Primary non-Hodgkin's lymphoma of the brain

Between 1980 and 1993, 26 patients were treated for primary lymphoma in the central nervous system at the Norwegian Radium Hospital. This is a rare disease with poor prognosis and thus represents a great therapeutic challenge. Immunocompromised, e.g. AIDS patients, are a group at high risk, but the incidence has increased among immunocompetent patients as well. Median patient age was 64 years; and none of the patients had any signs of immunodeficiency. 23 of the patients received radiation therapy. 13 of the patients received some form of chemotherapy. The overall median survival was 19 months. WHO performance status 0-2, unifocal lesion, absence of steroid dependency and normal serum levels of LDH were all associated with longer survival. Although complete remissions were achieved in most patients, relapses in the central nervous system were frequent.     

Primary non-Hodgkin's lymphoma of the liver

Primary non-Hodgkin's lymphoma of the liver is an extremely rare lymphoma subset that often presents with diagnostic difficulties to both clinicians and pathologists. Using MEDLINE search, 90 cases of primary hepatic lymphomas reported in the literature were reviewed. The epidemiology and etiology, clinical presentation, pathologic features, management, and outcome of these patients have been summarized and described. Results of this review show that middle-aged males are most often affected. Abdominal pain or discomfort, weight loss and fever are the most frequent presenting symptoms. Most cases have a solitary or multiple mass lesions in the liver, and are frequently misdiagnosed as having a primary liver tumor or metastatic cancer. Diffuse large cell lymphoma is the most commonly encountered histologic subtype. Surgery, chemotherapy and radiotherapy have been used alone or in combination as treatment but the outcome is generally poor. Although primary hepatic lymphoma is an aggressive disease, it is resectable, and responsive to chemotherapy and radiotherapy. Because of the profound therapeutic implications, it should be considered in the differential diagnosis for patients presenting with mass lesions in the liver or hepatic disease.     

Bilateral non-Hodgkin's lymphoma of the adrenal glands with adrenal insufficiency

A 66-year-old female presented with anorexia, fatigue, skin pigmentation, weight loss and low grade fever. Imaging studies demonstrated bilateral bulky masses confined to the adrenal glands. Ultrasonography guided needle biopsy of the mass showed findings of diffuse large B-sell lymphoma. Low levels of serum cortisol, urinary 17-OHCS and 17-KS, a high level of ACTH and a non-reactive pattern on the rapid ACTH test led to a diagnosis of Addison's disease. Only a partial regression was achieved by the first chemotherapy. She died due to disease progression, while the next course of chemotherapy had been postponed because of interstitial pneumonitis due to methotrexate.     

Bilateral primary non-Hodgkin's lymphoma of the testis

We report the case of a 72-year-old man with bilateral testicular masses that, on histologic section, were found to be synchronous non-Hodgkin's lymphomas. Workup was negative for systemic disease, indicating the possibility of bilateral primary testicular lymphomas. We discuss the evaluation and treatment of this lesion and review the literature concerning this subject.     

HIV-related primary non-Hodgkin's lymphoma of the vulva

We reported on a 25-year-old HIV-positive woman diagnosed with Ann Arbor Stage IEB primary extranodal immunoblastic lymphoma arising in the vulva. This is the first documented instance of an HIV-associated malignant lymphoma originating in the lower female genital tract, and only the 16th reported case of primary malignant lymphoma of the vulva. The nonspecific clinical presentation coupled with the unanticipated finding of lymphoma made diagnostic confirmation an arduous task, requiring weeks of persistence. The patient's disease was refractory to three courses of chemotherapy, but did respond to a brief palliative course of external beam irradiation prior to her demise 7 months after presentation. Our findings are presented, and 15 cases from the literature, the largest series to date, are discussed.     

Quality of life in patients with low-grade non-Hodgkin's lymphoma

Low-grade non-Hodgkin's lymphoma (NHL) is an indolent form of the disease with a generally slow course of progression. Although still usually incurable, low-grade disease has shown responsiveness to some of the newer chemotherapeutic and nonchemotherapeutic treatment options. However, since cure remains elusive, and since many patients with lowgrade NHL may have few or even no symptoms initially, the decision about whether or not to initiate treatment logically must include quality-of-life (QOL) issues. This paper summarizes clinical and diagnostic characteristics of low-grade NHL that have some bearing on QOL considerations. Adverse effects of the more common treatment approaches are discussed according to their QOL implications, illustrating the relevance of QOL to the clinical management of low-grade disease. Finally, data from an ongoing study using the Functional Assessment of Cancer Therapy (FACT) measurement system are presented. These data offer a basis for comparing the QOL of patients with NHL to that of individuals with other solid tumors, and also illustrate the effects of chemotherapy on QOL.     

Primary forms of extralymphatic non-Hodgkin's lymphoma

In 1981-1990, 584 patients with non-Hodgkin malignant lymphoma, 209 women and 375 men, aged between 17 and 75 years, were treated at the I Department of Medicine and Department of Hematology, Silesian Medical Academy in Katowice. In this group the primary localization outside lymph nodes was seen in 26 patients (9 women and 17 men), i.e. 4.6% of all patients with malignant non-Hodgkin lymphomas. Most frequently digestive tract was involved-19 patients (73.1%). Twenty three patients were treated surgically followed by chemotherapy and/or irradiation. Not operated patients were treated with either chemotherapy or both radio- and chemotherapy. Complete remission was achieved in 17 patients (65.4%), partial remission in 4 (15.4%) patients. Five patients did not respond to therapy (19.2%). A mean survival time was 49.4, 14.3, and 6.4 months, respectively.     

Immunotoxin combined with chemotherapy for patients with AIDS-related non-Hodgkin's lymphoma

BACKGROUND: The objective of this study was to develop and test a combined therapeutic approach for patients with AIDS-related lymphoma (ARL), employing agents with independent mechanisms of action and nonoverlapping toxicity. This study was designed to test the feasibility and tolerance of combining low dose chemotherapy with infusional immunotoxin in the treatment of ARL patients. METHODS: Previously untreated patients received low dose methotrexate, bleomycin, doxorubicin, cyclophosphamide, and vincristine (m-BACOD) on a 21- to 28-day schedule. Patients who did not have progressive disease by Cycle 3 received anti-B4-blocked ricin (anti-B4bR), a murine monoclonal antibody linked to modified ricin, 20 microg/kg/day for 7 days administered by continuous infusion on an outpatient basis. A repeat cycle of anti-B4-bR was administered during Cycle 4 of chemotherapy based on tolerance. Patients received two cycles of chemotherapy beyond complete remission up to eight cycles. Study endpoints were toxicity, development of human antimurine antibody (HAMA) and human antiricin (HARA), tumor response, and survival. RESULTS: Twenty-six of 44 patients received the immunotoxin therapy. Anti-B4-bR infusion was associated with transaminase elevation (Grade 3) in 14 of 26 patients (58%), and flulike symptoms were common. HAMA or HARA was observed in 8 patients (31%). The overall response rate was 57% (13 complete responses and 12 partial responses). The median survival for all patients was 8.9 months. CONCLUSIONS: This study demonstrates the safety and feasibility of using chemotherapy and immunotoxin therapies in combination and supports their further evaluation to improve the outcomes of patients with ARL.     

Primary non-Hodgkin's lymphoma of the common bile duct

Primary non-Hodgkin's lymphoma of the common bile duct is rare. To date, nine cases have been recorded in the literature. We report an additional case of a 39-yr-old woman presented with obstructive jaundice. Pathological studies of the surgical specimen disclosed that the wall of the common bile duct was transmurally infiltrated by non-Hodgkin's lymphoma of diffuse large cell type of B-cell lineage intimately associated with reticular fibers. The patient received postoperative brachytherapy, followed by six cycles of chemotherapy according to the CHOP regimen. There is no evidence of lymphoma recurrence 13 months after the surgery. Our analysis of the reported cases indicates that common bile duct non-Hodgkin's lymphoma is a rapidly progressive disease, terminating in death within a year. A complete surgical resection of the lymphoma followed by chemotherapy has shown a promising result.     

Treatment of children and young adults with early-stage non-Hodgkin's lymphoma

BACKGROUND: Children and young adults with early-stage non-Hodgkin's lymphoma have an excellent prognosis, but treatment is prolonged and is associated with many side effects. We performed two studies to determine whether therapy could be simplified. METHODS: Between 1983 and 1991, we conducted two consecutive trials in children and young adults (age, <21 years) with early-stage non-Hodgkin's lymphoma. In the first trial, patients were treated for 9 weeks with induction chemotherapy consisting of vincristine, doxorubicin, cyclophosphamide, and prednisone, followed by 24 weeks of continuation chemotherapy with mercaptopurine and methotrexate. Half the patients were randomly assigned to receive involved-field irradiation. In the second trial, after the 9 weeks of induction chemotherapy, the patients were randomly assigned to receive 24 weeks of continuation chemotherapy or no further therapy. RESULTS: A total of 340 patients were enrolled in the two trials, 12 of whom did not have complete remissions. One hundred thirteen patients received nine weeks of chemotherapy without radiotherapy, 131 received eight months of chemotherapy without radiotherapy, and 67 received eight months of chemotherapy with radiotherapy. At five years, the projected rates of continuous complete remission were 89, 86, and 88 percent for the three groups, respectively. At five years, event-free survival among the patients with early-stage lymphoblastic lymphoma was inferior to that among the patients with other subtypes of lymphoma (63 percent vs. 88 percent, P<0.001). Continuation therapy was effective only in patients with lymphoblastic lymphoma. CONCLUSIONS: A nine-week chemotherapy regimen without irradiation of the primary sites of involvement is adequate therapy for most children and young adults with early-stage, nonlymphoblastic non-Hodgkin's lymphoma.     

The relationship of non-Hodgkin's lymphoma with P16 protein expression

The expression of P16 protein was examined in 45 cases of non-Hodgkin's lymphoma (NHL), 4 cases of reactive hyperplasia of lymph node (RHLN) and 6 cases of normal spleen with immunohistochemical staining. Positive reaction of P16 protein was observed in some lymphocytes of RHLN and splenic nodule and around splenic sinus. The positive rate of P16 protein expression in NHL was 37.8% (17/45). The positive rates of P16 protein expression for the low intermadiate- and high-grade malignancy groups were 66.7% (10/15), 37.5% (6/16) and 7.1% (1/14) respectively. There was a significant difference between the low malignancy NHL and the high malignancy NHL (P < 0.005) in expression rate of P16 protein. It suggests that P16 protein expression may be one of the important parameters in estimating the prognosis of patients with NHL.     

Structural abnormalities of the X chromosome in non-Hodgkin's lymphoma

It has recently been reported that additional X chromosomes occur in over 30% of B-cell non-Hodgkin's lymphomas (NHL), and that monosomy of the X chromosome occurs in 38% of female patients with T-cell leukaemia or lymphoma. These observations have suggested a possible role for the X chromosome in the evolution of NHL. We have now examined 280 cases of NHL, and have identified 19 examples of structurally altered X chromosomes in the malignant cells from 17 of these cases. These abnormalities were mainly characterized by either a translocation involving Xp22, or a translocation/deletion involving Xq28. The relevance of these observations is discussed with respect to other published reports, and together they suggest that lymphoma-associated oncogenes may exist on the X chromosome at bands p22 or q28.     

Current guidelines for the management of aggressive non-Hodgkin's lymphoma

The prognosis of aggressive non-Hodgkin's lymphoma (NHL) has improved greatly during recent years with the use of combination chemotherapy. Planning the treatment must take into consideration the patient's age, performance status, histological subtype and disease extent and severity. Recently, a 4-part International Prognostic Index (IPI), based on 5 prognostic factors, has permitted the allocation of patients with NHL in 2 well defined prognostic groups: good prognosis (low and low-intermediate risk) and poor prognosis (intermediate-high and high risk). Conventional chemotherapy with CHOP (a chemotherapeutic regimen consisting of a combination of cyclophosphamide, doxorubicin, vincristine and prednisone) or other equivalent third-generation regimens may be considered the standard treatment for the good prognosis group. In the poor prognosis group the probability of long term survival is less than 40% with conventional chemotherapy. Therefore, an early intensification with high dose therapy following peripheral stem cell transplantation (PSCT) should be considered in the setting of randomised trials. Localised stage disease, defined as stages I-IE and II-IIE without adverse prognostic factors, has a very good prognosis with a long term survival exceeding 80% using brief conventional chemotherapy regimens plus involved field radiotherapy. Refractory or relapsing patients after the drugs of first choice are given who subsequently respond to salvage chemotherapy should be enrolled for a course of high dose consolidation chemotherapy followed by PSCT. Elderly patients without severe organ dysfunction can take advantage from specifically devised chemotherapy regimens, with a response rate similar to that of younger patients. However, despite major advances in the treatment of aggressive NHL, additional clinical trials are required to enable the clinician to define the best therapeutic programmes to treat patients with this disorder.     

Neuroradiologic diagnosis of primary non-Hodgkin's lymphoma of the brain]

The incidence of primary cerebral lymphomas has risen continuously during the past years. The neuroradiological signs, which are decisive for the differential diagnosis of cerebral lymphomas, are worked up and discussed in this study. Thirty CT and 27 MR investigations of a total of 32 patients (14 males, 18 females, age 60 +/- 15 years) with histopathologically proven cerebral lymphomas were analysed retrospectively. Multiple cerebral lymphomas were detected in 10/32 patients (31%). The cerebral lymphomas appeared on CT scans as hyperdense masses in 28/30 cases (93%). On T2-weighted MR scans 14/27 (52%) lymphomas were slightly hyperintense compared to white matter and 9/27 (33%) inhomogeneously isointense to poorly hyperintense. These were clearly T2-hypointense compared to T2-hyperintense perifocal oedema. In these cases CT density and T2-weighted signal intensity looked like grey matter. All cerebral lymphomas except one case took up contrast medium. An ependymal infiltration or a contact to the ventricle's wall were found in 24/32 cases and an infiltration or a contact to the leptomeningeal space in 15/32 cases. One should consider a cerebral lymphoma as a possible differential diagnosis when a cerebral mass shows the following signs: (1) in CT scans as a hyperdense mass, and on T2-weighted MR images hyperintense compared to white matter and hypointense compared to perifocal edema; (2) clear contrast enhancement and (3) infiltration or broad contact with the ependyma and/or the leptomeningeal space.