Non-Hodgkin's Lymphoma Presenting Research Articles

Primary thyroid lymphoma: a retrospective analysis of prognostic factors and treatment outcome for localized intermediate and high grade lymphoma.

Non-Hodgkin's lymphoma presenting in the thyroid gland is uncommon. A review of the King Faisal Specialist Hospital and Research Centre (KFSH & RC) experience was performed to assess treatment outcome and prognostic factors in this rare extranodal presentation of localized lymphoma. Sixty patients treated at KFSH & RC between 1975 and 1995 were identified, and their records were reviewed retrospectively. Eight patients who had stage III or IV disease, low grade, or did not complete their prescribed treatment were excluded from the study. There were 38 female and 14 male patients with a median age of 59.5 years at the time of diagnosis (range: 10-87 years). Thirty-five of the 52 patients underwent diagnostic partial or total thyroidectomy at other institutions based on a preoperative assumption of thyroid carcinoma. All 52 patients had non-Hodgkin's lymphoma of intermediate (94%) or high (6%) grade. Detailed staging was carried out in all patients; 16 patients (31%) had disease confined to the thyroid gland (stage IE), whereas 36 (69%) had associated disease in cervical lymph nodes and/or the mediastinum (stage IIE) disease. All patients were treated with curative intent. A total of 18 patients (35%) were treated with a single-modality treatment--radiotherapy alone in 2, chemotherapy alone in 13, and surgery alone in the remaining 3 patients. The majority of patients (34/52; 65%) were treated with a combined-modality approach. The overall relapse-free survival (RFS) and overall survival (OS) at 5 years were 72% and 88%, respectively. There were no significant differences in outcome between those treated with single-modality and those with combined-modality therapy. A univariate analysis showed that the presence of mediastinal lymph node involvement was the most important prognostic factor affecting both RFS and OS. Patients with Hashimoto thyroiditis and without "B" symptoms were found to have a significantly higher RFS without influence on the OS. However, patients who had a good performance status (PS) of 0, 1, and 2 were found to have a significantly higher overall survival in comparison to those with poor performance status. Age, sex, stage, histology, lactic acid dehydrogenase level, tumor bulk, and the treatment modality were not found to correlate with RFS or OS. Mediastinal involvement and PS were found to be the most important independent prognostic factors influencing RFS and OS.

Prelymphomatous presentation of T-cell non-Hodgkin lymphomas: A clinical and histopathological study of 11 cases

We report a retrospective study of 11 patients suffering from T-cell non-Hodgkin lymphomas preceded by immunological disorders including two chronic granulomatous diseases, one midline granuloma, four autoimmune hematologic disorders, one hypereosinophilic syndrome, two chronic lymphadenopathies, and one chronic angioedema. In the follow-up of these 11 patients, T-cell non-Hodgkin lymphomas were diagnosed. Even if we cannot exclude a fortuitous association, we feel that these conditions could constitute a ‘prelymphomatous’ stage.

Cauda Equina Compression by Malignant Lymphoma: Long-Term Survival Following Multimodality Therapy

Malignant lymphoma compression of the cauda equina is rare. In the literature only a few cases have been described with occasional long term survivors. We report another case of non-Hodgkin's lymphoma presenting with cauda equina compression. Significant palliation and long-term survival were achieved after therapy by decompressive, cytoreductive surgery; local irradiation; and chemotherapy.

Primary renal non-Hodgkin's lymphoma presenting as massive macrohematuria and bladder tamponade

Primary renal non-Hodgkin's lymphoma presenting as massive macrohematuria and bladder tamponade

Primary renal non-Hodgkin's lymphoma presenting as immune thrombocytopenia

Primary renal non-Hodgkin's lymphoma presenting as immune thrombocytopenia

The Clinical Presentation of Non-Hodgkin Lymphomas of the Major Salivary Glands

The aim of this study was to examine the clinical presentation of patients with malignant lymphoma of the major salivary glands. In a retrospective study, 26 patients with a non-Hodgkin lymphoma (NHL) of the major salivary glands were examined. The results showed a distinct preference for the female gender. Two groups with clinical differences were observed depending on lymphoma manifestation as either extranodal-parenchymal (extranodal) or with intra- or periglandular (nodal) lymph node disease. Differences between these two groups existed with regard to the length of clinical history, recurrent vs continuously progressing symptoms and presentation on ultrasound examination (multiple masses compared to solitary masses). Patients with an extranodal lymphoma always showed disease limited to the affected gland, whereas those patients with a nodal lymphoma presented with stage II or higher (Ann Arbor Classification). In these patients, local recurrence was also five times higher (5/13; 38.4%) than in patients with an extranodal lymphoma (1/13; 7.7%). In 1 patient (7.7%) with extranodal lymphoma, dissemination was observed, compared to 6/13 patients (46.2%) in the group with nodal disease. Seven out of 13 patients (53.8%) with nodal disease died due to lymphoma spread and 1/13 (7.7%) of the patients with extranodal disease. There seem to be distinct clinical differences in the course of patients with NHL of the major salivary glands, depending on extranodal or nodal disease presentation. The histopathological diagnosis, with special recognition of the particular lymphoma pathogenesis, constitutes an important prognostic factor in these patients.

Pathologic Splenic Rupture as the Presentation of Mantle Cell Lymphoma

Pathologic splenic rupture in non-Hodgkin's lymphoma (NHL) is a rare event, with 32 cases previously reported. Initial presentation of NHL with this complication is even rarer. We report such a case in an 80-year-old man with mantle cell lymphoma (MCL). It is notable that of the previously reported cases of pathologic rupture, three have occurred in MCL, suggesting that patients with this uncommon subtype of NHL may be particularly vulnerable to pathologic splenic rupture. Following splenectomy the patient's disease behaved in a high-grade fashion. Despite an initially encouraging response, his disease ran an aggressive course and he succumbed within four months. This case demonstrates the presentation of MCL with pathologic splenic rupture, as well as the potentially highly malignant behaviour of the disease.

Recurrent non-Hodgkin's lymphoma presenting as pathological fracture of the patella

We report a case of recurrent non-Hodgkin's lymphoma presenting as anterior knee pain due to pathological fracture of the patella. Anterior knee pains is an extremely common presenting complaint at orthopaedic clinics and the patella is an infrequent site both for primary and recurrent tumours. This case, treated by patellectomy, illustrates the difficulty of treatment of these lesions and highlights the importance of adequate radiological assessment in sites of previous tumour involvement.

Systemic non-Hodgkin's lymphoma presenting as a serpiginous choroidopathy: Report of a case and review of the literature

Intraocular lymphoma is a rare tumour, the two main types being primary central nervous system non-Hodgkin's lymphoma (CNS-NHL) and, less commonly, systemic lymphoma that has spread to involve the eye. We present a case of a systemic NHL resulting in a serpiginous choroidopathy. To our knowledge, this presentation has not previously been reported. The diagnosis of this case was made based on the temporal relationship between the clinical activity of the choroidopathy and its response to chemotherapy. Diagnosis of intraocular lymphoma on vitreous biopsy is notoriously difficult, and this case is presented along with a review of the literature regarding vitreous biopsy and diagnostic techniques.

Non-Hodgkin's lymphoma presenting with spinal cord compression; a clinicopathological review of 25 cases

The aim of this study was to retrospectively examine 25 patients with newly diagnosed non-Hodgkin's lymphoma (NHL) presenting with spinal cord or cauda equina compression as the first symptom that were referred to our department between 1985 and 1996. At presentation 17 patients were non-ambulatory; dual sphincter impairment was found in 9 patients with a further 8 patients having bladder dysfunction only. All patients had a tissue diagnosis. Five low-grade and 20 intermediate or high-grade tumours were identified. In this latter group 4 patients were treated palliatively and the remaining 16 patients received combination chemotherapy and/or radical radiation therapy. The overall survival at 5 years is 59%. The majority of patients became ambulatory, even if paretic at presentation. This is in marked contrast to reports of patients presenting in this fashion due to metastatic carcinoma. We urge this diagnosis be considered in all patients presenting with spinal cord compression attributed to malignancy.

Two Cases of Orbital Non-Hodgkin's Lymphoma Presenting with Hemophagocytic Syndrome

Two Cases of Orbital Non-Hodgkin's Lymphoma Presenting with Hemophagocytic Syndrome

Non-Hodgkin's Lymphoma Presenting as Anasarca: Probably Mediated by Tumor Necrosis Factor Alpha (TNF-α)

Two patients presented with anasarca, fevers and sweats. Subsequent evaluation revealed aggressive lymphoproliferative disease. Both patients were treated with CHOP chemotherapy. One patient responded with spontaneous, vigorous diuresis and complete resolution of the edema. She relapsed two months later with recurrent edema that responded a second time to salvage chemotherapy. The second patient died of gram positive sepsis a week after diagnosis. As anasarca is an unusual presenting symptom of non-Hodgkin's lymphoma, we postulated that the malignant cells were secreting a cytokine that resulted in “vascular leakage” of fluid and development of diffuse edema. Several serum cytokine levels were tested. Both patients had elevated TNF-α levels, which could have been the cause of the edema; or there might be yet another unidentified mediator that was responsible for the anasarca. We report these two cases to bring to attention the unusual nature of this presentation.

Nodal T Cell Blast Crisis in Chronic Myeloid Leukemia

We report the case of a 54 year old male with an original diagnosis of chronic myeloid leukemia (CML) who developed a nodal T cell blast crisis (BC) while he was in a complete hematological remission (CR). We describe the clinical presentation and the histological, immunophenotypic and molecular characterization of the lymph node blast cells. Our case, together with other rare similar reports in the literature, argue that a T cell nodal blast crisis of CML resembles the presentation of a T-cell non-Hodgkin's lymphoma.

Testicular lymphoma is associated with a high incidence of extranodal recurrence.

Testicular lymphoma is a rare extranodal presentation of non-Hodgkin lymphoma. The authors report long term follow-up information regarding a group of patients with testicular lymphoma evaluated at the Mayo Clinic and describe the outcome with special attention to patterns of recurrence. The medical records of patients with testicular lymphoma seen at the Mayo Clinic between January 1970 and March 1993 were reviewed. Patients were included if they had evidence of testicular involvement at the time of diagnosis of lymphoma. Pathology specimens were reviewed for confirmation of diagnosis. Sixty-two patients with a diagnosis of testicular lymphoma were identified. Their median age was 68 years, and 60 patients underwent orchiectomy as the initial therapeutic and diagnostic procedure. Most of patients (79%) had localized or regional disease at the time of presentation. Other treatment modalities after diagnosis included radiotherapy (37%), combination chemotherapy (37%), and combination chemotherapy and radiotherapy (16%). Although 88% of patients had no residual disease after primary treatment, 80% subsequently experienced disease recurrence. There was no significant difference in the rate of recurrence, including Ann Arbor Stage I disease. Treatment did not appear to affect the recurrence rate. At a median follow-up of 2.7 years, 60% of patients had died of disease. Late recurrences were observed, and there appeared to be no plateau in the disease free survival curve. In half (51%) of the patients with disease recurrence, only extranodal locations were involved. Thirteen patients experienced recurrence in the central nervous system, 11 of whom had parenchymal lesions. In 8 of these 13 patients, the central nervous system was an isolated site of disease recurrence. Testicular lymphoma is a unique and aggressive extranodal non-Hodgkin lymphoma. Better treatment strategies are needed to prevent recurrences. The risk of extranodal recurrence is high, especially in the central nervous system.

Uncommon presentations of non-Hodgkin's lymphoma

In this issue of Hospital Medicine, a case of a primary adrenal lymphoma (PAL) and a primary cutaneous B cell lymphoma (PCBCL) are presented (p. 759). These extranodal lymphomas are uncommon presentations of B cell non-Hodgkin's lymphoma.

AIDS-related non-Hodgkin's lymphoma presenting as gynaecomastia

It is well documented that patients who are HIV positive develop lymphomas as a result of their immunosuppression, the most common being high grade B cell non-Hodgkin’s lymphoma,’ development of which constitutes one of the definitions of AIDS.2 The lymphomas are frequently extranodal and in unusual sites including the brain, bone marrow, and the heart.’ Non-Hodgkin’s lymphoma represents 2.5-2.9s of all new AIDS case presentations in Europe.3

Pleural effusion in patients with non-hodgkin's lymphoma

Pleural effusion is reported in up to 20% of patients with non-Hodgkin's lymphoma (NHL), most often at presentation. However, the prognostic implications of such findings are not clear. The majority of the information in the literature is based on minor observational studies or case reports. Therefore, a case-controlled study was performed to verify the clinical significance of pleural effusion in NHL. Seventeen patients with pleural effusion at the time of presentation of NHL were identified. They were categorized by grade of NHL (based on the Working Formulation). Twenty-nine control patients with similar histopathologic characteristics who had Stage III/IV NHL without pleural effusion were matched to these cases by age, time of diagnosis, and treatment. Ten patients with intermediate grade NHL were matched with 23 controls. No statistically significant difference in complete remission or survival rates between these groups was found (P=0.69 and P=0.7, respectively). The remission and survival rates also were similar in the subgroup of patients and controls who were treated with aggressive chemotherapy. Similarly, no difference was found in these parameters between four cases and six matched controls with low grade lymphoma. No matched controls were found for the patients with high grade lymphoma, but these patients had an unfavorable outcome. Fourteen of the 17 studied patients had an exudative type of pleural effusion. Thoracentesis yielded a positive cytologic finding in every case. The presence of pleural effusion at the time of presentation of NHL does not adversely affect complete remission or survival rates.

Pleural effusion in patients with non‐hodgkin's lymphoma

BACKGROUND Pleural effusion is reported in up to 20% of patients with non-Hodgkin's lymphoma (NHL), most often at presentation. However, the prognostic implications of such findings are not clear. The majority of the information in the literature is based on minor observational studies or case reports. Therefore, a case-controlled study was performed to verify the clinical significance of pleural effusion in NHL. METHODS Seventeen patients with pleural effusion at the time of presentation of NHL were identified. They were categorized by grade of NHL (based on the Working Formulation). Twenty-nine control patients with similar histopathologic characteristics who had Stage III/IV NHL without pleural effusion were matched to these cases by age, time of diagnosis, and treatment. RESULTS Ten patients with intermediate grade NHL were matched with 23 controls. No statistically significant difference in complete remission or survival rates between these groups was found (P = 0.69 and P = 0.7, respectively). The remission and survival rates also were similar in the subgroup of patients and controls who were treated with aggressive chemotherapy. Similarly, no difference was found in these parameters between four cases and six matched controls with low grade lymphoma. No matched controls were found for the patients with high grade lymphoma, but these patients had an unfavorable outcome. Fourteen of the 17 studied patients had an exudative type of pleural effusion. Thoracentesis yielded a positive cytologic finding in every case. CONCLUSIONS The presence of pleural effusion at the time of presentation of NHL does not adversely affect complete remission or survival rates. Cancer 1998;83:1607-1611. © 1998 American Cancer Society.

Non-hodgkin’s lymphoma presenting nine years after ileal-pouch ileoanal anastomosis for ulcerative colitis

Non-hodgkin's lymphoma presenting nine years after ileal-pouch ileoanal anastomosis for ulcerative colitis

Non-hodgkin's lymphoma presenting nine years after ileal-pouch ileoanal anastomosis for ulcerative colitis

Winter, Annette M MD1, *; Koeppen, Hartmut MD2; Hanauer, Stephen B MD1 Author Information