better outcomes than conventional chemotherapy rescue therapy.
MALT lymphoma: MALT lymphoma accounts for approximately 8% of NHL, and this small cell lymphoma presents in extra-nodal sites. Recognizing that the gastric manifestations of this type of lymphoma are associated with H. pylori infection is an important step in recognizing it as a stand-alone disease.
To correctly diagnose MALT lymphoma, hematopathologists base the diagnosis on the characteristic small lymphocyte infiltrate, which is monoclonal to B cells and CD5 negative. In some cases, this can transform into a diffuse large B-cell lymphoma, and both diagnoses require a biopsy. The differential diagnosis includes benign extra-nodal organ lymphocytic infiltrates and other small cell B-cell lymphomas.
MALT lymphoma can occur in the stomach, orbit, intestines, lungs, thyroid, parotid, skin, soft tissue, bladder, kidney and central nervous system. It sometimes presents as a neoplastic mass that is detected on routine imaging, or as some local symptoms such as epigastric discomfort in gastric lymphoma. About 40% of these lymphomas are confined to the affected organ, while 30% of patients have invasion of the organ and adjacent regional lymph nodes. However, distant metastases can also occur, especially after transformation into diffuse large B-cell lymphoma. Many patients who develop this lymphoma have an autoimmune or inflammatory process such as dry syndrome (parotid MALT lymphoma), Hashimoto’s thyroiditis (thyroid MALT lymphoma), or H. pylori gastritis (gastric MALT lymphoma).
Patients with gastric lymphoma need to be screened for the presence of H. pylori infection. Endoscopy and ultrasonography can help determine the extent of gastric invasion. most patients with MALT lymphoma have a good prognosis, with a 5-year survival rate of 75%. Patients with low IPI scores have a 5-year survival rate of 90%, while those with high IPI scores fall to 40%.
Treatment: The disease can be cured when limited. Local treatment such as radiation therapy or surgery can be effective in eradicating the disease. Most patients with H. pylori-infected gastric MALT lymphoma can achieve long-term remission after eradication of the infection, so patients should be treated first with anti-H. pylori infection, which must be followed by strict endoscopy for a relatively long observation period, and local radiotherapy can be given to patients with poor sustained response. In order to preserve gastric function and ensure the quality of life of patients, surgical treatment is basically not considered at present. For advanced patients with extensive lesions, combined chemotherapy is often used as the main treatment, combined with local radiotherapy.
Sleeve cell lymphoma: Sleeve cell lymphoma accounts for 6% of all NHL. These lymphomas were previously classified among other subtypes, and only in the last decade or so have they been recognized as a separate group of diseases. The recognition that these lymphomas have characteristic chromosomal translocations t(11;14), i.e., translocations between the immunoglobulin heavy chain gene on chromosome 14 and the Bcl-1 gene on chromosome 11, with regular overexpression of Bcl-1 protein and, most characteristically, Cyclin D1, affirmed the existence of this class of lymphomas.
The hematopathologist made the correct diagnosis of set cell lymphoma based on the morphologic findings and the fact that the tumor was a B-cell lymphoma. As with other subtypes of lymphoma, an appropriate biopsy is important. The differential diagnosis of set cell lymphoma includes other small cell B-cell lymphomas. In particular, both set cell lymphoma and small lymphocytic lymphoma have characteristic CD5 expression. The nuclei are often slightly jagged.
The most common manifestation of lymphoma is enlarged lymph nodes, often accompanied by systemic symptoms. Almost 70% of patients have stage III or IV disease at the time of diagnosis, often with bone marrow and peripheral blood infiltration. Extra-nodal organs may be invaded, and gastrointestinal invasion is particularly important in the recognition of the disease. Patients with lymphomatous polyp lesions in the large intestine frequently have condylomatous lymphoma. Patients with gastrointestinal invasion often have pharyngeal lymphatic ring invasion, etc. The 5-year survival rate for lytic lymphoma is approximately 25%, with only a minority of patients with high IPI scores surviving 5 years, and up to 50% for patients with low IPI scores.
Treatment: The efficacy of CHOP regimens for nodular lymphoma is unsatisfactory, with only a minority of patients achieving complete remission. Enhanced combination chemotherapy regimens (such as HyperCVAD/MTX-AraC and EPOCH) accompanied by autologous or allogeneic bone marrow transplantation are often indicated for young patients. Several international clinical trials have shown better clinical outcomes with the combination of rituximab(R) and chemotherapy, so R-HyperCVAD/MTX-AraC, R-EPOCH, R-CHOP and rituximab in combination with cladribine are currently recommended as first-line treatment options. Hematopoietic stem cell transplantation is considered as first-line consolidation therapy for young patients. Rituximab in combination with thalidomide has shown better clinical results and can be used as a second-line treatment option. The proteasome inhibitor bortezomib has shown initial efficacy in refractory relapsed set of lymphomas and may also be used as a second-line treatment option.
Follicular lymphoma: Follicular lymphoma accounts for 22% of NHL worldwide and 30% of NHL in the United States. This type of lymphoma can be correctly diagnosed based on morphologic findings alone.
An evaluation by a hematopathologist of an appropriate biopsy is sufficient to make the diagnosis of follicular lymphoma. The tumor consists of small lytic cells and large cells in varying proportions that constitute follicular growth. Confirmation of the B-cell immunophenotype, the presence of t(14;18) and abnormal expression of BCL