Maffucci’s syndrome: a case report

Maffuccis syndrome is a congenital nonhereditary syndrome characterized by a combination of enchondromatosis and hemangiomatosis. It is associated with an increased risk of malignancy. We report a case of a 24-year-old woman who developed severe bone distortion due to generalized enchondromas since the age of 2 years. Physical examination showed a marked shortening of both upper and lower limbs and cutaneous hemangioma in the legs. Radiological study showed symmetric well-circumscribed radiolucent cystic lesions in the hands and severe metaphysical irregularity and a shortening of all long bones. The biopsy of the third right metacarpal showed enchondroma tissue. Histological examination of the skin specimen showed a hemangioendothelioma. The diagnosis of Maffuccis syndrome was established. Abdominal ultrasound, mammography, cerebral computed tomography (CT) scan, and cervical CT were normal.     

Autologous tandem transplantation in patients with primary progressive or relapsed/refractory lymphoma

Patients with primary progressive or refractory Hodgkins disease (HD) or aggressive non-Hodgkins lymphoma (NHL) have a particularly poor prognosis. Here we report the results of autologous tandem transplantation in these patients. Patients aged 18–55 years with primary progressive or refractory relapsed HD and aggressive NHL were included. Patients received high-dose etoposide (2000 mg/m²) followed by peripheral blood stem cell harvest (PBSC). The first high-dose chemotherapy (TMC) consisted of thiotepa (750 mg/m²), mitoxantrone (40 mg/m²), and carboplatin (990 mg/m²). Patients with no change (NC), partial remission (PR), or complete remission (CR) after TMC then received BEAM with carmustine (300 mg/m²), etoposide (1200 mg/m²), cytarabine (1600 mg/m²), and melphalan (140 mg/m²). Patients with bulky disease (>5 cm) or residual lymphoma received involved field radiotherapy. Twenty-five patients were included (HD=10, NHL=15, median age 34 years). Two patients with HD achieved a CR and five patients a PR [response rate (RR) 70%]. Three patients (30%) experienced treatment failure including two deaths due to peritransplant complications. Five patients with aggressive NHL were in CR and two patients in PR (RR 46%). Of the eight patients (56%) with treatment failure, three had progressive disease and five died from peritransplant complications. Freedom from treatment failure (FFTF) and overall survival (OS) for all patients after 12 months was 28% and 40%, respectively. Tandem HDCT followed by autologous stem cell transplantation (ASCT) offers a chance of cure in these poor prognostic patients, but is associated with risks.J.-P. Glossmann and J. O. Staak have contributed equally to this work.     

γ/δ Receptor-expressing T-cell clones from a cutaneous T-cell lymphoma suppress hematopoiesis

Summary Recently we described a cutaneous T-cell lymphoma expressing the / T-cell receptor [5]. The patient suffering from this lymphoma showed low numbers of myeloid and T cells in peripheral blood, while B and NK cells were relatively increased. In vitro culture of the patient's bone marrow (BM) cells revealed a significant suppression of myeloid/monocyte colony formation (GM-CFU) compared with normal controls. This was not due to infiltration of the BM with lymphoma cells. We speculated that a soluble factor either secreted or induced by the lymphoma cells might be responsible for the marked suppression of hematopoiesis in this patient. From a skin biopsy with infiltrating / T-lymphoma cells we established T-cell clones bearing the / T-cell receptor and resembling the phenotype of the lymphoma cells. The supernatant (SN) of these / T-cell clones reduced the number of colonies in a CFU-GM assay (using normal control BM) in comparison to SN of / T-cell clones established from the same biopsy. This suppression was seen mainly on day 7 of culture and was not neutralized by the addition of placenta-CM. The main mediator of this suppression seems to be IFN-,since it was detectable in high amounts in the SN of these / T-cell tumor clones as well as in the serum of the patient. In addition, anti-IFN- antibodies can reverse the T-cell SN-mediated suppression of CFU-GM. We conclude that high serum levels of interferon-, which is secreted in high amounts from / T-cells grown from a biopsy of a cutaneous lymphoma, can suppress hematopoiesis.Abbreviations TCR T-cell receptor - IFN- interferon- - SN supernatant - placenta CM placenta conditioned medium - BM bone marrow - CFU-GM myeloid/monocyte colony formation - NK cells natural killer cells - Ab antibody M. Wilhelm was supported by theDeutsche Forschungsgemeinschaft (DFG Wi 728-2)     

Presence of interferon and anti-interferon in patients with systemic lupus erythematosus

Summary Sera from 61 patients with systemic lupus erythematosus (SLE) were serially screened over a period of at least 2 years for IFN and anti-IFN antibodies. IFN concentrations were measured both with a cytopathic effect assay and a more sensitive radioimmunoassay. Of the patients 15% (9/61) had IFN in their serum at one or more occasions as measured in the bioassay (6 IU/ml); employing a RIA (1 IU/ml) 28% (17/61) of the patients studied were positive for IFN-. Fifteen patients had a measurable interferonemia over 2–16 months; only two patients had detectable IFN in their serum at only one occasion. In five patients, hourly and daily variations of the IFN titer as measured by RIA were found to amount to less than 80%. The IFN activity found in these sera was characterized as IFN- by means of acid stability, cross-reactivity on heterologous cells, trypsin sensitivity, and neutralization by homologous and heterologous antisera. IFN antibodies were quantified with a neutralization bioassay, an ELISA, and a radioimmunoassay. Of the 61 patients 5% (3) possessed high titers of anti-IFN antibodies which persisted over 2 years. The IFN- antibody positive patients had an inactive form of the disease over years without visceral involvement but decreased serum complement levels (C4, C3, CH50) and repeated episodes of Quincke-like edema.     

Results of three courses of adriamycin, bleomycin, vindesine, and dacarbazine with subtotal nodal irradiation in 189 patients with nodal Hodgkin’s disease (stage I, II and IIIA)

Aim of the study. To evaluate in a prospective trial three courses of an ABVD-like chemotherapy (CT) regimen given before radiation therapy (RT) (subtotal nodal irradiation (STNI)) in favorable stage Hodgkins disease (HD). The efficacy, risk factors and medium-term toxicities are reported. Patient characteristics. Stage I or II with at least one of the following factors, mediastinal involvement, histological type 3, age > 40 years, ESR rate > 50 mm, or stage IIIA. 189 patients with newly diagnosed HD were treated between 01/86 and 01/94 (stage I, n = 33; stage II, n = 129, stage IIIA, n = 27). Three courses of an ABVD-like regimen (adriamycin 25 mg/m², bleomycin 10 mg, vindesine 2 mg/m² and dacarbazine 250 mg/m² day 1 and 8) were given before STNI at 36/40 grays. At diagnosis, prognostic factors were distributed as follows: B symptoms (n = 54), bulky mediastinum (n = 41), hemoglobin < 12 g/dl (n = 37), ESR > 50 (n = 65), age > 45 (n = 24). Results. After chemotherapy, 90% had an objective response (partial response > 75%) and 98% were in complete remission (CR) at the end of RT. Three patients had primary refractory disease and 13 patients (7%) relapsed, 3 at the initial site, 4 at previously uninvolved sites and 6 at both. With a median follow-up of 60 months, 170 patients are in 1st CR, 5 in 2nd or greater CR and 11/14 patients have died from HD. Bulky mediastinum (p = 0.009), age > 45 years (p = 0.03) and ESR >50 mm (p = 0.05) were adverse prognostic factors for survival. Bulky mediastinum (p = 0.009) was the only prognostic factor for freedom from progression. Toxicities. Two patients died from treatment related toxicity and one patient died with an osteogenic sarcoma. No secondary leukemia has so far been detected. 24 pregnancies were reported. Cardiopulmonary toxicity was always < grade 1 (WHO) in 95 patients evaluated. Two patients over 45 years old had a myocardial infarction. Conclusion. With an acceptable medium-term toxicity, this treatment achieved 85% survival at 5 years.     

Acute hematologic effects of interferon alpha,interferon gamma,tumor necrosis factor alpha and Interleukin 2

Summary This study was designed to investigate acute effects of various doses of the cytokines IFN-alpha, IFN-gamma Interleukin 2 and tumor necrosis factor alpha on white blood cell differential counts. Before initiation of phase II trials, a dose-determination phase was performed, where three different dose levels of each cytokine were applied as a single dose. White blood cell differential counts were assessed immediately before and 2, 12, 24, 48 and 168 h after injection. Patients enrolled suffered from metastatic cancer or chronic active hepatitis. In addition, IFN-alpha was administered to five healthy volunteers. Results indicate that cytokines cause rapid and transient changes in the numbers of leukocyte subsets. Hematologic changes were cell-type- and cytokine-specific: transient lymphopenia was observed after administration of all four cytokines, reaching a nadir 12 to 24 h after subcutaneous injection. Administration of TNF-alpha and IFN-gamma also caused transient monocytopenia. Neutrophilia developed after administration of Interleukin 2, IFN-alpha and TNF-alpha. We conclude that cytokines play a key role in the regulation of peripheral blood cell traffic by their capacity to influence homing patterns of peripheral blood leukocytes.     

Long-term results of autologous stem cell transplantation for Hodgkin’s disease (HD) and low-/intermediate-grade B non-Hodgkin’s lymphoma (NHL): a report from the Austrian Stem Cell Transplantation Registry (ASCTR)

Between 1990 and 2001, 68 patients with advanced Hodgkin's disease (HD) and 86 patients classified as low-/intermediate-grade B non-Hodgkin's lymphoma (NHL) were reported to the Austrian Stem Cell Transplantation Registry (ASCTR). Following autologous stem cell transplantation (SCT) for HD, overall survival was 56% [95% confidence interval (CI): 40-72%] with a disease-/progression-free survival of 49%, reaching a plateau at 5 years. Using multivariate Cox regression analysis BEAM conditioning (carmustine, cytarabine, etoposide and melphalan) was predictive for favourable outcome, better disease-/progression-free survival and a significantly lower risk for relapse. The cumulative incidence of relapse was 30%, even for patients in complete remission at time of SCT. The cumulative risk for developing a secondary malignancy increased continuously over time, achieving 20% at 7 years and 46% at 10 years with previous radiotherapy as the only risk factor in the multivariate analysis. Overall survival for NHL patients was 45% (95% CI: 26-64%) with a disease-/progression-free survival of 26% at 7 years. In the multivariate Cox regression analysis stage of disease at time of SCT was the most powerful parameter for overall survival, disease-/progression-free survival and relapse. Mantle cell lymphoma, greater than or equal to three lines of previous therapy, and a conditioning regimen other than BEAM were also predictive for death. The main reason for treatment failure was relapse (cumulative incidence 54-75%). Because of the high risk of relapse/progression in both disease categories and the additional high rate of second malignancies in HD patients, allogeneic stem cells should be considered a valuable alternative for selected patients. The efficacy of allotransplantation following reduced-intensity conditioning should be tested in randomised trials.     

Rapidly-progressive glomerulonephritis in a patient with Behcet’s disease: successful treatment with intravenous cyclophosphamide

We report a case of rapidly-progressive glomerulonephritis complicating Behcets disease (BD). A 44-year-old male has suffered from recurrent oral ulcers and retinal vasculitis developed 2 years ago. He complained of abdominal pain and papulopustular skin lesions. Multiple ulcers were seen on the colon on colonoscopy. Routine renal work-up revealed heavy proteinuria and hematuria. Renal biopsy demonstrated crescentic glomerulonephritis. Most symptoms improved after steroid therapy, except for urinary abnormalities. At this point, intravenous monthly cyclophosphamide pulse therapy was undergone. After the sixth pulse therapy, proteinuria and hematuria were dramatically improved and renal function was well preserved.     

Association of systemic and thyroid autoimmune diseases

Objective: There are few large cohort studies available on the association of systemic and thyroid autoimmune diseases. In this study, we wished to determine the association of Hashimotos thyroiditis (HT) and Graves disease (GD) with systemic autoimmune diseases. Methods: One thousand five hundred and seventeen patients with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), mixed connective tissue disease (MCTD), Sjögrens syndrome (SS) and polymyositis/dermatomyositis (PM/DM) were included in the study. The HT and GD were diagnosed based on thorough clinical evaluation, imaging and fine-needle aspiration cytology (FNAC). The frequency of HT and GD in these diseases was assessed. In addition, 426 patients with HT or GD were assessed and the incidence of SLE, RA, SSc, MCTD, SS and PM/DM among these patients was determined. Prevalence ratios indicating the prevalences of GD or HT among our autoimmune patients in comparison to prevalences of GD or HT in the general population were calculated. Results: Altogether 8.2% of systemic autoimmune patients had either HT or GD. MCTD and SS most frequently overlapped with autoimmune thyroid diseases (24 and 10%, respectively). HT was more common among MCTD, SS and RA patients (21, 7 and 6%, respectively) than GD (2.5, 3 and 1.6%, respectively). The prevalences of HT in SLE, RA, SSc, MCTD, SS and PM/DM were 90–, 160–, 220–, 556–, 176– and 69-fold higher than in the general population, respectively. The prevalences of GD in the same systemic diseases were 68-, 50-, 102-, 76-, 74- and 37-fold higher than in the general population, respectively. Among all thyroid patients, 30% had associated systemic disease. In particular, 51% of HT and only 16% of GD subjects had any of the systemic disorders. MCTD, SS, SLE, RA, SSc and PM/DM were all more common among HT patients (20, 17, 7, 4, 2 and 2%, respectively) than in GD individuals (2, 5, 5, 1, 2 and 1%, respectively). Conclusion: Systemic and thyroid autoimmune diseases often overlap with each other. HT and GD may be most common among MCTD, SSc and SS patients. On the other hand, these systemic diseases are often present in HT subjects. Therefore it is clinically important to screen patients with systemic autoimmune diseases for the co-existence of thyroid disorders.Take home message: Autoimmune thyroid diseases, such as Hashimotos thyroiditis and Graves disease are often associated with systemic autoimmune diseases, most commonly with Sjögrens syndrome and mixed connective tissue disease.     

Spondylodiscitis as the first manifestation of Whipple’s disease –a removal worker with chronic low back pain

Whipples disease is a rare systemic infectious disease caused by the actinobacterium Tropheryma whipplei. Spondylodiscitis is an extremely rare manifestation of the infection and has previously been described in only three case reports. We present a 55-year-old man with persistent lumbago and signs of systemic illness, but without any gastrointestinal symptoms or arthralgia. The signal response in the lumbar spine in magnetic resonance tomography, both native and after intravenous gadolinium administration, was compatible with spondylodiscitis at the L4/L5 level. Culture of a specimen obtained by radiographically guided disc puncture and repeated blood cultures remained sterile. Tropheryma whipplei was detected by PCR amplification in material obtained from the disc specimen, from a biopsy of the terminal ileum and from the stool. The histology of duodenum, terminal ileum, colon and disc material was normal and, in particular, showed no PAS-positive inclusions in macrophages. Long-term antibiotic treatment with sulphamethoxazole and trimethoprim was successful, with marked improvement of the low back pain and normalisation of the systemic inflammatory signs. The possibility of Whipples disease must be suspected in the case of a culture-negative spondylodiscitis even if there are no gastrointestinal symptoms and no arthralgia present.Abbreviations PAS Periodic acid–Schiff - PCR Polymerase chain reaction - PSA Prostate-specific antigen - EDTA Ethylene diamine tetra-acetic acid     

Pulmonary artery aneurysm in Behcet’s disease: a case report

The pulmonary artery is the second most common site of arterial involvement in Behcets disease. A 32-year-old man presented with bilateral ankle edema, abdominal discomfort, and hemoptysis. He had a history of recurrent oral and genital aphthous ulcerations for 1 year. The diagnosis of Behcets disease was made on the basis of the criteria published by the International Study Group for Behcets Disease. His chest X-ray revealed left hilar enlargement. A helical computed tomography (CT) scan showed a pulmonary aneurysm with intramural thrombosis in the left pulmonary artery and enlarged hepatic veins. Treatment with colchicine and cyclophosphamide was given for 24 months, and helical thoracic CT was performed again. Helical CT showed that the pulmonary aneurysm was reduced by treatment. Helical CT could be used in Behcets disease for the diagnosis and follow-up of pulmonary involvement.     

Systemic necrotizing vasculitides in Turkey: a comparative analysis of 40 consecutive patients

Objective: The aim of this study was to analyze and compare the demographic and clinical features and prognosis of patients with different systemic necrotizing vasculitides (SNV) in Turkey. Patients and methods: Twenty-three patients with Wegeners granulomatosis (WG), 15 with polyarteritis nodosa (PAN), and two with Churg-Strauss syndrome were included in the study. The clinical and laboratory features of patients with WG and PAN were compared, and survival analysis was performed for the WG patients. Results: Twenty-one patients with WG had systemic disease involving kidneys, and two had localized disease. Fifteen patients were placed in the PAN group, 12 of whom were classified as having classic PAN and three with microscopic polyangiitis. Median follow-up time was 37 months (range 1–81) for WG patients and 41 months (range 5–132) for the PAN group. Upper respiratory tract, pulmonary, and renal involvement were significantly more frequent in the WG group than in PAN. Peripheral nervous system involvement was more frequent in the PAN group. In WG, survival was calculated as 59% at 35 months. High initial vasculitis damage index scores were found to be predictive for mortality. Conclusion: This study revealed that the most frequent type of SNV was WG in a tertiary rheumatology setting in Turkey. There was initial organ damage in most of the patients, frequently caused by severe renal involvement. In contrast to other published series, overt cardiovascular and gastrointestinal involvement were not observed in our patients with SNV.     

Monostotic Paget’s disease of the tibia in Korea

The incidence of Pagets disease has been estimated to be about 3%, but it is extremely rare in Asia, especially in Korea. In addition, monostotic involvement seems to be far less frequent. In this report, we describe a case of monostotic Pagets disease localized in the right tibia.Abbreviations ALP alkaline phosphatase     

Primary adrenal lymphoma presenting as Addison’s disease: case report and review of the literature

Primary adrenal lymphoma (PAL) is an extremely rare entity and adrenal insufficiency is a common complication. Bilateral enlargement of adrenal glands should raise the suspicion of lymphoma, especially in patients with clinical or laboratory features of adrenal insufficiency. Most of these cases are highly aggressive tumors and should be treated with multiagent chemotherapy. The role of bilateral adrenalectomy and/or radiotherapy cannot be estimated. Our patient with PAL was an 80-year-old man who presented with clinical and laboratory features of adrenal insufficiency. Combination chemotherapy plus rituximab was unsuccessful and our patient died from progressive disease.     

Crohn’s disease complicated by adult-onset Still’s disease

A 31-year-old man with Crohns disease developed arthritis, spiking fever, and skin rash indistinguishable from that of adult-onset Stills disease. He was admitted to our hospital because of a periumbilical intestinal skin fistula. Crohns disease had been diagnosed in 1991, and had required intestinal resection twice, and schizophrenia had been diagnosed in 1993. He developed polyarthritis and spiking fever, accompanied by a macular skin rash on both forearms. Marked hepatosplenomegaly and bilateral pleural effusion were detected on computed tomography examination. These findings are indistinguishable from those of adult-onset Stills disease. Because his mental status had deteriorated following high-dose prednisolone on a previous admission, he was treated with an immunosuppressive agent on this occasion, with the treatment being successful. This is the first report of adult-onset Stills disease complicating Crohns disease. In patients with Crohns disease, polyarthritis and skin rash can easily be misdiagnosed as enteropathic arthritis with erythema nodosum associated with the Crohns disease. Although adult-onset Stills disease may not be fatal, early diagnosis is important because it can, in rare cases, result in life-threatening complications.     

VECP-Bleo regimen as treatment of advanced stage aggressive non-Hodgkin’s Lymphoma in elderly patients a GOELAMS protocol

We evaluated the value of a CHOP-like regimen in the treatment of Non-Hodgkins lymphoma (NHL) in elderly patients in which Doxorubicin was replaced by Epiadramycin and Bleomycin was added.One hundred and thirty-four previously untreated intermediate or high-grade NHL aged 60 years or more were included in the study. Two patients were stage II Bulky, 51 stage III and 81 stage IV. Fifty percent of patients presented poor performance status and 79 out of 119 patients had increased LDH level. Complete remission was obtained in 67% of the patients and partial remission in 8%. With a median follow-up of 58 months, the median overall survival was 25 months (95%CI = (18 41)) with a 35% survival rate at 5 years (95%CI = (26 45)). Univariate and multivariate prognostic factor analysis was performed. For overall survival, univariate analysis showed histology, LDH level, performance status and age greater than 70 year old as prognostic factors. Multivariable analysis showed poor performance status and LDH level as prognostic factors of overall survival, independently than being more than 70 year old. For event-free survival and disease-free survival, the only prognostic factor was 2 microglobulin.This study confirms that CHOP-like regimen give about 35% of long-term survivors in the treatment of aggressive non-Hodgkins lymphoma in elderly patients and that new therapeutic approaches are needed to improve the prognosis of these patients.     

Myocardial perfusion in new-onset angina patients with single vessel disease

The aim of this study was to investigate myocardial perfusion of the left ventricle during stress (exercise scintigraphy with thallium-201) in 25 patients having new-onset angina (NA) with single-vessel disease. In 14 cases (56%) the lumen of the vessel was obstructed by more than 75% and in 6 cases (24%) it was totally occluded. Angiographically visible collaterals to the occluded arteries from other vessels were present in all these cases. All of the patients studied had some form of myocardial perfus ion disturbance. In 13 patients (52%) perfusion defects were limited to distribution within the obstructed artery; the other 9 patients (36%) had defects in the pool of the intact artery with or without defects in the pool of the obstructed artery. In the 4 patients (44,4%) having a totally occluded artery, perfusion defects were observed only in the pool of the intact artery supplying the collaterals, while in 5 patients (55,6%) perfusion defects were found both in the pool of the intact artery supplying the collaterals as well as in the obstructed vessel. We conclude that in patients with NA, stress dependent perfusion defects are present not only in the pool of the obstructed artery but also in the intact artery supplying the collaterals. This points to the possible presence of steal syndrome in these patients.     

Lack of association of tumor necrosis factor-alpha gene polymorphisms with disease susceptibility and severity in Behçet’s disease

Although it has been reported that the MHC class I molecule, HLA-B51, is a risk factor for Behçets disease (BD), contribution of the tumor necrosis factor (TNF) genes, which are located in the vicinity of the HLA-B locus, to the genetic susceptibility for BD has yet to be elucidated. The purpose of this study was to analyze the effect of TNF- promoter polymorphisms at positions –308, –238 and –376 on the susceptibility, severity and clinical features of BD. The TNF- gene sequences from 107 patients with BD and 102 healthy subjects were amplified by the polymerase chain reaction. Sequence analysis of the TNF- gene locus, which contains promoter polymorphisms at positions –376, –308, and –238, was performed with a DNA sequencing kit on automated sequencer. The patients were classified according to disease severity and clinical features. Serum TNF- level in the study groups was measured by sandwich enzyme immunoassay. In patients with BD the frequencies of TNF- –308 (19.4% vs 18.4%), –238 (3.7% vs 5.9%), and –376 (0.9% vs 2.9%) gene polymorphisms were not found to be significantly different from those in healthy subjects. The TNF- gene polymorphisms did not show any association with disease severity or clinical features. Serum TNF- level was significantly higher in patients with BD than in healthy controls (3.10±1.45 pg/ml vs 2.43±1.94 pg/ml, P < 0.01). Serum TNF- level was not found to be significantly associated with disease severity, activity, clinical findings and TNF- genotypes. The results of this study suggest that the TNF- gene polymorphisms are unlikely to play an important role in the pathogenesis and severity of BD.     

Treatment results of tonsillar lymphoma: a 10-year experience

Primary extranodal non-Hodgkins lymphomas of the head and neck account for 10–20% of all non-Hodgkins lymphomas. Primary tonsillar lymphoma accounts for less than 1% of head and neck malignancies, although the tonsil is the most common primary extranodal site of head and neck non-Hodgkins lymphomas. In this study we analyzed our cases of tonsillar lymphoma treated in our institution during the last 10 years to compare the finding of this study with those of previous studies. We reviewed the cases of tonsillar lymphoma treated in the Radiation Oncology Department of Shiraz University from 1992 to 2002. Clinical data were obtained from patients files. The patients were treated by combined chemotherapy [a median of six cycles of a CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisolone)] and radiation therapy (40–50 Gy to the primary site and neck). Chemotherapy mainly preceded radiotherapy, although the sequence of radiotherapy and chemotherapy was determined by individual physicians and patients choice. Surgery was used mainly to establish the diagnosis, and tonsillectomy was performed for localized small lesions. Between 1992 and 2002, 19 patients with stage IE (10), IIE (7), and IIIE (2) disease were treated. Median and mean age was 48 and 44 years (range: 22–76 years), respectively, at the time of diagnosis, with a male to female ratio of 1.2:1. The vast majority of patients presented in early stages with aggressive histology. High-grade tumors seemed to affect mainly young people (p=0.226). Diffuse large B-cell lymphomas were the most prevalent. Male patients were significantly younger than females (p=0.021). The patients were treated by combined chemotherapy and radiation therapy. All patients achieved and maintained complete remission with a median of 60 months relapse-free survival and a 5-year cause-specific survival rate of 100%. All patients developed some degree of oropharyngeal mucositis. Three patients (16%) experienced grade 3 or 4 neutropenia. Mild (grade I) xerostomia remained persistently in four patients (21%). A late fatal side effect was observed in one patient who developed radiation-induced sarcoma 7 years after initial diagnosis and died 8 months later without evidence of recurrent lymphoma. Complete follow-up was obtained in all patients. The follow-up period ranged from 18 to 141 months with a median of 60 and a mean of 60.4 months. At the time of last follow-up, all patients but one were alive. Age, sex, stage, bulk of disease, performance status, number of chemotherapy cycles, number of involved sites, histologic subtypes, and radiation dose were analyzed as prognostically significant for disease-specific survival in our cases. Significant prognostic factors were not identified by multivariate analysis. Combined chemotherapy and radiation therapy is safe, highly effective, and probably curative for most patients with primary tonsillar lymphoma.An erratum to this article can be found at