Long-term outcome of peripheral T-cell lymphomas: Ten-year follow-up of the International Prospective T-cell Project.

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of haematological cancers with generally poor clinical outcomes. However, a subset of patients experience durable disease control, and little is known regarding long-term outcomes. The International T-cell Lymphoma Project (ITCLP) is the largest prospectively collected cohort of patients with PTCLs, providing insight into clinical outcomes at academic medical centres globally. We performed a long-term outcome analysis on patients from the ITCLP with available 10-year follow-up data (n = 735). The overall response rate to first-line therapy was 68%, while 5- and 10-year overall survival estimates were 49% and 40% respectively. Most deaths occurred prior to 5 years, and for patients alive at 5 years, the chance of surviving to 10 years was 84%. However, lymphoma remained the leading cause of death in the 5- to 10-year period (67%). Low-risk International Prognostic Index and Prognostic Index for T-cell lymphoma scores both identified patients with improved survival, while in multivariate analysis, age >60 years and Eastern Cooperative Oncology Group performance status 2-4 were associated with inferior outcomes. The favourable survival seen in patients achieving durable initial disease control emphasizes the unmet need for optimal front-line therapeutic approaches in PTCLs.

Evaluation of CD7 and terminal deoxynucleotidyl transferase (TdT) expression in CD34+ myeloblasts from patients with myelodysplastic syndrome.

There is an emerging use of flow cytometry to evaluate patients with myelodysplastic syndrome (MDS). We have studied CD7 and TdT expression in the CD34+ myeloid blast cell population in 55 bone marrow samples of patients with MDS. CD7 and/or TdT were detected in 38 out of 55 patients (69%). CD7 expression was not related to other bad prognosis data but conversely, we found an association between TdT+ CD34 myeloblasts and high-risk MDS patients according to the International Prognostic Scoring System. Therefore, CD7 and TdT may help to establish the diagnosis of MDS and, TdT expression also seems to be a useful marker in distinguishing risk groups.

[Molecular analysis in 13 cases of chronic Philadelphia-positive myeloid leukemia]. / Análisis molecular en 13 casos de leucemia mieloide crónica Ph positivo.

Thirteen cases of Ph-positive CML have been studied from a cytogenetic and molecular point of view. All cases had Ph chromosome identified as t(9;22). Three of these cases were in blast crisis, and additional alterations to the Ph chromosome were found. The molecular study was made using the 3'bcr probe to analyze the ability to detect the translocation at the molecular level in bone marrow, peripheral blood, other tissues and in cases with mosaicism. Likewise we have evaluated the possibility to obtain molecular results from Carnoy-fixed samples which have been previously used in cytogenetic studies. In all cases we have detected rearrangement in the bcr region; this shows the interest and usefulness of this new methodology in the study of CML in different situations.

[Subleukemic acute monophagocytic leukemia. A biological phenotype close to malignant histiocytosis]. / Leucemia aguda monofagocítica subleucémica. Un fenotipo biológico próximo a la histiocitosis maligna.

An 80 year-old woman presented subleukaemic acute monoblastic leukaemia (AML-M5a). Her bone marrow showed invasion by highly dysplastic histio-monocytic cells of great size and wide cytoplasm, with intense phagocytic activity (erythrophagocytosis was frequently seen), and with abnormal karyotype (50XX, +8, +8, +16, +21). The different malignant and reactive features of the mononuclear phagocytic system are commented, along with the haemophagocytic activity of the histio-monocytic cells in different states. The cytogenetic anomalies more frequently found in AML-M5 are also dealt with as compared to this patient's. The case reported here seems to correspond to subleukaemic acute "monophagocytic" leukaemia, with a biologic phenotype close to that of malignant histiocytosis.

[Iron deficiency dyserythropoiesis in patients with chronic gastrointestinal and gynecologic hemorrhages. Study of 39 cases]. / Diseritropoyesis ferropénica en pacientes con hemorragias crónicas gastrointestinales y ginecológicas. Estudio de 39 casos.

A defect of haemoglobin synthesis is the classically recognized mechanism affecting the erythron functionalism in chronic iron deficiency. The poor erythroblastic bone-marrow response, plus a number of dyserythropoietic nuclear features, have led to think of an impairment of the cell cycle of erythroblasts in iron-lack anaemia. The aim of the present work was to study such hypothesis, not proven so far. Ten subjects with normal haemopoiesis and 39 patients with iron-lack anaemia of different aetiologies (namely, 19 with digestive tract bleeding, 16 with gynaecological bleeding, and 4 with haemorrhages on other locations) were included in a previously reported protocol. The scheme of such protocol consisted of: 1) bone-marrow erythroblast quantification; 2) analysis of their maturation gradient; 3) erythroblast mitotic index; 4) measure of the mitotic time in bone-marrow culture; 5) tritiated-thymidine incorporation to short-term bone-marrow culture and quantification of the erythroblastic labelling index. To these were added the degree of nuclear dyserythropoiesis according to Hill and Lewis, and the reticulocyte production index. The following mean values were found in the control group: erythroblasts, 25.5 (+/- 3.63) %; E1-E4, 47.66 (+/- 3.09) %; IDN, 0.67 (+/- 0.27); MI, 2.82 (+/- 0.66) %; MT, 1.05 (+/- 0.15 hr); LI, 34.88 (+/- 5.82) %. The mean values found in iron-lack anaemias were as follows: erythroblasts 39.42 (+/- 9.1) %; E1-E4, 42.25 (+/- 4.11) %; IDN, 7.77 (+/- 4.69); MT, 1.81 (+/- 0.95) hr; LI, 13.08 (+/- 6.51) %. The statistical analysis (Student's t) showed highly significant differences (p less than 0.001) in the increased IDN and decreased MI and LI in iron deficiency patients.(ABSTRACT TRUNCATED AT 250 WORDS)

[Essential thrombocythemia: a myeloproliferative state on the rise. Clinico-biological study and course of 44 cases]. / La trombocitemia esencial: un cuadro mieloproliferativo "en alza". Estudio clínico-biológico y evolutivo de 44 casos.

Forty-four cases of essential thrombocytosis (ET) were diagnosed in the last 20 years, 19 males and 24 females (M/F: 0.76), aged between 3 and 86 years (median, 62 years), and 9 of them being under 40 years of age. The M/F ratio for patients under 60 years was 0.5, whereas it was 1.09 for patients over 60. The clinical forms at onset were: asymptomatic, 36.5%; as a bleeding disorder (BD), 20.4%; as thrombotic disease (TD) 22.7%; BD/TD, 13.6%, and others, 6.8%. The most important biological features included platelet count over 1.000 x 10(9)/L (59.1%), abnormal platelet aggregation, chiefly with ADR (56.5), mild reticulin myelofibrosis (55%), abnormal karyotype (2.6%), moderately high LDH levels (56.8%) and pseudo-hyperkalaemia (40%). The initial therapeutic approach was: observation (12 cases), antiaggregating agents (6 cases), and chemotherapy (BSF, HU, etc.) in the remainders. One patient evolved quickly into acute myelogenous leukaemia and two others suffered a late transformation into polycythaemia vera (PV) and myeloid metaplasia, respectively. The median survival was over 11 years, this being longer in patients under 60 years of age, in those with platelet count at diagnosis between 600 and 1000 x 10(9)/L and in those without initial symptoms of thrombosis. The advent of electronic blood-cell counters has made ET no longer a rare chronic myeloproliferative disease, its incidence coming now closer to that of PV; thus, in the last four quinquennial periods the incidence of ET/PV has evolved as following: 1/19, 4/16, 13/18 and 26/29.

[Myelodysplastic syndromes. Hematologic phenotypes, cytogenetic expression and clinical course in 133 cases (1979-1989)]. / Síndromes mielodisplásticos. Fenotipos hematológicos, expresión citogenética y patrones evolutivos de 133 casos (1979-1989).

One hundred and thirty-three cases of myelodysplastic syndromes studied during the last ten years were revised. Of them, 79 were males and 54 females, and their ages ranged between 15 and 91 years (median, 69 years). Five patients (3.7%) had secondary myelodysplasias. The haematological phenotype (FAB) of the cases was: RA, 41.3%; SRA, 24%; RAEB, 18%; RAEBT, 3.7%; CMML, 8.3%. Leucopenia/thrombocytopenia without initial anaemia was present in 4.5% of the cases. Abnormal karyotype was found in 54 patients (40.6%), MIKA in 41 cases and MAKA in 13 cases. The cytogenetic anomalies most commonly found were +8, 5q-, -7, 11q- and 13q-. Cytogenetic abnormalities were commonest amongst the RAEB (50%), and least frequent in CMML (18.2%). Thirty-one patients evolved into acute leukaemia (29 ANLL and 2 ALL). Such blastic changes were more frequent in RAEB (62.5%) and rarest in SRA (9.4%), and they appeared mostly in patients with complex karyotype (MAKA) (53.8%) as compared with those who had normal karyotype (17.7%). Short-lasting complete remission was achieved by 40% of the patients treated with conventional chemotherapy. The survival of the group as a whole (median 30 months) varied in accordance with the haematological phenotype: SRA, 81 months; RA, 65 months; CMML, 13 months; RAEB +/- T, 8 months. The finding of a MAKA karyotype significantly shortened the survival (4 months) with regard to MIKA (44 months) or normal karyotype (39 months). The following median survivals were attained after patients' staging (Bournemouth's criteria): stage A, 84 months; stage B, 22 months, and stage C, 5 months.(ABSTRACT TRUNCATED AT 250 WORDS)

[Acute promyelocytic leukemias: clinico-biological aspects, prognostic factors, therapeutic response, and possibilities of cure in 34 cases (1970-1988)]. / Leucemias agudas promielocíticas: aspectos clínico-biológicos, factores pronósticos, respuesta terapéutica y posibilidades de curación de 34 casos (1970-1988).

Thirty-four new cases of acute promyelocytic leukaemia (M3) were diagnosed at the authors' Centre between 1970 and 1988 (19 males and 15 females) with ages between 5 and 73 years (median age, 32 years). Three cases were of the hypogranular variant or M3-v (8.8%). The clinical picture included: haemorrhagic diathesis (85%), pallor/malaise (82%), fever/infection (41%), hepatomegaly (26%), splenomegaly (12%). Leucopenia of less than 5 x 10(9)/L was present in 23/34 cases, laboratory signs of DIC in 26/31, increased LDH, over 400 U/mL, in 6/31, and abnormal karyotype in 7/15. One of the patients rejected any treatment; two others died of brain haemorrhage before therapy was started, and seven died in the first two weeks of treatment. Of the 31 patients treated, complete remission (CR) was achieved in 21 cases (67.7%). Allogeneic BMT was carried out in two of them, with further relapse and death. Post-remission treatment was given to the remaining 19 patients, and there were 13 relapses. Six patients have been in CR, 5 of them after cessation of therapy, for the last 1.5-11.5 years. Age under 50 years and leucocyte count below 5 x 10(9)/L at diagnosis were favourable prognostic factors according to the univariate statistical analysis performed. The survival plateau of the actuarial curve was reached beyond 2.75 years by 15% of all the patients treated (33 cases), 23% of the patients who achieved CR (21 cases), 31% of the patients under 50 years of age and 5 x 10(9)/L leucocyte count at diagnosis (15 cases) and 36% of these last achieving CR (13 cases).