Extranodal lymphomas account for a third of all cases of non-Hodgkin lymphoma with the gastrointestinal tract being the most common extranodal site. The most common location is the stomach followed by the small intestine, colon and rectum. Colorectal lymphomas are rare and comprise 10–20% of all gastrointestinal lymphomas and only 1% of all colorectal malignancies. Presenting symptoms include abdominal pain, weight loss, and anorexia. Diagnosis depends on the clinical setting with elective cases being diagnosed with colonoscopy and emergent cases being diagnosed in the operating room. Colonic lymphomas are frequently located proximal to the hepatic flexure. Management depends on the aggressiveness of the lymphoma subtype. Indolent tumors, which are resistant to standard chemotherapeutic regimens, are treated with surgical resection. Aggressive lymphoma subtypes are managed with chemotherapy and surgery with late-stage disease patients being referred to clinical trials.
Lymphoma; non-Hodgkin lymphoma; gastrointestinal; intestinal tumors
Extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT lymphoma) is the third most common non‐Hodgkin lymphoma subtype, accounting for around 6–8% of all non‐Hodgkin lymphomas in the Western hemisphere. Although MALT lymphomas are clinically indolent, the disease is typically chronic, requiring long‐term clinical surveillance and, often, repeated biopsies. Pathologists thus play a central role in the diagnosis and management of these patients. The optimal diagnosis and management of a MALT lymphoma requires careful integration of morphological, immunohistochemical and molecular information, together with close cooperation with the clinician treating the patient. This review discusses recent developments in the molecular pathogenesis of MALT lymphoma and provides strategies for integrating this information into daily pathological practice.
Background: The prognosis for patients with most forms of T-cell lymphoma is poor. Allogeneic hematopoietic stem-cell transplantation (HSCT) may improve the outcome.
Patients and methods: This study examines the outcome of 52 patients who underwent ablative or nonablative allogeneic HSCT for peripheral T-cell lymphoma (PTCL) or advanced mycosis fungoides/Sezary syndrome over a 12-year period at a single institution. We divided the patients into those with predominantly nodal histologies: peripheral T-cell not otherwise specified (PTCL NOS), angioimmunoblastic (AITL), or anaplastic large cell lymphoma, T/null type (systemic) (ALCL), and predominantly extranodal histologies: natural killer (NK)/T cell, enteropathy type, hepatosplenic, subcutaneous panniculitic, mycosis fungoides, or T cell or NK cell other.
Results: Median follow-up of survivors is 49 months. Non-relapse mortality and relapse at 3 years was 27% and 43%, respectively. The incidence of grade II–IV acute graft-versus-host disease (GVHD) was 21%. The incidence of extensive chronic GVHD at 2 years was 27%. The 3-year progression-free survival was 30%: 45% in patients with predominantly nodal histologies (PTCL NOS, AITL, and ALCL) and 6% in patients with predominantly extranodal histologies (P = 0.016). Overall survival at 3 years was 41% for all patients.
Conclusion: Allogeneic HSCT can produce long-term remissions in relapsed/refractory T-cell lymphoma, especially those with nodal histologies.
allogeneic transplant; CTCL; GVHD; mycosis fungoides; NK lymphoma; T-cell lymphoma
Primary cutaneous lymphomas are the second most common site of extranodal non-Hodgkin lymphoma. A specifically type named extranodal marginal zone B-cell lymphomas are indolent low-grade neoplasma.
We report a case of a 42-year-old white man with multiple subcutaneous tumors located on the trunk and neck. The histopathological exam showed a non-epidermotropic, dense lymphocytic infiltrate. Histologic, immunohistochemical and cytologenetic analysis diagnosed primary cutaneous B-cell lymphoma MALT type. Investigation for other extranodal MALT lymphoma gastrointestinal tract, lung, salivary and thyroid glands was negative. The patient refused radiotherapy, but he accepted every 6 months close follow-up. Over a seven years period, we noticed a progressively disappearance of the skin lesions.
The necessity of aggressive treatment of this disease with excellent prognosis is discussed.
The treatment necessity of primary cutaneous B-cell lymphoma MALT type is discussed.
primary cutaneous B-cell lymphoma; MALT type; therapy
Peripheral T-cell lymphomas are a group of rare neoplasms originating from clonal proliferation of mature post-thymic lymphocytes with different entities having specific biological characteristics and clinical features. As natural killer cells are closely related to T-cells, natural killer-cell lymphomas are also part of the group. The current World Health Organization classification recognizes four categories of T/natural killer-cell lymphomas with respect to their presentation: disseminated (leukemic), nodal, extranodal and cutaneous. Geographic variations in the distribution of these diseases are well documented: nodal subtypes are more frequent in Europe and North America, while extranodal forms, including natural killer-cell lymphomas, occur almost exclusively in Asia and South America. On the whole, T-cell lymphomas are more common in Asia than in western countries, usually affect adults, with a higher tendency in men, and, excluding a few subtypes, usually have an aggressive course and poor prognosis. Apart from anaplastic lymphoma kinase-positive anaplastic large cell lymphoma, that have a good outcome, other nodal and extranodal forms have a 5-year overall survival of about 30%. According to the principal prognostic indexes, the majority of patients are allocated to the unfavorable subset. In the past, the rarity of these diseases prevented progress in the understanding of their biology and improvements in the efficaciousness of therapy. Recently, international projects devoted to these diseases created networks promoting investigations on T-cell lymphomas. These projects are the basis of forthcoming cooperative, large scale trials to detail biologic characteristics of each sub-entity and to possibly individuate targets for new therapies.
Lymphoma, T-cell/epidemiology; Killer-cells, natural; Prognosis; Lymphoma, T- cell/ pathology; Lymphoma, T-Cell/classification; Hematologic neoplasms; South America; Europe
Mantle cell lymphoma is a well-recognized distinct clinicopathologic subtype of
B-cell non-Hodgkin lymphoma. The current World Health Organization (WHO)
classification subdivides this entity into aggressive and other variants. The
disease has a predilection for older males, and patients typically present at an
advanced stage with frequent splenomegaly and extranodal involvement including
bone marrow, peripheral blood, gastrointestinal, and occasional central nervous
system involvement. Early studies of therapy outcomes in this disease revealed
that while response rates where high, relapse was expected after a limited
period of time. Prolonged survival was uncommon, with initial median survival
rates typically in the 3–4-year range. Those with a high proliferative
rate, blastoid morphology, and selected clinical features were recognized as
having a worse prognosis. Therapeutic approaches have diverged into aggressive
therapies with high response rates and promising progression free survival
rates, which may be applied to younger healthy patients, and less aggressive
approaches. Aggressive therapies include intensive chemotherapy alone or
chemotherapy followed by autologous stem cell transplant, which has been shown
to be most effective when applied in first remission. Whether these more intense
therapies result in improved survival as compared with less aggressive therapies
is not well established. Allogeneic transplant has also been investigated,
although high treatment-related mortality and the risk of chronic graft
versus host disease and the relatively advanced age of this
patient population have tempered enthusiasm for this approach. A number of less
aggressive therapies have been shown to produce promising results. Consolidation
and maintenance strategies are an active area of investigation. A number of
newer agents have shown promising activity in relapsed disease, and are being
investigated in the front-line setting. Overall survival rates are improving in
this disease, with current studies suggesting a median survival of 5 or more
chemotherapy; mantle cell lymphoma; stem cell transplant
Aim—To report the clinical and histological features and outcome of primary and secondary malignant lymphomas of the urinary bladder.
Methods—Eleven cases of malignant lymphoma of the urinary bladder were obtained from the registry of cases at St Bartholomews and the Royal London Hospitals. The lymphomas were classified on the basis of their morphology and immunophenotype, and the clinical records were reviewed.
Results—There were six primary lymphomas: three extranodal marginal zone lymphomas of mucosa associated lymphoid tissue (MALT) type and three diffuse large B cell lymphomas. Of the five secondary cases, four were diffuse large B cell lymphomas, one secondary to a systemic follicular follicle centre lymphoma, and one nodular sclerosis Hodgkins disease. Four patients with secondary lymphoma for whom follow up was available had died of disease within 13 months of diagnosis. Primary lymphomas followed a more indolent course. In one case, there was evidence of transformation from low grade MALT-type to diffuse large B cell lymphoma. The most common presenting symptom was haematuria. Cystoscopic appearances were of solid, sometimes necrotic tumours resembling transitional cell carcinoma, and in one case the tumours were multiple. These cases represented 0.2% of all bladder neoplasms.
Conclusions—Diffuse large B cell lymphoma and MALT-type lymphoma are the most common primary malignant lymphomas of the bladder. Lymphoepithelial lesions in MALT-type lymphoma involve transitional epithelium, and their presence in high grade lymphoma suggests a primary origin owing to transformation of low grade MALT-type lymphoma. Primary and secondary diffuse large B cell lymphomas of the bladder are histologically similar, but the prognosis of the former is favourable.
Key Words: bladder • lymphoma • mucosa associated lymphoid tissue lymphoma
A patient with primary B cell non‐Hodgkin's lymphoma of the sciatic nerve is described. He presented with neuropathic symptoms in the left leg, initially diagnosed as tarsal tunnel syndrome. Magnetic resonance imaging (MRI) identified the abnormality in the sciatic nerve. A fascicular biopsy of the sciatic nerve showed a diffuse large B cell non‐Hodgkin's lymphoma. The patient was treated with chemotherapy and rituximab (anti‐CD20 monoclonal antibody). Four months later he was in remission, and remains so 48 months from presentation. Primary lymphoma of single peripheral nerves may be a unique subtype of extranodal lymphoma, which usually follows an aggressive course and has a variable response to current therapeutic strategies. MRI is useful, alongside electrophysiological studies, in patients with atypical peripheral nerve symptoms.
We report the case of a 65 year-old man who presented with epigastric pain and guaic-positive stool. Upper and lower endoscopy revealed abnormalities in the gastric antrum and terminal ileum. Biopsy of these sites revealed histologically and immunophenotypically distinct lymphomas: gastric extranodal marginal zone lymphoma in the background of Helicobacter pylori infection and follicular lymphoma of the terminal ileum. After treatment with an H. pylori eradication regimen, repeat endoscopy showed resolution of the gastric extranodal marginal zone lymphoma and persistence of the ileal follicular lymphoma. Interestingly, molecular studies performed on the biopsy specimens revealed a common IgH rearrangement, suggesting a common precursor cell responsible for these two malignant processes. We present this unique case with a review of the literature, highlighting treatment principles for these two subtypes of indolent gastrointestinal non-Hodgkin lymphoma.
follicular lymphoma; extranodal marginal zone lymphoma; MALT; Helicobacter pylori
Primary intestinal non-Hodgkin lymphoma (NHL) is a heterogeneous disease with regard to anatomic and histologic distribution. Thus, analyses focusing on primary intestinal NHL with large number of patients are warranted.
We retrospectively analyzed 581 patients from 16 hospitals in Korea for primary intestinal NHL in this retrospective analysis. We compared clinical features and treatment outcomes according to the anatomic site of involvement and histologic subtypes.
B-cell lymphoma (n = 504, 86.7%) was more frequent than T-cell lymphoma (n = 77, 13.3%). Diffuse large B-cell lymphoma (DLBCL) was the most common subtype (n = 386, 66.4%), and extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) was the second most common subtype (n = 61, 10.5%). B-cell lymphoma mainly presented as localized disease (Lugano stage I/II) while T-cell lymphomas involved multiple intestinal sites. Thus, T-cell lymphoma had more unfavourable characteristics such as advanced stage at diagnosis, and the 5-year overall survival (OS) rate was significantly lower than B-cell lymphoma (28% versus 71%, P < 0.001). B symptoms were relatively uncommon (20.7%), and bone marrow invasion was a rare event (7.4%). The ileocecal region was the most commonly involved site (39.8%), followed by the small (27.9%) and large intestines (21.5%). Patients underwent surgery showed better OS than patients did not (5-year OS rate 77% versus 57%, P < 0.001). However, this beneficial effect of surgery was only statistically significant in patients with B-cell lymphomas (P < 0.001) not in T-cell lymphomas (P = 0.460). The comparison of survival based on the anatomic site of involvement showed that ileocecal regions had a better 5-year overall survival rate (72%) than other sites in consistent with that ileocecal region had higher proportion of patients with DLBCL who underwent surgery. Age > 60 years, performance status ≥ 2, elevated serum lactate dehydrogenase, Lugano stage IV, presence of B symptoms, and T-cell phenotype were independent prognostic factors for survival.
The survival of patients with ileocecal region involvement was better than that of patients with involvement at other sites, which might be related to histologic distribution, the proportion of tumor stage, and need for surgical resection.
intestine; non-Hodgkin lymphoma; prognosis; histopathology
Extranodal marginal zone lymphoma is a low-grade B cell lymphoma that presents with an indolent clinicopathologic nature. Although this tumor can occur in various sites, including the gastrointestinal tract and lungs, it develops and spreads extremely rarely along the trachea and central airway. We report a case of extranodal lymphoma of mucosa-associated lymphoid tissue with tracheobronchial involvement. An 83-year-old woman presented with a cough and dyspnea. Bronchoscopic evaluation confirmed diffuse, multiple nodular lesions in both the trachea and large bronchi, and she was diagnosed with an extranodal marginal zone lymphoma of the tracheobronchial tree. After systemic chemotherapy, she survived for more than 18 months.
Extranodal marginal zone lymphoma; mucosa-associated lymphoid tissue; tracheobronchial involvement
Primary gastrointestinal lymphoma comprises 10-15% of all non-Hodgkin lymphomas and encompasses 30-40% of the total extranodal lymphomas. Approximately 60-75% of cases occur in the stomach, and then the small bowel, ileum, cecum, colon and rectum. Lymphoid neoplasms may consist of mature B, T and less commonly extranodal NK/T cells. Of these, the two most frequently encountered histologic subtypes are extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), where Helicobacter pylori infection is implicated in a number of cases, and diffuse large B cell lymphoma. Several B cell lymphomas are associated with chromosomal aberrations. Enteropathy-associated T cell lymphoma, type I in particular, usually arises in a background of celiac disease. T cell gene rearrangement confirms clonality. NK/T cell neoplasms are invariably associated with Epstein-Barr virus infection and are often aggressive; thus, differentiation from a benign NK-cell enteropathy is paramount. Although incidence of other hematopoietic malignancies in the gastrointestinal tract such as plasma cell myeloma associated with amyloidosis, plasmablastic lymphoma, Hodgkin disease, histiocytic sarcoma and mast cell sarcoma is extremely rare, these entities have been documented, with the latter two demonstrating aggressive clinical behavior. Endoscopic ultrasonography is an important adjunct in disease staging and follow-up. Conservative antibiotic treatment of stage I MALT lymphomas with associated Helicobacter pylori infection achieves good clinical outcome with high remission rate. Chemotherapy, radiation and rarely surgery are reserved for advanced diseases or cases resistant to conservative therapy and those not associated with Helicobacter pylori infection.
Gastrointestinal lymphomas; MALT lymphoma; NK/T-cell enteropathy
Among non-Hodgkin lymphoma subtypes, T-cell phenotype confers a poor clinical prognosis. For more aggressive histologies, patients frequently present with advanced disease that is inherently chemoresistant. For cutaneous histologies, disease progresses less rapidly, but is debilitating and often incurable long term. Here we report the retrospective analysis of data from 27 patients with mature T-cell lymphoma treated with salvage allo-HCT at the City of Hope using a reduced-intensity fludarabine/melphalan conditioning regimen between the years 2001 and 2008. Eleven of the twenty-seven patients had cutaneous T-cell lymphoma (CTCL). The majority of patients had advanced disease at the time of transplant (17/27 or 63%). Median followup was 36 months. We observed 2-year overall survival (OS) of 55%, progression-free survival (PFS) of 47%, and cumulative incidence of relapse/progression and non-relapse mortality (NRM) of 30% and 22%, respectively. For CTCL, patients had a 2-yr PFS of 45% and NRM of 27% compared to patients with other histologies, who had PFS of 62% and NRM of 19%. Overall, our results suggest that meaningful long term survival rates and disease control can be achieved with acceptable non-relapse mortality in patients with mature T-cell lymphomas, including CTCL using reduced intensity conditioning with melphalan and fludarabine.
T-cell lymphoma; reduced intensity conditioning; allogeneic transplant
Although the incidence of T-cell non-Hodgkin's lymphoma (NHL) is higher in Far East Asia than in Western countries, its incidence and clinical course in Korea are not well-defined. Therefore, we assessed the relative frequency and clinical features of T-cell NHL in Korea.
We performed a retrospetcive analysis of 586 patients with NHL.
101 (17.2%) had T-cell NHL. The most frequent subtypes of T-cell NHL were extranodal NK/T-cell lymphoma, nasal type (NASAL), peripheral T-cell lymphoma, unspecified type (PTCL-U), and anaplastic large cell lymphoma, T/null cell, primary systemic type (ALCL). The seven pathological subtypes could be classified into three prognostic subgroups. When patients with the three most frequent subtypes were grouped together, their survival was reflected in the International Prognostic Index (IPI) scores. Univariate analysis of IPI elements and other clinical features showed that clinical stage and extranodal sites were significant predictors of survival. Cox multivariate analysis showed that the number of extranodal sites was the only independent prognostic indicator.
The relative frequency of T-cell NHL seems to be decreasing in Korea, although NASAL remains frequent. Korean patients with ALCL appear to have an unfavorable prognosis. Large-scale studies are warranted for Korean patients with T-cell NHL.
Lymphoma; T-cell; Peripheral
Despite the well-defined role of autologous haematopoietic stem cell transplantation (autoHCT) in the treatment of patients with relapsed or refractory Hodgkin lymphoma (HL), relapse remains the main cause of transplant failure. We retrospectively evaluated long-term outcome and prognostic factors affecting survival of 132 patients with refractory (n = 89) or relapsed HL (n = 43) treated with autoHCT following modified BEAM. With a median follow-up of 68 months, the 10-year overall survival (OS) and progression-free survival (PFS) were 76 and 66 %, respectively. The 10-year cumulative incidence of second malignancies was 7 %. In multivariate analysis, age ≥45 years, more than one salvage regimens and disease status at transplant worse than CR were factors predictive for poor OS. In relapsed HL, age at transplant, response duration (<12 vs. ≥12 months) and the number of salvage regimens were independent predictors for PFS. In the refractory setting, disease status at autoHCT and the number of salvage regimens impacted PFS. The number of risk factors was inversely correlated with PFS in both relapsed and refractory HL (p = 0.003 and <0.001, respectively). The median PFS for patients with >1 risk factor in the relapsed and refractory setting was 5 and 11 months, respectively, in comparison with the median PFS not reached for patients with 0–1 risk factor in both settings. We conclude that high proportion of patients with relapsed/refractory HL can be cured with autoHCT. However, the presence of two or more risk factors helps to identify poor prognosis patients who may benefit from novel treatment strategies.
Hodgkin lymphoma; Autologous haematopoietic stem cell transplantation; BEAM; Prognostic factors; Lymphocyte recovery; Positron emission tomography
The outcome and management of relapsed lymphoma after allogeneic hematopoietic cell transplantation (HCT) is difficult. Therapeutic options may include donor lymphocyte infusion (DLI), reduction of immunosuppression (RIS), chemotherapy, radiation, immunotherapy, second HCT and experimental treatments, but reported data contrasting the response and efficacy of these salvage treatments is limited. We describe the treatments, response, prognosis and long-term survival of 72 patients with relapse of lymphoma after allogeneic HCT.
Between 1991 and 2007, 227 lymphoma patients underwent allogeneic HCT. Of these, 72 (32%) developed relapse/progression after their HCT at a median of 99 days (0–1898 days); 37 had early (<100 days) post-HCT relapse. Forty-four had non-Hodgkin's lymphoma (7 mantle cell, 5 indolent, 15 diffuse large B cell, 4 Burkitt's and 13 T/Natural Killer cell) and 28 patients had Hodgkin's lymphoma. At the time of HCT, 62 patients were in remission (22 in complete [CR] and 40 in partial [PR]), one had stable while 9 had progressive disease. Seventeen cases received myeloablative and 55 received a reduced intensity conditioning regimen. At relapse, most patients had generalized lymphadenopathy, extranodal organ involvement and advanced disease. Five patients received no intervention for the post-HCT relapse. Immunosuppressive treatment was reduced or withdrawn as the first line therapy in 58 patients (80.5%); 47 were treated using combinations of conventional chemotherapy (n=22), rituximab (n=27), interferon (IFN) (n=1), DLI (n=7), second HCT (n=2), local radiation (n=23) and other therapy (n=6). Thirty-eight patients had an objective response (CR in 30, PR in 8) and 2 had stable disease (SD). At the post-HCT relapse, favorable prognostic factors for survival after HCT included good ECOG performance status (0–2), normal lactate dehydrogenase (LDH), early stage disease (stage I–III), isolated extranodal organ involvement and later relapse (>100 days) post-HCT. Three year survival after HCT was significantly better in late than early relapse (53% (95% confidence interval (CI) [34–69%] vs. 36%, [20–52%], p=0.02). Of 72 relapsed patients, 29 (40%) survive at a median of 34 (3–148) months post transplant. The most common cause of death was underlying lymphoma (79%).
The overall prognosis of relapsed/progressive lymphoma after allogeneic HCT is disappointing, yet half of patients respond to withdrawal of immunosuppression and additional therapies. Novel treatments can control lymphoma with acceptable morbidity. Particularly for patients with later relapse, ongoing treatment after relapse can yield meaningful benefit and prolonged survival.
Allogeneic hematopoietic cell transplantation; lymphoma; relapse
Extranodal natural killer (NK)/T-cell lymphoma of nasal type (NKTCL) is a malignant disorder of cytotoxic lymphocytes of NK or more rarely T cells associated with clonal Epstein-Barr virus infection. Extranodal NKTCL is rare in Western countries, but in Asia and Central and South America it can account for up to 10% of non-Hodgkin's lymphomas. It is an aggressive neoplasm with very poor prognosis. Although the pathogenesis of extranodal NKTCL remains poorly understood, some insights have been gained in the recent years, especially from genome-wide studies. Based on our own experience and knowledge of the literature, we here review some of the genomic and functional pathway alterations observed in NKTCL that could provide a rationale for the development of innovative therapeutic strategies.
Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type (extranodal MZL) is a distinct subgroup of non-Hodgkin's lymphoma. Pulmonary extranodal MZL is a rare entity and accounts for less than 0.5% of primary pulmonary malignancies. Only a few cases of simultaneous occurrence of lung cancer and pulmonary extranodal MZL have been reported. A 60-year-old woman was referred to our hospital with a pulmonary nodule. She was diagnosed with lung adenocarcinoma by percutaneous needle biopsy. The protrusions into the left main bronchus were found by accident while performing bronchoscopy during lung cancer evaluation. The bronchial lesions were diagnosed as extranodal MZL. Although the patient underwent surgical resection for the lung adenocarcinoma, the pulmonary extranodal MZL was left untreated; it was monitored during follow-up visits. To our knowledge, this is the first report of synchronous lung adenocarcinoma and primary extranodal MZL of the main bronchus.
Lymphoma, B-Cell, Marginal Zone; Adenocarcinoma of Lung; Lung Neoplasms
Extranodal natural killer/T-cell lymphoma (ENKTL) is a distinct subtype of non-Hodgkin’s lymphoma and is rare in the colon. Synchronous adenocarcinoma and ENKTL of the colon has not been reported in the literature. In the present study, we report a 63-year-old male who suffered from intermittent bloody stools for 2 mo. He did not have fever, body weight loss or night sweat. Endoscopic and imaging studies revealed a 4.5-cm ulcerative mass in the ascending colon and a 3.0-cm polypoid, easy bleeding mass in the sigmoid colon, respectively. Thought to have double carcinoma of the colon, he received simultaneous right hemicolectomy and sigmoidectomy. The pathological diagnosis was a synchronous ENKTL (ascending colon) and adenocarcinoma (sigmoid colon). The literature on synchronous adenocarcinoma and malignant lymphoma of the colon was also reviewed.
Synchronous cancers of the colon; Colonic adenocarcinoma; Colonic lymphoma; Extranodal natural killer/T-cell lymphoma; Epstein-Barr virus
New developments in the treatment of patients with relapsed/refractory Hodgkin's lymphoma undergoing autologous stem cell transplantation are summarized including modern prognostic markers, the role of functional imaging, the role of newer drugs, different conditioning regimens, and maintenance therapy.
Despite the relatively high long-term disease-free survival (DFS) rate for patients with Hodgkin lymphoma (HL) with modern combination chemotherapy or combined modality regimens, ∼20% of patients die from progressive or relapsed disease. The standard treatment for relapsed and primary refractory HL is salvage chemotherapy followed by high-dose chemotherapy and autologous stem cell transplantation (ASCT), which has shown a 5-year progression-free survival rate of ∼50%–60%. Recent developments in a number of diagnostic and therapeutic modalities have begun to improve these results. Functional imaging, refinement of clinical prognostic factors, and development of novel biomarkers have improved the predictive algorithms, allowing better patient selection and timing for ASCT. In addition, these algorithms have begun to identify a group of patients who are candidates for more aggressive treatment beyond standard ASCT. Novel salvage regimens may potentially improve the rate of complete remission prior to ASCT, and the use of maintenance therapy after ASCT has become a subject of current investigation. We present a summary of developments in each of these areas.
Autologous stem cell transplant; Hodgkin's lymphoma; High-dose chemotherapy
Extranodal natural killer/T cell lymphoma, nasal type, is a non-Hodgkin lymphoma, most commonly affecting the nasal cavity, paranasal sinuses and nasopharynx. Clinically it is characterised by destruction of facial tissues, commencing in the midline. In most cases it arises from malignant transformation of natural killer cells (NK); sometimes from malignant transformation of cytotoxic T cells.
Extranodal NK/T cell lymphoma, nasal type, is rare, but even more rare in black persons. The purpose of this article is to report a severe case of extranodal NK/T cell lymphoma, nasal type, in an elderly black male.
Midline lethal granuloma; Extranodal NK/T cell lymphoma
The head and neck are two of the most common sites of extranodal non-Hodgkin's lymphoma (NHL). However, primary tumors of the infratemporal fossa are infrequent, and NHL in this region is extremely rare.
We present a case of a 41-year-old female that presented with swelling in the right preauricular region that had persisted for the past two years. The patient was diagnosed as having a small lymphocytic NHL. She initially underwent chemo-radiation but reported relapse. The tumor was excised and again the patient underwent chemotherapy. The patient remained symptomatic and developed a second primary squamous cell carcinoma in the right retromolar trigone.
Discussion and conclusion
We discussed NHL with an emphasis on extranodal manifestations. Extranodal NHL that is limited to a single site can be managed by surgery and regular follow up. To the best of our knowledge, this is only the second case of primary NHL of the infratemporal fossa to be reported in the literature.
The subtype distribution of lymphoid neoplasms in Southwest China was analyzed according to WHO classifications. This study aims to analyze subtype distribution of lymphomas in southwest China.
Lymphoid neoplasms diagnosed within 9 years in a single institution in Southwest China were analyzed according to the WHO classification.
From January 2000 to December 2008, a total number of 6,382 patients with lymphoma were established, of which mature B-cell neoplasms accounted for 56%, mature T- and NK-cell neoplasms occupied 26%, and precursor lymphoid neoplasms and Hodgkin lymphomas were 5% and 13%, respectively. Mixed cellularity (76%) was the major subtype of classical Hodgkin lymphoma; and the bimodal age distribution was not observed. The top six subtypes of non-Hodgkin lymphoma were as follows: diffuse large B-cell lymphoma, extranodal NK/T-cell lymphoma, nasal type, extranodal marginal zone lymphoma of mucosa associated lymphoid tissue, follicular lymphoma, precursor lymphoid neoplasms, and chronic lymphocytic leukemia/small lymphocytic lymphoma. Extranodal lymphomas comprised about half of all cases, and most frequently involved Waldeyer's ring, gastrointestinal tract, sinonasal region and skin.
The lymphoid neoplasms of Southwest China displayed some epidemiologic features similar to those reported in literature from western and Asian countries, as well as other regions of China, whereas some subtypes showed distinct features. The high frequency of mature T/NK cell neoplasms and extranodal lymphomas, especially for extranodal NK/T-cell lymphoma, nasal type, is the most outstanding characteristic of this series.
Distribution; Lymphoma; Subtype; WHO classification
Reduced-intensity-conditioning (RIC) prior to allogeneic hematopoietic cell transplantation (HCT) is increasingly employed as a potentially curative option for patients with advanced lymphoma; however relapse remains a major challenge. Unfortunately, little data exist on the outcomes, predictors of survival, and results of specific management strategies of such individuals. One-hundred-one consecutive relapses occurred and were evaluated in 280 lymphoma patients following RIC-HCT. Characteristics included: aggressive non-Hodgkin lymphoma (NHL) (n=42), indolent NHL (n=33) and Hodgkin’s Lymphoma (HL) (n=26). Median time to relapse was 90 (range 3 - 1275) days and graft-versus-host-disease at relapse was present in 56 (55%) patients. Interventions following relapse included no therapy (n=14), withdrawal of immunosuppression alone (n=11), chemoradiotherapy (n=60), and donor lymphocyte infusion/second HCT (n=16). Overall survival (OS) at 3 and 5 years following relapse was 33% (95%CI 23-44%) and 23% (95%CI 13-34%), respectively. Aggressive NHL (vs indolent disease, HR=2.29, p=.008) and relapse <1 month after HCT (vs >6 months HR=3.17 p=.004) were each associated with increased mortality. Estimated 3-year OS after relapse for aggressive, indolent and HL was 16% (95%CI 5-32%), 40% (95%CI 19-61%) and 47% (95%CI 29-64%), respectively. The 1-year survival for patients relapsing within 1 month of HCT was 24% as compared to 52%, 74%, and 77%, for those relapsing at 1-3 months, 3-6 months, and >6 months after HCT. We conclude that despite relapse of lymphoma after RIC-HCT, some patients can experience prolonged survival with better post-relapse outcomes occurring in patients with indolent NHL, HL or late relapse.
Peripheral T-cell lymphomas (PTCLs) comprise a rare and heterogeneous subset of non-Hodgkin’s lymphomas (NHLs) that arise from post-thymic T-cells or natural killer (NK)-cells at nodal or extranodal sites. Worldwide, PTCLs represent approximately 12% of all NHLs and the 2008 World Health Organization (WHO) classification includes over 20 biologically and clinically distinct T/NK-cell neoplasms that differ significantly in presentation, pathology, and response to therapy. Because of the rarity and heterogeneity of these diseases, large clinical trials have not been conducted and optimal therapy is not well defined. Most subtypes are treated with similar combination chemotherapy regimens as used for aggressive B-cell NHL, but with poorer outcomes. New treatment combinations and novel agents are currently being explored for PTCLs and this review highlights a number of options that appear promising.
treatment; non-Hodgkin’s lymphoma; novel therapy; natural-killer cells