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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical features of 34 patients with Burkitt's lymphoma diagnosed at the American University Medical Center (AUMC) are described. Ages ranged between 3 and 20 years (median, 7 years). Seventy-three percent of the patients were younger than 8 years. Three cases occurred among siblings. The primary site of disease at presentation was the abdomen, 23 patients; jaw, 6; jaw and abdomen, 2; Waldeyer's ring, 2; and mediastinum, 1. Of those who had abdominal disease, the involvement was diffuse and extensive in abdomen and pelvis in 9, apparently confined to the ileocecal region in 5, mesenteric nodes and small intestine in 5, large intestine in 1, and ovary in 3. One patient presented with paraplegia. The bone marrow was studied in 19 patients; it was positive in 5 and suspicious in 2. None had frank leukemia. CSF was studied in 4 patients at presentation and was negative. Eight patients developed meningeal lymphoma during the course of the disease. Liver involvement was documented in 3 patients. Peripheral lymphadenopathy was observed at presentation in 11 patients (9, neck; 2, inguinal + axillary). In contrast to African Burkitt's, the majority of our patients presented with abdominal disease, and in contrast to the American form, our patients were younger with a median age similar to that of African Burkitt's. Thirty percent of the patients had jaw tumor at presentation--a figure intermediate between the African and the American Burkitt's.
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PMID:Burkitt's lymphoma in the Middle East. A study of 34 cases. 404 76

Several studies using sensitive in situ hybridization techniques show that, in non-immunocompromised patients, Epstein-Barr virus (EBV) is more frequently detected in lymphomas expressing T cell markers than in B cell lymphomas. Among lymphomas expressing T cell markers, the presence of EBV is highly related to the site of origin of the tumor, being found in nearly all sinonasal lymphomas, in only a proportion of Waldeyer's ring, lung, gastrointestinal and nodal lymphomas, and undetectable in most primary cutaneous lymphomas. The role of EBV in their pathogenesis can be suggested in at least a proportion of extranodal lymphomas (nasal, lung, Waldeyer's ring, gastrointestinal) with T cell markers in which EBV genome is found in most if not all tumor cells (EBV-associated lymphomas) and the transforming LMP-1 protein is frequently expressed. Among these, sinonasal lymphomas constitute a distinct clinicopathologic entity which may present as lethal midline granuloma, are strongly associated with EBV and can be regarded in most cases as true NK cell lymphomas.
Leukemia 1996 Jun
PMID:Epstein-Barr virus in T and natural killer (NK) cell non-Hodgkin's lymphomas. 864 60

Adult T-cell leukemia/lymphoma (ATL) usually presents with node- and/or cutaneous-based diseases, with a leukemic picture. It is not clear whether ATL initially manifests with lesions in Waldeyer's ring (WR) in an ATL-endemic area. In the present study, we examined distributions of immunophenotypes and the presence of human T-cell leukemia virus type 1 (HTLV-1) and Epstein-Barr virus (EBV) genomes in 62 cases of non-Hodgkin's lymphoma (NHL) of WR (NHL-WR) from two areas of Japan: Osaka, an ATL-nonendemic area (25 cases), and Okinawa, an ATL-endemic area (37 cases). In both areas, age ranged from 10 to 94 years (median, 64 yr), with a male-to-female ratio of 1.81:1. Twelve patients were in Stage I, 34 in Stage II, and 6 in Stage III; stage was unknown in 10. The 25 Osaka cases were B-cell type (16 diffuse immunoblastic type (DIB), 8 diffuse large cell predominantly noncleaved cell (DLNC), 1 cleaved cell (DLC)). Of the 37 Okinawa cases, 20 were B-cell type (3 DIB, 10 DLNC, 2 DLC, 5 other). The remaining 17 cases showed T-cell phenotype (4 DIB, 3 DLC, 3 diffuse large cell not otherwise specified, 7 other). There was a significant difference in the distribution of immunophenotypes between these two areas (P < .001). Combined studies of polymerase chain reaction (PCR) and in situ hybridization revealed the presence of EBV genome in 1 (4%) of 24 Osaka cases, 2 (13%) of 16 B-cell NHLs from Okinawa, and 3 (23%) of 13 T-cell NHLs from Okinawa. HTLV-1 proviral genome was found in 11 (85%) of 13 T-cell lymphomas from Okinawa but could not be detected in B-cell lymphomas from Osaka and Okinawa. Combined clinical, histologic, serologic, and PCR findings suggest that NHL-WR in an ATL-endemic area could be an initial manifestation of ATL.
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PMID:Non-Hodgkin's lymphoma of Waldeyer's ring as a manifestation of lymphoproliferative diseases associated with human T-cell leukemia virus type 1 in southwestern Japan. 931 Sep 58

A case of adenotonsillar enlargement from chronic lymphatic leukaemia leading to severe obstructive sleep apnoea (OSA) is described. Nasal continuous positive airway pressure (nCPAP) therapy was poorly tolerated because even at high pressures (+15 cm H2O) obstructive hypopnoeas persisted. Tonsillectomy resulted in significant improvement, though OSA persisted. nCPAP was reintroduced and at low pressures (+6 cm H2O) was efficacious in alleviating the patient's symptoms. This report highlights the need to consider OSA as a cause of constitutional symptoms in adults with lymphoreticular disease, especially where there is involvement of Waldeyer's ring.
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PMID:Chronic lymphatic leukaemia presenting as severe obstructive sleep apnoea. 944 Nov 19

Mantle-cell lymphoma (MCL) is a B-cell malignancy with distinct molecular genetics and pathological features. Peripheral blood involvement has been reported with variable frequency, but information on the natural history of cases presenting with leukemia is lacking. This study aimed to determine the clinical and prognostic features of such cases. We studied clinical features, tumor characteristics, prognostic factors and outcome in 58 patients with leukemic presentation of MCL. Diagnosis was based on morphology, immunophenotype, presence of t(11;14), histology and cyclin D1 expression. The median age was 62 years and male:female 2.4:1. Presenting features included splenomegaly (74%), lymphadenopathy (45%), hepatomegaly (17%) and, in a minority, gastro-intestinal involvement or involvement of Waldeyer's ring; 10% had lymphocytosis alone. Six patients developed central nervous system disease. Median lymphocyte count was 58 x 10(9)/l, 55% had anemia and 17% had thrombocytopenia. Morphology of peripheral blood showed small-cell MCL in 15% of cases, typical MCL in 46% and blastoid MCL in 39%. Immunological markers showed a typical phenotype (CD5+ CD23 -) in 68%, and atypical phenotypes, CD5- CD23- in 17% or CD5+ CD23+ in 15%. CLL scores were 0, 1 or 2 in 96%. Median overall survival was 36 months. Good response to first-line treatment (P = 0.0008) and splenomegaly (P = 0.03) were favorable prognostic factors, while other features including morphology and CD38 expression had no impact on survival or treatment response. This analysis demonstrates that except for splenomegaly, survival of MCL patients presenting with leukemia is not significantly influenced by clinical or tumor characteristics. Splenectomy is a useful treatment option in this group of patients.
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PMID:The leukemic presentation of mantle-cell lymphoma: disease features and prognostic factors in 58 patients. 1537 Feb 45

We sought to determine the frequency of primary extranodal lymphoma (ENL) and its characteristics in Kanagawa, a human T-cell leukemia virus type 1 (HTLV-1) nonendemic area in Japan. Subjects were 847 newly diagnosed patients with malignant lymphoma at the Yokohama City University Hospital and 8 affiliated hospitals mainly located in Kanagawa prefecture from 1999 to 2005. We compared the clinicopathological characteristics of primary ENL with primary nodal lymphoma (NL). Histological specimens were evaluated according to the World Health Organization classifications. A total of 395 (46.6%) and 452 (53.4%) patients had primary ENL and primary NL, respectively. The frequency of primary ENL increased with age. Primary extranodal sites included the gastrointestinal tract (30.4%), Waldeyer's ring (17.8%), orbits (7.0%), soft tissue and subcutaneous tissue (5.2%), bone (4.6%), skin (4.3%), thyroid gland (4.3%), testis and prostate (3.3%), bone marrow (3.3%), nasal and paranasal cavities (2.6%), salivary glands (2.3%), lung and pleura (2.0%), breast (1.8%), central nervous system (1.0%), uterus and ovary (0.5%), and others (9.8%). Among the 395 cases of primary ENL, diffuse large B-cell lymphoma (61.2%) was most frequently diagnosed, followed by extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (13.3%) and follicular lymphoma (5.6%). The frequency of primary ENL is approximately 50% of the total lymphoma cases in Kanagawa, an HTLV-1 nonendemic area in Japan. This frequency appears to be higher than that in Western countries.
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PMID:Features of primary extranodal lymphoma in Kanagawa, a human T-cell leukemia virus type 1 nonendemic area in Japan. 1856 29