Oligoblastic leukaemia with (8;21) translocation and haemophagocytic syndrome and granulocytic cannibalism.

We report a 47-year-old man with oligoblastic leukaemia (8;21) translocation, phenomenon of cannibalism by granulocytic cells and haemophagocytic syndrome. The patient responded to intensive chemotherapy with disappearance of haemophagocytosis, granulocytic and histiocytic. We conclude that: (1) granulocytic cannibalism and haemophagocytic syndrome can be unusual myelodysplastic features; (2) the oligoblastic leukaemia with presence of cytogenetic abnormalities related to AML in young patients are probably more close to acute leukaemia than to myelodysplastic syndrome.

Autologous stem cell transplantation (ASCT) with immunologically purged progenitor cells in patients with advanced stage follicular lymphoma after early partial or complete remission: toxicity, follow-up of minimal residual disease and survival.

The role of autologous stem cell transplant (ASCT) in indolent lymphomas is a controversial issue. From 1994 to 1999, we performed ASCT with immunologically purged progenitor cells in 15 patients with advanced stage follicular lymphoma (FL) after early partial or complete remission. Results of the purging strategy and follow-up of minimal residual disease after transplant were analyzed with PCR amplification of bcl-2/IgH rearrangement for the t(14;18) translocation. A comparison of transplanted patients with a group of controls was carried out to evaluate differences in progression-free survival and overall survival. Eighty percent of patients received one chemotherapy regimen before ASCT and were in first remission. All the patients received cyclophosphamide plus hyperfractionated total body irradiation as the conditioning regimen. Nine patients were transplanted with bone marrow (BM) and six with peripheral blood progenitor cells (PBPC). Engraftment was delayed in one patient transplanted with BM. Two patients died during the transplant procedure. Ten of 12 evaluable patients were PCR positive in the BM for bcl-2 rearrangement at diagnosis. Six of them (60%) were still positive after chemotherapy, and one patient was transplanted with a positive hematopoietic product after purging. With a median follow-up of 27 months, six of eight evaluable patients still remain PCR negative in the BM. With a median follow-up of 4.7 years from diagnosis, progression-free survival was 83% (95% CI: 63-100). The risk of disease progression of non-transplanted patients was 19.2 times higher than that of transplanted patients (P = 0.01), but no differences were found in overall survival. Regarding patients in first remission, the risk of relapse was 12.6 times higher in non-transplanted than in transplanted patients (P = 0.04). This procedure seems to offer a good chance to achieve a PCR-negative state and prolonged freedom from recurrence. According to these results, prospective randomized trials are warranted.

[Peripheral T-cell lymphomas without primary skin involvement. An anatomicoclinical study of 29 consecutive cases]. / Linfomas T periféricos sin afectación cutánea primaria. Estudio anatomoclínico de 29 casos consecutivos.

BACKGROUND: To analyze peripheral non-cutaneous T-cell lymphomas (NC-TCL) and to test the significance of the IPI. PATIENTS AND METHODS: Twenty-nine consecutive patients diagnosed with NC-TCL according to the REAL classification. RESULTS: At diagnosis were common: B symptoms (21%), stage III/IV (55%), high LDH level (55%) and extranodal involvement (79%). Twelve (48%) patients achieved complete remission. The IPI identified groups of patients with different response to therapy and survival. CONCLUSIONS: NC-TCL with high or high-intermediate IPI had a short survival if treated with CHOP.

Distribution and incidence rates of lymphoid neoplasms according to the REAL classification in a single institution. A prospective study of 940 cases.

Recently, a new classification system for lymphoid neoplasms, known as the REAL classification, has been proposed. Our aim is to know the distribution of lymphoid neoplasms according to this schema and compare it with the Updated Kiel classification. We also estimate incidence rates of lymphoid neoplasms in our area. From January 1993 to November 1996, 940 patients were diagnosed of lymphoid neoplasm in our center. Histologic material was prospectively classified according to both the REAL and the Updated Kiel classifications. According to the REAL classification, distribution of all cases of lymphoid neoplasms was as follows: 73.6% B-cell neoplasm, 9.4% T-cell neoplasms, 9.6% Hodgkin's disease and 7.4% unclassifiable. Considering only non-Hodgkin's lymphomas (NHL), 87.2% of cases could be categorized according to the REAL and 77.7% with the Updated Kiel classification. These figures differed due to unrecognized categories in the Kiel schema. Annual incidence rate per 100,000 inhabitants was 20.1 for lymphoid neoplasms, and NHL alone was 9.0. In conclusion, the REAL classification allowed us to categorize more cases of NHL than did the Updated Kiel classification, fundamentally because of the inclusion of some recently described entities.

Diffuse large B-cell lymphoma: is morphologic subdivision useful in clinical management?

The diffuse large B-cell lymphoma category of the REAL classification encompasses different morphologic lymphoma subtypes in a single entity. The aim of this study is to determine the influence of the morphologic subdivision within this category with respect to clinical features and response to treatment. From January 1993 to October 1996, 132 patients were diagnosed de novo with diffuse large B-cell lymphoma in our institution. All cases were classified according to the REAL and the Updated Kiel classifications, and immunohistochemical study was performed in all of them. Sixty-three per cent of patients received chemotherapy with a curative approach. Of the 105 assessable patients, 80 cases (74%) were classified as centroblastic (CB) and 25 cases (26%) as immunoblastic (IB), according to the updated Kiel classification. These 2 subsets of lymphomas did not differ with respect to major clinical features and laboratory parameters. Both groups had a similar complete response rate with a uniform therapeutic approach and the overall 2-yr survival did not show statistical differences (49% in CB vs. 45% in IB). In conclusion, for clinicians, morphologic subdivision of the diffuse large B-cell lymphoma category into CB and IB subtypes has little clinical and prognostic significance.

Post-transplant lymphomas: a 20-year epidemiologic, clinical and pathologic study in a single center.

BACKGROUND AND OBJECTIVES: To study the incidence, clinical presentation, pathologic features and outcome of post-transplant lymphomas (PTL) during the past 20 years. DESIGN AND METHODS: We undertook a descriptive study of all biopsy-proven cases of PTL diagnosed in our hospital from 1979 through 1999. The average annual incidence rate of PTL was analyzed at 5-year intervals from 1979 to 1999. Risk ratios were estimated by comparing the incidence of PTL among transplanted patients with that of lymphoma observed in the general population of the region. Survival analysis was performed at the univariate level using the Kaplan Meier technique and at the multivariate level by Cox hazard models. RESULTS: Seventeen of 1,860 transplanted patients developed a PTL (0.9%). The risk of PTL was calculated to be almost 8-fold higher than the risk of lymphoma in the general population. The risk was highest among those who had received a heart transplant (RR=35.6). The mean time between transplant and the diagnosis of PTL was 31 +/- 29 months. Of all PTL, 88% were of B-cell origin and 53% of the cases tested were Epstein-Barr virus (EBV)-positive. The median survival was 24 months. The majority of patients with allograft involvement died within the 2 months following diagnosis (hazard ratio 5.3; 95% CI 1.4-20.7). INTERPRETATION AND CONCLUSIONS: Organ transplantation is a major risk factor for the development of lymphoma, a disease with a particularly bad prognosis when it develops at the site of the allograft. Early diagnosis and more specific treatment may improve PTL survival.