bluuebunny/crossref_metadata_2025_split huggingface dataset:
Lymphohistiocytosis: A multi‐factorial syndrome of macrophagic activation clinico‐pathological study of 38 cases
Authors: Goldberg J. , Nezelof C. 
Published: February 2007

AbstractLymphohistiocytosis (LH) is defined by a widespread infiltrate of non‐malignant lymphocytes and macrophages, involving principally the liver, spleen and central nervous system and associated with a severe lymphoid atrophyFor this histological study, 38 juvenile cases of LH were selected and reviewed. Morphological and histochemical studies revealed that the macrophages were activated. They appeared to be OKM1, OKT9 and HLADr positive and showed a patent or occult erythro and thrombocytophagocytosis. Comparatively, most of the lymphoid cells did not bear any immunoglobulin and did not label with a wide panel of monoclonal antibodies including T3, T4, T6, T8, T11The review of the clinical and biological data of these 38 cases suggests that LH does not represent a single entity but can be regarded as a non‐specific response to various causes. Three different conditions associated with LH can be isolatedThe first group consists of patients in whom the disease is characterized by an early onset, high familial incidence and an inevitably fatal course. This category contains the largest number of cases (29 out of 38) and corresponds to the classic description of Familial Erythrophagocytic Reticulosis by Farquhar and ClaireauxThe second group includes mainly male patients and children over 2 years old. Inheritance is not a predominant feature. The course of the disease is comparatively long. A viral infection was present in three of the seven cases. This category shares many of the clinico‐pathological features of the Virus Hemophagocytic Syndrome and is presumably related to a chronic and latent viral infectionA third group can be identified on the basis of the presence of pigmentation abnormalities and immunodeficiency disorders probably associated with cytoskeletal abnormalities. This group includes Chediak‐Higashi disease and Griscelli's diseaseThe fact that some genetic factors and immunodeficiency disorders are present in these three groups is probably significant and suggests that LH is a condition brought about by multiple factors—of which the common denominator is an apparent activation of the Mononuclear Phagocytic System.
An investigation of Ki‐1 positive large cell lymphoma with antibodies reactive with tissue macrophages
Authors: Jones D. B. , Gerdes J. , Stein H. , Wright D. H. 
Published: February 2007

AbstractThe diagnosis of true histiocytic lymphoma (THL) represents one of the most difficult and controversial areas of lymph node pathology. Recently, Stein et al. (1985) have demonstrated that a series of tumours presenting with morphological and immunocytochemical features, previously considered as being indicative of THL, share antigenic markers with Reed‐Sternberg and mononuclear Hodgkin's cells and, therefore, may be derived from lymphocytes, rather than histiocytes. In this study we have re‐investigated some of these cases with a panel of monoclonal antibodies variously reactive with macrophages. Although all cases contain a heavy infiltrate of reactive histiocytes the tumour populations are negative for histiocyte antigens.
DNA‐abnormality in hairy cell leukemia
Authors: Arrenbrecht Stefan , Heider Sonia 
Published: February 2007

AbstractSpleen and light density peripheral blood leukocytes of 10 hairy cell leukemia (HCL) patients and total leukocytes of one patient and blood donor cells were stained quantitatively for cellular DNA. The DNA content of single cells was measured by flow cytometry (FC) and compared to the DNA content of sheep cells admixed as an internal controlEight of eleven patients (72 per cent) showed deviations from blood donor DNA content. Two female patients showed increased cellular DNA content, the six male patients had hypodiploid cells. Chromosomal aberrations are therefore likely to exist in the majority of HCL patientsSeparation of HCL cells into sheep erythrocyte rosette and non rosette forming cells revealed similar DNA abnormalities in both cell populations, suggesting that the leukemia encompasses cells with B and with T markers.
Cytosine arabinoside in the management of recurrent leukaemia
Authors: Ganesan T. S. , Barnett M. J. , Amos R. J. , Piall E. M. , Aherne G. W. , Man A. , Lister T. A. 
Published: February 2007

AbstractA Phase II study of high dose cytosine arabinoside (ara‐C) with different schedules in patients with recurrent acute myelogenous leukaemia (AML) and myeloid blast crisis of chronic myeloid leukaemia has been conducted at St. Bartholomew's Hospital. Ara‐C was infused continuously for seven days at a dose of 100–200 mgs/m2 daily from day 1 with 1–2 g/m2 (3 h infusions) twice daily from day 2 for six daysNineteen patients with acute myelogenous leukaemia and four patients with myeloid blast crisis of chronic myeloid leukaemia (CML) were treated. Complete remission was achieved in 4/19 patients with AML and in a further four patients an antileukaemic effect was observed. There were eight early deaths and three patients failed to show any response to therapy. All four patients with myeloid blast crisis of CML failed to respond to the treatmentToxicity was considerable with gastro‐intestinal and hepatotoxicity being the most serious problemsPharmacokinetic studies revealed that mean basal levels achieved with continuous infusion prior to high‐dose ara‐C were 102 ng/ml and peak levels were of the order of 104 ng/ml. The considerable toxicity of the regimen, without clinical advantage over less intensive programmes, resulted in its termination.
Treatment‐related leukemia in Hodgkin's disease: A multi‐institution study on 75 cases
Authors: Brusamolino E. , Papa G. , Valagussa P. , Mandelli F. , Bernasconi C. , Marmont A. , Bonadonna G. , Tura S. , Bosi A. , Mango G. , Mazza P. , Rossi E. , Leoni F. , D'Onofrio G. , D'Arcangelo E. 
Published: February 2007

AbstractHematological and cytogenetic characteristics of 75 cases of therapy‐related acute non lymphoid leukemia (t‐ANLL) occurring in Hodgkin's disease (HD) are analysed in this multi‐institution study. Combined radio and chemotherapy had been given in 88 per cent of patients, either as adjuvant (44 per cent) or as salvage modality (44 per cent). Radiotherapy alone and chemotherapy alone had been given in 3 per cent and 9 per cent respectively. Eighty per cent of patients were in remission of HD and 71 per cent off‐therapy while developing leukemia. The median latent time from remission of HD to leukemia was 34 months. The myeloblastic variety of leukemia accounted for 43 per cent of total cases; the myelomonocytic and monocytic for 17 per cent and 4 per cent, the promyelocytic and erythroblastic variants for 5 per cent and 7 per cent of t‐ANLL. Twenty four per cent of cases were unclassifiable; one of these was TdT‐positive. Dysplastic features of erythrocytic line were invariably present with circulating erythroblasts; defects of granulocytes, circulating megathrombocytes and micromegakaryocytes were also present. Bone marrow hypoplasia and marked fibrosis were documented in 47 per cent and 30 per cent of cases. Preleukemia heralded overt leukemia in 73 per cent of cases; 37 per cent had refractory anemia with no excess of blasts; 16 per cent of preleukemias were unclassifiable. Cytogenetics revealed chromosome abnormalities in 83 per cent of cases; 72 per cent presented chromosome 5 and/or 7 monosomy or partial deletion (5q‐ or 7q‐) of the long arm (94 per cent in the combined modality therapy group). In 3 cases, a pure monosomy 7 was observed; in none 5q‐alone. Response rate to conventional therapy was 14 per cent; low and high‐dose cytarabine were of little benefit. Long‐term CR (28+ and 16+ months) was achieved in 2 cases with allogeneic bone marrow transplantation (BMT) as first‐line therapy. A better knowledge of t‐ANLL in HD and new therapies, including BMT, may improve the prognosis of this late complication of intensive HD treatment.
Gastrointestinal lymphoma in Chinese: A retrospective analysis
Authors: Liang R. , Todd D. , Chan T. K. , Ng R. P. , Ho F. C. S. 
Published: February 2007

AbstractEighty‐four Chinese patients with gastrointestinal lymphoma were reviewed. There were 45 gastric and 39 intestinal lymphomas. The median age was 45 years and the male to female ratio 1.14:1. Diffuse histiocytic (60 per cent) or diffuse large cell (53.5 per cent) lymphoma comprised a majority of the cases. A high incidence of gastrointestinal bleeding at presentation was observed in our patients. According to a modified staging classification, a larger proportion of patients with intestinal lymphoma had advanced disease (Stage III and IV) than those with gastric lymphoma (82 per cent versus 55 per cent, p < 0.02) and gastric lymphoma carried a better prognosis. The modified staging classification used in this study appeared to stratify better the patients into 2 sub‐groups of localized (Stage I and II) and advanced (Stage III and IV) disease, which correlated very well with the prognosis. Good treatment results were obtained following intensive therapy in our patients with localized disease (Stage I and II). The poorer treatment results of patients with advanced disease (Stage III and IV) call for better chemotherapy regimens and earlier diagnosis. Surgical resection of the primary gastrointestinal lesion is recommended to prevent haemorrhage and perforation following chemotherapy, which occurred in 38 per cent of our patients whose gastrointestinal lesions were not resected.
Large cell transformation of subclinical small lymphocytic leukemia/lymphoma: A variant of richter's syndrome
Authors: Strauchen James A. , May Michael M. , Crown John 
Published: February 2007

AbstractThe authors report three cases of diffuse aggressive non‐Hodgkin's lymphomas (large cell, two; mixed small and large cell, one) arising in association with clinically silent small lymphocytic proliferations involving bone marrow, and lymph nodes. Immunologic marker studies suggested origin from a common B cell clone in two cases; in one case the large cell lymphoma lacked detectable surface markers. Some cases of diffuse aggressive lymphomas of adults may arise by large cell transformation of clinically silent small lymphocytic proliferations, a process akin to Richter's syndrome.
Non‐hodgkin's lymphoma of unfavourable histology: A multivariate analysis of factors predicting the response to chop
Authors: Pereira Arturo , Cervantes Francisco , Montserrat Emilio , Llebaria Carlos , Rozman Ciril 
Published: February 2007

AbstractForty‐eight patients withde novonon‐Hodgkin's lymphoma (NHL) of unfavourable histology received CHOP as first‐line chemotherapy. A complete remission (CR) was achieved in 64.5 per cent patients. Overall 4‐year projected survival was 48 per cent with a median follow‐up of 40.5 months. Two pretreatment characteristics, high LDH serum levels and bulky abdominal disease, were negatively associated with survival at the proportional hazards regression model and were used to calculate each patient's relative‐risk. Such analysis allowed to identify two prognostic subgroups according to their outcome to CHOP. Firstly, a high‐risk subgroup that showed an 8 per cent CR rate, most patients dying within the first year after diagnosis. Secondly, a low‐risk subgroup that showed an 83.5 per cent CR rate and a 4‐year projected survival of 66 per cent. From the above results two major conclusions can be drawn: (1) the CHOP combination is an effective treatment for unfavourable NHL patients with a low relative‐risk and (2) new therapeutic approaches should be explored for NHL patients with a high relative‐risk at diagnosis.
VAD chemotherapy—toxicity and efficacy—in patients with multiple myeloma and other lymphoid malignancies
Authors: Anderson H. , Scarffe J. H. , Lambert M. , Smith D. B. , Chan C. C. , Chadwick G. , McMahon A. , Chang J. , Crowther D. , Swindell R. 
Published: February 2007

AbstractThirty‐three patients with multiple myeloma (11 untreated, 15 refractory and seven relapsed patients) have received vincristine and adriamycin infusion therapy with oral dexamethasone (VAD). The median number of courses received was five. In addition 16 patients with lymphoid malignancy have received a median of four courses of VAD.Three patients who relapsed after VAD have received further VAD therapy making 52 patient treatments assessable for toxicity. Ten per cent had nausea, 4 per cent vomiting, 4 per cent total alopecia, 25 per cent constipation, 33 per cent paraesthesiae, 8 per cent proximal myopathy, 33 per cent dyspepsia, 23 per cent proven bacteraemia, and 19 per cent chest infections. Infections were not usually associated with neutropenia. Shingles was seen in four patients with myeloma, but none of the patients with lymphoid malignancy.The response rate in myeloma was 9/11, for previously untreated patients, 3/7 for relapsed, and 8/15 for refractory patients. Responses have been seen in other lymphoid malignancies—1/2 patients with relapsed acute lymphoblastic leukaemia had a complete remission. Two out of seven patients with chronic lymphocytic leukaemia achieved a partial remission, and a further three had a clinical improvement. Three out of six patients with non‐Hodgkin lymphoma and one patient with macroglobulinaemia achieved a partial remission.
Follicular non‐Hodgkin's lymphoma in Hong Kong Chinese: A retrospective analysis
Authors: Liang Raymond , Todd David , Chan T. K. , Ng R. P. , Choy D. , Loke S. L. , Ho F. C. S. 
Published: February 2007

AbstractFollicular lymphoma is relatively rare in Hong Kong Chinese. Fifty‐two patients with follicular lymphoma were reviewed. The histology was nodular lymphocytic poorly differentiated (NLPD) in 75 per cent, nodular mixed (NM) in 21 per cent and nodular histiocytic (NH) in 4 per cent. Our patients appeared to have a higher proportion of NLPD and a lower proportion of NM lymphoma than the western series. Fifty patients were analysed excluding the two patients with NH lymphoma. They had a median age of 50 and a male to female ratio of 0.92. Seventy‐two per cent of them presented with asymptomatic lymph node enlargement. Twenty per cent had B symptoms and 32 per cent bulky tumour. Twelve per cent had stage I disease, 2 per cent stage II, 30 per cent stage III and 56 per cent stage IV. A high incidence of bone marrow involvement (48 per cent of all patients) was found.All seven stage I‐II patients responded to involved‐field radiotherapy alone and none of them has relapsed. The 43 stage III‐IV patients were treated with chemotherapy without deferral and a majority of them received CVP (51.1 per cent) or chlorambucil alone (34.9 per cent). The complete response rate of stage III‐IV patients was 81 per cent and 26 per cent of the complete responders relapsed. The 5‐years disease‐free survival (DFS) and overall survival of all patients (stage III and IV) were 50 per cent and 65 per cent respectively. The DFS curve showed a pattern of continuous relapses. Stage III patients appeared to have a better complete response rate, a lower relapse rate and superior disease‐free survival than stage IV patients but the differences did not reach statistical significance. However, the overall survival of stage III patients was significantly better than stage IV patients (p < 0.02). Other factors including sex, age, presence of bulky tumour, B symptoms, histologic subtypes and the chemotherapeutic regimes did not significantly affect their prognosis.
Molecular analysis of Hodgkin's disease‐derived cell lines
Authors: Tesch Hans , Jücker Manfred , Falk Martin H. , Bornkamm Georg W. , Jones David B. , Diehl Volker 
Published: February 2007

AbstractThree Hodgkin's disease‐derived cell lines were analysed for the organization of immunoglobulin and T cell receptor genes, for the expression of the interleukin 2 (IL‐2) receptor gene, and for the cellular oncogene c‐myc. All three cell lines have characteristic genotypic markers of lymphoid cells and can be classified as immature lymphoid cells with respect to the incomplete expression of their antigen receptor genes. This immature genotype is in contrast to the expression of activation antigens Ki‐1 (CD 30), IL‐2 receptor (CD 25), and HLA‐DR. The results obtained are in agreement with studies obtained from primary Hodgkin's lymphomas, which are activated by as yet unknown mechanisms.
Angiofollicular lymph node hyperplasia associated with myelofibrosis case report and immunologic marker studies
Authors: Bleiweiss Ira J. , Dumitrescu Otilia L. , Jagirdar Jaishree 
Published: February 2007

AbstractA case of angiofollicular lymph node hyperplasia (AFH) (hyaline vascular type) of the mediastinum associated with myelofibrosis is reported with immune marker studies. We hypothesize that the myelofibrosis is more than a mere coincidence since other myeloproliferative disorders have been observed in conjunction with AFH. Immunohistochemistry on cryostat sections of lymph node using the biotin‐avidin fluorescence technique showed the presence of numerous T helper cells and very few T suppressor/cytotoxic cells in the parafollicular areas. Concentric rings of lymphocytes showed the phenotype of mantle zone lymphocytes (i.e. IgM, IgD positivity), while the germinal centres were ill‐formed as shown by Leu 14 (a Pan B cell marker) staining. By electron microscopy, follicular dendritic reticulum cells were readily found. The above findings suggest an abnormality of germinal center formation.
Low field strength magnetic resonance imaging of bone marrow in acute leukaemia
Authors: Richards M. A. , Webb J. A. W. , Malik S. , Jewell S. E. , Amess J. A. L. , Lister T. A. 
Published: February 2007

AbstractThe accuracy of low field strength (0.08 Tesla) magnetic resonance imaging (MRI) of bone marrow for the detection of acute leukaemia in adults has been assessed by comparison with bone marrow biopsy results. Spin lattice relaxation time (T1) measurements from patients were compared with those from 90 volunteers. Eighteen patients were studied at the time of diagnosis of leukaemia. Bone marrow T1 was prolonged in all cases. One of two patients with refractory anaemia with excess of blasts in transformation (RAEBt) had prolonged bone marrow T1, the other had normal T1. T1 at the time of diagnosis for patients with acute leukaemia or RAEBt correlated with the cellularity and blast cell count in the marrow. None of the 17 patients who were studied when in long‐term remission of leukaemia had prolonged marrow T1.Serial studies were undertaken in five of the newly diagnosed patients. An increase in bone marrow T1 was observed in each of four patients studied seven days after the start of treatment, at a time when they showed a decrease in leukaemic cells on peripheral blood examination. T1 measurements made 3 weeks after the commencement of chemotherapy were similar to pretreatment values and did not reflect the reduction in leukaemic infiltration observed on bone marrow needle aspirate. The implications and possible explanations for these findings are discussed.
Intensive chemotherapy for acute non‐lymphoblastic leukemia after primary myelodysplastic syndrome
Authors: Martiat Philippe , Ferrant Augustin , Michaux Jean‐Louis , Sokal Gérard 
Published: February 2007

AbstractTwenty‐five patients with a primary myelodysplastic syndrome (MDS) transformed into acute non‐lymphoblastic leukaemia (ANL) were treated with intensive chemotherapy. A complete remission (CR) was obtained in six patients (24 per cent). In five of these six patients two courses of chemotherapy were needed to achieve CR. In eight patients chemotherapy cleared the bone marrow of blasts, but the aplasia was fatal. A partial effect on bone marrow blasts was seen in four patients and no effect in another six. Eleven patients (44 per cent) died from the consequences of chemotherapy‐induced cytopenia. A short interval between MDS and transformation into ANL was associated with a better chance of achieving complete remission. Age, karyotype, type of MDS, peripheral blood or bone marrow findings had no influence on the result of chemotherapy. The median survival from start of treatment was 5 months (range 0.5–24 months). In the patients who achieved a CR, the median duration of the remission was 7 months (range 3–12 months). The poor response rate, the short duration of the remissions and the high treatment‐related mortality suggest that current intensive anti‐leukemic chemotherapy in ANL after primary MDS is of limited benefit.
Diagnostic features and survival in typical and prolymphocytoid variants of chronic lymphocytic leukemia
Authors: Scott Colin Stephen , Stark Allistair N. , Head Christine , Roberts Bryon E. 
Published: February 2007

AbstractDiagnostic features were evaluated with regards to survival in 203 cases of typical and prolymphocytoid chronic lymphocytic leukemia. Excluding 27 (13 per cent) patients with second malignancies, survival analyses indicated that the prognostic factors with greatest significance were age at presentation (< 65 years and 65 years or more: p = 0.0004), proportions of prolymphocytoid cells (< 10 per cent and 10 per cent or more: p < 0.0001 age corrected) and surface immunoglobulin (SIg) density (weak and more than weak: p = 0.001 age corrected). In contrast, sex, absolute numbers of prolymphocytoid cells, SIg light chain type and FMC7 expression were not prognostically significant. These observations suggest that the recognition and delineation of prolymphocytoid CLL variants by morphological and immunophenotypic criteria, although important in diagnostic terms, may have less relevance with respect to prognosis and patient management.
Angiotropic large cell lymphoma (ALCL): Morphological, immunohistochemical and genotypic studies with analysis of previous reports
Authors: Domizio P. , Hall P. A. , Cotter F. , Amiel S. , Tucker J. , Besser G. M. , Levison D. A. 
Published: February 2007

AbstractThe entity ‘angioendotheliomatosis proliferans systemisata’ was first described 28 years ago as a cutaneous small vessel neoplasm of presumed endothelial origin. Since then, 101 similar cases have been reported under a variety of different names, most with systemic as well as cutaneous lesions, and a lymphoid histogenesis of the tumour cell is now favoured. Review of these cases has shown a characteristic clinical presentation with predominant neurological and dermatological features, although the diagnosis was made at autopsy in 53 per cent of patients. Most therapeutic regimens have proved ineffective with a median survival of 5 months from date of clinical presentation. Aggressive combination chemotherapy can produce complete and lasting remission and a partial response to steroids is sometimes seen. We have examined a case of this condition showing unusual clinical features. Immunohistochemical studies confirm the lymphoid origin of the tumour cells with B cell phenotype. Antigen receptor gene rearrangement studies indicate the presence of the same clonal population of B cells in multiple sites. We suggest that the term ‘angioendotheliomatosis proliferans systemisata’ should be dropped and support the use of ‘angiotropic large cell lymphoma’ to describe this unusual condition.