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Background and Objectives: Strategies to improve the efficiency of bronchoalveolar lavage (BAL) are needed. We conducted a study to establish the diagnostic value of BAL in patients with hematological malignancies and pulmonary infiltrates., Patients and Methods: The correlation of cytologic and flow cytometric study of BAL with the microbiological findings and the clinical evolution was determined., Results: Seventy BAL were performed and flow cytometric study was analyzed in 23 of them. Fifty-three patients did not present any adverse event attributable to BAL. Anti-infectious therapy was modified in 64 (91%) patients. T lymphocyte count >0.3×10 9 /l in peripheral blood was associated with longer OS at 3 years (53 vs. 22%, p=.009). Higher CD4 (>20/μL) and CD8 (>35/μL) lymphocyte counts in the BAL were associated with a longer OS at 3 years: 82 vs. 21% (p=.030) and 80 vs. 23% (p=.059)., Conclusions: Our study confirms the clinical value of BAL for treatment decision making in patients with hematological malignancies and acute respiratory failure., (Copyright © 2016 Elsevier España, S.L.U. All rights reserved.)

Background and Objectives: Tyrosine kinase inhibitors (TKI) have improved the management of patients with chronic myeloid leukemia (CML). However, a significant proportion of patients do not achieve the optimal response or are resistant to TKI. ABL kinase domain mutations have been extensively implicated in the pathogenesis of TKI resistance. Treatment with second-generation TKI has produced high rates of hematologic and cytogenetic responses in mutated ABL patients. The aim of this study was to determine the type and frequency of ABL mutations in patients who were resistant to imatinib or had lost the response, and to analyze the effect of second-generation TKI on their outcome., Patients and Methods: The presence of ABL mutations in 45 CML patients resistant to imatinib was evaluated by direct sequencing and was correlated with the results of the cytogenetic study (performed in 39 cases). The outcome of these patients after therapy with nilotinib or dasatinib was analyzed., Results: ABL mutations were detected in 14 out of 45 resistant patients. Patients with clonal cytogenetic evolution tended to develop mutations more frequently than those without clonal evolution. Nine out of the 15 patients with ABL mutation responded to a treatment switch to nilotinib (n=4), dasatinib (n=2), interferon (n=1) or hematopoietic stem cell transplantation (n=2)., Conclusion: The frequency of ABL mutations in CML patients resistant to imatinib is high and is more frequent among those with clonal cytogenetic evolution. The change to second-generation TKI can overcome imatinib resistance in most of the mutated patients., (Copyright © 2012 Elsevier España, S.L. All rights reserved.)

Background and Objective: The growing use of antineoplastic treatments has led to an increase in the incidence of therapy-related leukemias (TRL). The objective was to describe the characteristics of TRL., Patients and Methods: Twenty-three cases of TRL were registered. Chemotherapeutic agents used for the first tumor, time interval, clinical and biological characteristics, treatment and prognosis of the TRL were analyzed., Results: Median age was 61 years. Cytotoxic agents used in previous neoplasm consisted of alkylating agents (17 patients), inhibitors of DNA topoisomerase II (14), antitubulin agents (12), radiotherapy (9, in 6 with radiotherapy) and antimetabolites (6). Median time from diagnosis of the first neoplasm to TRL was 3 years (range 1.2-15.8). Thirteen patients received intensive chemotherapy [with stem cell transplantation (SCT) in 3] and the other 10 received symptomatic treatment (median survival 3 years versus 0.079 years, P=0.004)., Conclusions: In this study, TRL were associated with exposure to alkylating agents, antitubulin agents and topoisomerase II inhibitors. Response to treatment and prognosis were poor, although chemotherapy and SCT may prolong survival., (Copyright © 2010 Elsevier España, S.L. All rights reserved.)

Background and Objectives: Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare entity, presenting especially in adult smoker women. It is characterized by an increase of serum IgM, DR7-HLA haplotype, cytogenetic abnormalities and multiple IgH/BCL-2 rearrangements. To date, it has not been elucidated whether this is a benign or premalignant disorder. We analyzed the PPBL characteristics with especial attention to its evolution., Patients and Methods: Thirty-five PPBL patients from 5 hospitals in Catalonia were retrospectively analyzed. A simultaneous morphologic review of the blood smears was performed by members of the GCCH in a 16 multiple-observer optic microscope. Clinical and biological data were also analyzed., Results: PPBL presents in the majority of cases with persistent polyclonal B-cell lymphocytosis and affects primarily smoker women. The morphologic hallmark, in absence of viral infections, is the presence of activated lymphocytes with bilobulated and/or cleaved nuclei, and nuclear pockets in the ultrastructural study. Increased serum IgM, HLA-DR7 haplotype, chromosomal abnormalities such as i(3)(q10) and multiple IgH/BCL-2 rearrangements were detected. Thirty-four out of 35 patients are alive after a median follow up of 70.7 months. One patient died because of lung adenocarcinoma and another developed a follicular lymphoma without relation to PPBL., Conclusions: PPBL has an asymptomatic and stable evolution, although it frequently presents genetic abnormalities. It remains unknown whether it is a premalignant entity, similar to monoclonal gammopathies of unknown significance. Hence, accurate cytologic diagnosis and follow-up are essential., (Copyright © 2010 Elsevier España, S.L. All rights reserved.)

Background and Objective: The splenic function of patients followed by the Department of General and Digestive Surgery in the Hospital Universitari Germans Trias i Pujol (HUGTiP) from 1985 to 2003 for different degrees of splenic trauma according to the classification of the American Association for the Surgery of Trauma (AAST) 1994 was quantified and related to the treatment received (non surgical, total splenectomy with or without splenosis and splenectomy plus autotransplantation) to detect splenic dysfunction predisposing the development of postsplenectomy sepsis (PSS)., Patients and Method: 43 patients underwent an isotopic study with dynamic splenic gammagraphy and pitted erythrocytes (Normarsky optics) and submembranous vacuoles (transmission electron microscopy) were evaluated., Results: The non surgical group presented normal phagocytic and filtration function with the median speed of splenic enhancement being 3.46 Kcts/s2 (interval: 0.8-6.98). The percentage of pitted erythrocytes was 2% (0-8.8), the number of pits per erythrocyte was 0.03 (0-0.12) and the percentage of erythrocytes with 1, 2, 3 and 4 pits was 1.6%, 0.4%, 0% and 0%, respectively. The percentage of red cells with submembranous vacuoles was 2.55% (0-5.6), the number of vacuoles per red cell was 0.03 (0-0.06) and the percentage of red cells with 1, 2, 3 and 4 vacuoles was 2%, 0.2%, 0% and 0%, respectively. In the operated group, the splenic enhancement speed was 0.08 Kcts/s2 (0-1.75) (p < 0.0001). The percentage of pitted erythrocytes was 38% (0.2-64) (p < 0.0001), the number of pits per erythrocyte was 0.86 (0-1.8) (p < 0.0001) and the percentage of erythrocytes with 1,2,3 and 4 pits was 16.39%, 7.2%, 3.59% and 2.52%, respectively (p < 0.0001). The percentage of red cells with submembranous vacuoles was 11.2% (1.8-31.9) (p = 0.0006); the number of vacuoles per cell was 0.16 (p = 0.0022) and the percentage of red cells with 1, 2, 3 and 4 vacuoles was 6.51%, 1.73%, 0.4% and 0.2%, respectively (p = 0.0246, 0.0010, < 0.0001 and 0.0002, respectively)., Conclusions: Splenic function of patients with a history of splenic trauma receiving conservative treatment is normal, independently of the degree of the lesion, thereby reinforcing the use of this therapeutic approach to avoid the development of postsplenectomy sepsis. In the patients treated with splenectomy, with or without splenosis, splenic function was absent or very altered being partially conserved in cases treated with splenectomy plus autotransplantation.

Background and Objective: Although indications of stem cell transplantation (SCT) are increasing, a transplant may not be performed in all planned cases for several reasons. Our objective was to investigate the reasons for which SCT was not performed in patients referred to a transplant unit., Patients and Method: Pretransplant data of 129 patients consecutively referred to a transplant unit between December 1999 and November 2002 were collected. Frequency and causes of non transplantation were analyzed. Transplanted and non transplanted patient's characteristics were compared., Results: In 119 out of 129 patients, an autologous SCT was indicated and in 10 of them an allogeneic SCT was planned. Mean (SD) age was 46 (14) years (range, 13-69) and 69 (53.5%) were males. One hundred eighteen patients had malignant hematological diseases and 11 had solid tumours. Sixty-one patients showed complete response and 68 had a partial response. At the time of the analysis, 93 SCT had been performed in 90 (69.8%) patients. Autologous SCT was performed in 81 patients (two SCT in one patient) and allogeneic SCT in 10 (two in one patient). Two (1.5%) patients were still awaiting SCT. An SCT was not performed in 37 (28.7%) patients. Causes of non transplantation included: in 12 (32.5%) cases, relapse and/or progression at the time the SCT had been planned; in 12 (32.5%), delay or change in the therapeutic decision; in 9 (24%), poor mobilization; and in 4 (11%), patient's refusal. When delay in SCT was excluded from the analysis, the frequency of no transplant was 19.4%. In 21 (57%) patients of the non-SCT group, peripheral stem cells were previously collected by apheresis. Both groups (SCT and non-SCT) were comparable regarding patients' and disease characteristics except for a more advanced age in the non-SCT group (51 [12] vs 44 [14] years, p<0.005) and status of the disease at the pretransplant visit., Conclusions: The frequency of not performing an SCT in patients referred to a SCT unit was considerable. Relapses and disease progression and poor mobilization were the main causes for it. Delay was also a relatively common cause.

Background: Our purpose was to evaluate the results of the promotion of blood donation in patients undergoing stem cell transplantation (SCT) and to compare it with this promotion in remaining in-patients., Patients and Methods: The hematologist and the blood bank staff informed the patient undergoing SCT about the need for transfusions. Donors were selected for blood or platelet donation. In remaining in-patients, promotion was performed by the blood bank staff by interviewing patients., Results: Between January 2000 and May 2001, 48 SCT patients were included: 41 (85%) yielded 453 possible donors. Of them, 306 donated blood, 77 platelets and 82 were excluded. The number of donations was 484 (389 whole blood and 105 platelets), and 25% (n = 98) of patients donated blood subsequently. Among in-patients 1,950 interviews were carried out: 533 (27.3%) yielded 901 donors (p < 0.001). Overall, 1,146 blood donations were obtained and 15% (135) of these donors donated blood subsequently (p < 0.001)., Conclusion: Collaboration of the hematologist in the promotion of blood donation leads to an increase in the rate and frequency of donations.

Background: To analyse the etiology, diagnostic methods and response to therapy in 30 episodes of pneumonia diagnosed in 17 patients with chronic lymphocytic leukemia (CLL) between 1995 and 2000., Patients and Method: In each episode of pneumonia the following data were analysed: age, gender, treatment of CLL, antiinfectious prophylaxis, granulocytopenia, CD4/CD8 lymphocytes ratio, hipogammaglobulinemia, origin of pneumonia (nosocomial or community-acquired), localisation, respiratory insufficiency, need for mechanical ventilation, antimicrobial therapy and response. Diagnostic methods included blood and sputum cultures, fiberoptic bronchoscopy and search for antigens in urine (Legionella pneumophila serogroup 1, galactomannan, and Streptococcus pneumoniae)., Results: Median age of the series was 60 yr. (range 50-86) and 12 patients were male. Chlorambucil and prednisone were used in 13 cases and fludarabine in 8. Granulocytopenia was present in 14 episodes, hypogammaglobulinemia was seen in 22 and CD4/CD8 ratio was lower than 1 in 8 out of 14 evaluable cases. Etiology of pneumonia was established in 16 episodes (53%). Fiberoptic bronchoscopy was the most useful technique (83% of positive diagnoses) followed by blood cultures (38%). Two patients were diagnosed of aspergillosis at autopsy. Pneumococcus was the most frequent agent (5 cases) followed by Pseudomonas aeruginosa (4), Pneumocystis carinii (2) and Aspergillus fumigatus (2). One out of the two patients with P. carinii pneumonia had received fludarabin and the remaining was treated with prednisone for long time. Ten patients (30%) had died: P. aeruginosa (3 cases), P. carinii (2), A. fumigatus (2), Mycobacterium xenopi (1), and unknown microorganism (2)., Conclusions: In this series of CLL patients the frequency of etiologic diagnosis of pneumonias was good. Pneumococcus was the most frequent microorganism. Pneumonias caused by opportunistic microorganisms were associated to the treatment with fludarabin or prednisone and were associated to a high mortality rate.

Background: In spite of not being considered as an AIDS defining illness, Hodgkin's disease (HD) has specific clinical and biological features in HIV-infected patients., Patients and Methods: Study of clinicopathologic and analytic characteristics, Epstein-Barr virus (EBV) detection (polymerase chain reaction), and prognosis in 15 patients with HD and HIV infection., Results: Thirteen patients had B symptoms, 10 extranodal involvement and 12 advanced HD. The most frequent histologic subtypes were mixed cellularity (6) and lymphocyte depletion (6). The mean (SD) of CD4 lymphocytes was 0.10 (0.08) x 10(9)/l. The presence of EBV in lymph node biopsy was demonstrated in 3 out of 4 patients investigated. Complete remission (CR) was achieved in 7 out of 14 treated cases (50%), the median overall survival was 26 months and the 2 year event-free survival probability was 60%., Conclusions: In HIV-infected patients, HD presents in advanced stages, unfavourable histologic subtypes, frequent extranodal involvement and B symptoms. The prognosis is poor, mainly because of a low CR rate.

Background: To analyze the usefulness of bone marrow (BM) study in the diagnosis of fever of unknown origin (FUO) in patients infected by the human immunodeficiency virus (HIV) in a single center during a period of 42 months., Patients and Methods: 182 episodes of FUO in 162 patients p3th HIV infection were studied. Age, sex, risk factor for HIV infection, hemoglobin level, counts of leucocytes, neutrophils, lymphocytes, CD4 positive lymphocytes, platelets and levels of hepatic enzymes, albumin and beta 2-microglobulin were studied. BM aspirate was performed in all episodes for cytologic and microbiologic examination, and BM biopsy was done in 43. Analysis of factors related with the probability of diagnosis by BM examination was carried out., Results: The median age was 33 years (range, 22-70), and 123 were males. Drug abuse was the most frequent risk factor for HIV infection (63%). One hundred thirty patients had previous AIDS diagnosis before they were evaluated for unexplained fever. A specific diagnosis was achieved in 161 episodes (88%) and the most frequent diagnoses were Mycobacterium spp. (55%) and Leishmania spp. (14%) infections. Fifty-four episodes (30%) were diagnosed by BM examination, and in 36 (20%) BM study was the only diagnostic tool. Examination of the BM aspirate yielded the diagnosis in 40 out of the 178 episodes (13%), whereas BM biopsy was a diagnostic tool in 8 (19%); in 9 additional cases (21%) granulomas were observed. Microbiologic study of BM smears for mycobacterial infections was positive in 28 of the 143 episodes (19%), and the culture for Leishmania was positive in 2 out of the 42 cases. The presence of thrombocytopenia (< 75 x 10(9)/l) and elevated serum levels of aspartate-aminotransferase (AST) (> 100 U/l) were the factors associated with a high probability to obtain the diagnosis through BM study., Conclusions: In patients infected by the HIV and unexplained fever, BM examination is an useful procedure for the diagnosis, particularly in areas where infections by Mycobacterium spp. and Leishmania are prevalent. So that, in our setting, systematic use of this procedure is justified for diagnosis of FUO in those patients.

Background: Abnormalities of the long arm of chromosome 3 (3q) involving bands 3q21 and/or 3q26 occur in 2-6% of myeloid malignancies. Trilineage myelodysplasia, especially in the megakaryocytic line, is a characteristic feature. Additional abnormalities of chromosomes 5 and 7 are usually present. The response to treatment and prognosis are poor., Patients and Methods: The main clinical, cytologic (bone marrow aspirate and biopsy) and cytogenetic characteristics as well as the response to the treatment in 10 patients with 3q abnormalities diagnosed in a single hospital in a period of 8 years are referred., Results: Eight patients had acute non-lymphoblastic leukemia, being de novo in five of them. The median value of hemoglobin was 87 g/l (range: 51-148), white blood cells count 5.8 x 10(9)/l (1.2-47.1) and platelet count 34 x 10(9)/l (5-182). The morphological findings in the study of the bone marrow were: dyserythropoietic features (7 patients), dysgranulopoietic abnormalities (5 patients) and small-sized megakaryocytes with hypolobulated nuclei (8 patients). Fibrosis was observed in the 4 cases in which a bone marrow biopsy was performed. In addition to the 3q alteration, abnormalities of chromosomes 7 (4 patients), 5q (2 patients) and +8 (2 patients) were present. Four patients received intensive chemotherapy and in two of them a complete remission was achieved, but relapse occurred at 3 and 5 months, respectively. All patients have died, the median survival being 7 months., Conclusions: 3q abnormalities define a subtype of myeloid malignancies with characteristic clinical and morphological features. The response to therapy and survival are poor.

Background: To analyze the etiology, treatment response and prognostic factors in 88 episodes of pneumonia in patients with hematological malignancies diagnosed in one center during a period of 30 months., Patients and Methods: 88 episodes of pneumonia in 69 adult patients were studied. Age, sex, hematological malignancy and its treatment, existence of neutropenia and hypogammaglobulinemia, administration of immunosuppressive agents, type (hospital or community-acquired) and localization of pneumonia, presence of respiratory failure and treatment response were reported. For etiologic diagnosis of pneumonias, cultures of blood, sputum, pleural fluid and bronchoalveolar lavage (BAL), as well as Legionella pneumophila, Aspergillus fumigatus and Streptococcus pneumoniae antigen detection in urine were performed. Univariate and multivariate analyses of prognostic factors for pneumonia evolution were carried out., Results: The median age was 63 years. Acute leukemias (AL) were the predominant hematological malignancies. Microbiologic documentation was obtained in 40 (45%) of pneumonias. Fiberoptic bronchoscopy with BAL (71%) was the diagnostic procedure with highest yield followed by blood cultures (25%). Streptococcus pneumoniae (13) was the most frequent isolated pathogen, followed by Legionella pneumophila (6) and Pseudomonas aeruginosa (6). A significantly higher prevalence of Streptococcus pneumoniae was observed in community-acquired pneumonia. The overall mortality rate was 20%. Respiratory failure (p = 0.0009), existence of neutropenia (p = 0.0023), age equal or higher than 60 years (p = 0.012) and prolonged administration of immunosuppressive agents (p = 0.015) were the prognostic factors associated with unfavourable evolution of pneumonias in the multivariate analysis., Conclusions: The etiologic diagnosis of pneumonia in patients with hematological malignancies was only achieved in a half of cases. In our series, the high prevalence of Legionella pneumophila can be attributed to the special epidemiologic characteristics of our hospital. Prognostic factors of pneumonia are related to individual factors as well as to the hematological status of patients.

Background: To analyze the main clinical and biological data and the response to therapy in 15 patients with primary gastrointestinal lymphoma (PGIL) from a series of 76 patients with HIV related non-Hodgkin's lymphoma (NHL) diagnosed in a single institution in a 13 years period., Patients and Methods: The main clinical, biological and evolutive data were recorded. Pathologic diagnosis of PGIL was made according to the REAL classification. Clinical stage was determined by the Ann Arbor system modified by Rohatiner et al. Response to therapy as well as overall survival (OS) were studied. Results were compared with non-PGIL HIV-related NHL patients., Results: Mean age of the series was 38 years. Thirteen patients were male, and 8 intravenous drug abusers. Then had bad performance status (ECOG 2-4) and 11 B symptoms. All patients had a high grade malignant PGIL and the localization was gastric in 10 cases. The most frequent symptoms were abdominal pain (11 cases), gastrointestinal bleeding (4) and dysphagia (3). Ten patients had advanced stages (IIE2-IV). The median CD4 cell count was lower in PGIL patients (92 x 10(6)/l vs 148 x 10(6)/l; p < 0.05). Thirteen patients received intensive chemotherapy with CHOP regimen (in 5 surgical procedures were previously made). Complete response (CR) was obtained in 4 patients (31%) and 1 of them relapsed. Median OS was 10 months vs 16 months non-PGIL HIV-related lymphoma patients (p < 0.05)., Conclusions: PGIL in HIV patients often presented advanced stages and high grade of malignancy. The most common localization is the stomach, and these patients usually have bad performance status and a low CD4 lymphocyte count. Response to therapy is poor. In our series OS was worse in PGIL patients than in the rest of HIV-related NHL, possibly due to the high degree of immunosuppression in the formers.

Background: To analyze the response to eradicative therapy and prognostic factors in 52 patients with primary gastrointestinal lymphoma (PGIL) diagnosed at a single institution in a 13 year period., Patients and Methods: The main clinical, biological and evolutive data were recorded. Pathologic diagnosis of PGIL was made according to the Working Formulation. Clinical stage was determined by the Ann Arbor system modified by Mushoff. The results of therapy as well as the influence of such characteristics on complete remission (CR), disease-free survival (DFS) and overall survival (OS) were studied., Results: Mean age of the series was 53 years (SD 15). Thirty patients were males. HIV infection preceded PGIL diagnosis in 10 cases. Seventeen had bad performance status (ECOG 2-4) and 30 B symptoms. The PGIL localization was gastric in 31 cases and 29 had a low grade malignant lymphoma. B phenotype was demonstrated in 98% and 22 patients presented advanced stages (IIE2-IV). Treatment was radical surgery followed by intensive chemotherapy in 32 cases, intensive chemotherapy alone in 17, and surgical resection in 3. CR was obtained in 34 patients and 6 of them relapsed. The projected DFS from CR at 9 years was 72% and OS was 26%. CR and survival were not influenced by PGIL localization and treatment type. The main unfavourable prognostic factors were advanced stage (CR and OS), B symptoms (DFS and OS) and advanced ECOG score (CR, DFS and OS). Previous HIV infection had an independent prognostic influence on both CR and OS., Conclusions: In patients with PGIL, the achievement of CR, DFS and survival have been independent of the type of eradicative treatment used. Performance status, B symptoms and clinical stage have been the main prognostic factors. HIV infection carried an independent prognostic significance.

Spinal cord compression is an infrequent event in patients with non-Hodgkin's lymphomas (NHL) and early diagnosis and therapy are required. The main clinical and histologic characteristics as well as the response to therapy in 10 NHL patients with spinal cord compression diagnosed at a single center in a 7-year period are referred. The main initial clinical manifestations were pain in dorsal or lumbar regions (5 cases) and paraparesia or paraplegia (5 patients). Infection by human immunodeficiency virus (HIV) was present in four cases. In 8 patients spinal cord compression was the initial manifestation of NHL. Pathologic diagnosis showed intermediate or high-grade lymphoma in 8 out of 10 cases and immunologic phenotype was B-cell in all cases. Laminectomy followed by radiation therapy and chemotherapy was performed in 4 cases, radiotherapy and chemotherapy in two, isolated chemotherapy in 3 and radiation therapy was administered to the remaining case. Complete response was observed in 4 cases and 6 cases were resistant to treatment. No patient with HIV infection responded to treatment. Six patients had died, median survival time for the whole series being 4 months, and the actuarial probability of survival was 40% at the first year.

The purpose of this study has been to refer the main clinico-biologic characteristics, the evolution and the response to therapy in 6 patients with acute myelomonocytic leukemia with eosinophilia and inversion of chromosome 16 (AML4Eo inv[16]) belonging to a series of 92 patients with acute myeloblastic leukemia diagnosed in a single hospital between 1987 and 1995. The main clinical manifestations were anemic syndrome and hemorrhage. Anemia and thrombocytopenia were present in all cases, high white blood cell count in 4, monocytosis in 5 and eosinophilia in one. Bone marrow aspirate showed myeloid and monocytic blast infiltration (43-62%), eosinophilia (5-19%) and atypical monocytic precursors (6-18%). Induction therapy consisted in one or two cycles of daunorubicin (or idarubicin), cytosine arabinoside and etoposide, followed by two cycles of consolidation treatment, the first with mitoxanthrone and cytosine arabinoside and the second with amsacrine and cytosine arabinoside. One patient died in the induction phase, while complete remission was obtained in the remaining 5. One patient died during the consolidation therapy. Allogeneic bone marrow transplant (BMT) was performed to one patient and autologous BMT to another. The first patient remains in complete remission (CR) at 68 months from diagnosis, and the second relapsed 12 months after BMT. Another patient relapsed at 13 months from diagnosis and the remaining persists in CR 13 months from diagnosis. Actuarial probabilities of CR duration and survival were 50% at 5 years. The clinico-biologic characteristics and the response to therapy of patients with AML4Eo inv(16) are similar to those referred in other series. There is a high probability of CR attainment and, probably, the relapse rate is lower than that of other subtypes of AML.

Background: The aim of this study was to analyze the influence of age on the clinical and biological features as well as the results of treatment in 41 adult patients with acute lymphoblastic leukemia (ALL)., Patients and Methods: The patients diagnosed with ALL from January 1989 to October 1995 in a single center were studied. Two groups of patients were analyzed based on age. The main clinical, hematologic and biochemical parameters, morphologic subtype and immunologic phenotype and the results of the cytogenetic study were analyzed. Likewise, the attainment of complete remission (CR), its duration and overall survival (OS) were also studied. Comparison of the cited variables and the results of treatment among the two groups of patients was performed., Results: Group I was made of 19 (11 males, 8 females) patients > or = 50 years of age (mean age 65 +/- 9 years). Group II included 22 patients (11 males and 11 females) with a mean age of 28 +/- 11 years. Significant differences were only observed between the two groups in regard to the proportion of peripheral blood blasts (p < 0.02), serum LDH values (p = 0.05) and the performance status at the time of diagnosis ( p < 0.00007). In the patients in group 1 cytogenetic alterations were more frequent (10/16 vs 4/20, p < 0.02), being mainly pseudodiploid. Complete remission was achieved in 7/16 patients in group I and in 17/22 in group II (p < 0.02). The median duration of CR was 34 and 18 months, respectively. The median OS was 7 months in group 1 and 15 months in group II with an estimated survival at 5 years of 0% in group I and 38% in group II (p < 0.05)., Conclusions: Patients with acute lymphoblastic leukemia over the age of 50 years have a worse general status and more cytogenetic alterations (particularly structural) than younger adult patients, presenting a lower probability of achieving complete remission and a shorter survival.

Although cardiac infiltration is common in advanced stage of acute leukaemia, it is not usually diagnosed at life and it is extremely rare for it to become pseudotumoral. A 25-years-old patient with an acute monoblastic leukaemia who had a leukaemic infiltration which affected the main part of the left ventricle at the time of diagnosis, is referred. The heart infiltration was detected by a two dimension echocardiography. In spite of a massive infiltration, heart failure was not present and the left ventricle's ejection fraction was 50%. Even though chemotherapy was administered, the patient died four days after diagnosis due to septic shock of respiratory origin. The most relevant autopsy finding was a widespread pseudotumoral infiltration of the left ventricle, the back side of the right ventricle and the interventricular wall. The pseudotumoral infiltration of the heart by acute leukaemia is uncommon and must be differentiated from granulocytic sarcoma. The usefulness of the different diagnostic procedures is discussed.

Lymphomas of the marginal spleen zone are an entity recently considered as separate by the International Lymphoma Study Group. There are B-cell non Hodgkin's lymphomas (NHL) of low grade malignancy with a characteristic phenotype that allows to differentiate from mantle lymphomas and other B-cell lymphoproliferative syndromes. The case of a 69-year-old female patient admitted for abdominal pain due to large splenomegaly is reported. Pancytopenia and the presence of atypical large-sized lymphocytes with extensive cytoplasm and a rounded nucleus with indentations, reticulated appearing chromatin and one or several nucleoli were of note in the hemogram. Microscopic examination of the bone marrow demonstrated moderate-degree lymphocytary infiltration with grade I reticulin fibrosis. Laparotomy with splenectomy was performed. White pulp invasion with multifocal infiltration of the red pulp by lymphocytes of the same characteristics as those observed in the peripheral blood and bone marrow were observed on microscopic bone marrow examination. Immunophenotypic study of these lymphocytes was positive for CD19, CD20 and CD22 while being negative for CD5, CD10, CD23, CD25, CD11c and FMC7, the phenotype belonging to the lymphocytes of marginal spleen zone. Following splenectomy the patient recovered hemoperipheral counts and did not undergo additional treatment. The patient died due to septic shock of respiratory origin 4 months later. The clinical, morphologic and immunophenotypic features of marginal spleen zone lymphomas are reported with emphasis on the differences with other B-cell non Hodgkin's lymphomas of low malignancy.

Granulocytic sarcoma (GS) is a solid tumor of extramedullary localization constituted by immature precursors from the granulocytic series. GS may be diagnosed in different malignant blood diseases involving the granulocytic series, acute non lymphoblastic leukemia (ANLL) being the most frequent, followed by myelodysplastic syndromes (MDS) and chronic myeloproliferative syndromes, specially chronic myeloid leukemia (CML) in blastic crisis. Although the diagnosis of GS is suspected with conventional cytologic and anatomopathologic studies, histochemical staining and immunohistochemical techniques are often required for definitive diagnosis. Five cases (4 males, 1 female; age range 22-77 years) diagnosed with GS in one center over a period of nine years (1984-1993) are described. The GS were located in the lymph nodes, the jaw, paravertebral region, gallbladder and retroperitoneum, respectively. Two patients had refractory anemia with excess of blasts (RAEB). Three patients had ANLL; in one GS constituted the form of relapse, in another GS presented at the time of diagnosis and in the remaining patient GS preceded the diagnosis of ANLL. All the patients died from 2 to 8 months after diagnosis of GS with no response to treatment being observed. Immunohistochemical study of the tumor was performed in 4 patients, being positive for lysozyme and the monocytic MAC-387 monoclonal antibody. Immunocytochemical study of the tumor blasts was carried out with positivity for CD15 being observed. Although uncommon, GS should be suspected in patients with ANLL or MDS with tumors of any localization and at any time during its evolution. Immunocytochemical and immunohistochemical studies are of great value to differentiate GS from other tumors, particularly anaplastic non Hodgkin's lymphomas.

Background: The aim of the present study was to determine the prognostic factors for treatment response, relapse-free survival (RFS) and overall survival (OS) in a series of 73 patients with primary extranodal lymphomas (PEL)., Methods: All the patients diagnosed with PEL in one center from January 1984 to December 1993 were studied. The Working Formulation was used for histologic classification and the stage was determined according to the Ann Arbor classification modified by Mushoff. Age, sex, localization of the lymphoma, the presence of B symptoms, the existence of bulky disease, human immunodeficiency virus (HIV) infection, the main hematological and biochemical parameters, the histologic grade of malignancy, the immunologic phenotype and stage were reported. Univariate and multivariate analyses of prognostic factors for complete response (CR), RFS and OS were carried out., Results: The most frequent localization of PEL was the gastrointestinal tract (43 cases). Fourteen cases had HIV infection. CR was obtained in 37 out of the 55 patients (67%) of whom 8 (21%) had relapsed. Serum LDH level was the main prognostic factor for CR attainment (p = 0.01). The variables which negatively affected RFS were the presence of HIV infection (p = 0.02), advanced age (p = 0.018) and the existence of B symptoms (p = 0.02). OS was shorter in patients with high LDH levels (p = 0.004) and more aggressive histologic subtype (p = 0.0002)., Conclusions: The prognostic factors observed in the patients with primary extranodal lymphomas are the same as those found in nodal non Hodgkin's lymphomas. The stage is not a factor of bad prognosis if chemotherapy is included in the treatment.

Background: The digestive tract constitutes the most frequent localization of the extranodular non Hodgkin's lymphoma. The chief clinical and histological characteristics were analyzed as were the evolution and response to treatment of 25 patients diagnosed with primary gastrointestinal lymphoma (PGIL) in one center over a period of eight years., Methods: To establish the diagnosis of PGIL the Dawson criteria were used and the state was determined by the Ann-Arbor classification modified by Musshoff. To histologically classify of the PGIL the Working Formulation was followed. The influence of the clinical, histological characteristics and the state of the obtention of complete remission (CR), the survival free period of the disease (SFD) and global survival (GS) were analyzed., Results: The mean age of the series was 56 years (standard deviation 15 years) (12 males). The most frequent localization was gastric followed by the small intestine and the large intestine. Abdominal pain and weight loss were the most frequent clinical manifestations in the PGIL, regardless of its site. In 12 patients the PGIL was of an intermediate grade of malignancy, 8 were of low grade and 5 of high grade. The state was IE in 11 patients, IE1 in 9 and IIE2 in 5. The most used treatment was radical surgery followed by polychemotherapy. Complete remission was obtained in 15 patients and 2 recurred. The foreseen SFP at 7 years was 69% of the cases and GS was 53%. In IE state patients was most frequently obtained and GS was also more prolonged in these patients., Conclusions: In the present series the gastric localization of patients with primary gastrointestinal lymphoma was more frequent than intestinal localization. The HNL of intermediate and high grades of malignancy predominated those of low grade. The prognosis of patients with primary gastrointestinal lymphoma in a IE state was more favorable than the remaining patients. In turn, surgery constituted a good option in the initial treatment of patients with primary gastrointestinal lymphoma.

Three patients with renal adenocarcinoma and one with metastatic malignant melanoma were treated with continuous intravenous infusion of 18 x 10(6) IU/m2/day interleukin-2 during 5 days per week (2 weeks). Overall 60% of the calculated dose was administered owing to the development of severe toxicity. Among the hematological effects, eosinophilia was found in all patients, which was more marked 15-20 days after therapy was started. In addition, 15% of atypical lymphocytes were found in peripheral blood. These cells, denominated Pinocchio cells, show a cytoplasmatic prolongation with azurophilic granulation. Their cytochemical study disclosed a marked positivity for acid phosphatase and alpha-naphthyl acetate esterase, and a variable positivity for dipeptidyl-amino peptidase (DAP IV). The immunophenotype revealed that is a T lineage cell population, basically CD 3+, with small or absent positivity for the monoclonal CD 19 antibody. The presence of Pinocchio cells, which are effector cells mediating tumor destruction by an apoptosis mechanism, is related to the administered dose of interleukin-2.