Síndromes linfoproliferativos crónicos en Chile: estudio prospectivo de 132 casos / Chronic lymphoproliferative disorders in Chile: a prospective study in 132 patients

Background: Chronic lymphoproliferative disorders include a variety of diseases which are often a diagnostic problem for clinical hematologists. Aim: To study prospectively the distribution and incidence of chronic lymphoproliferative disorders in Chile and compare them with those of other Western, Latin American and Oriental countries. Patients and methods : A group of 132 patients were studied in a 36 months period (1999-2001), with a panel of monoclonal antibodies. A score for chronic lymphocytic leukemia was employed to differentiate it from other B-cell disorders. Results : The median age was 63 years old (range 32-94). Most patients had B-cell tumors (109) and the rest (23), T-cell tumors (82 percent vs 18 percent). Forty five percent of patients with B-cell tumors had a chronic lymphocytic leukemia (CLL), while the others were disseminated lymphomas. The incidence of T-cell tumors was slightly higher than that of other Western countries. Noteworthy is that the most common of these disorders was adult T cell leukemia/lymphoma (ATLL), in concordance with the high HTLV-1 seroprevalence in Chile. Conclusions : A morphologic, immunophenotypic and pathological study in a large number of patients with chronic lymphoproliferative disorders in Chile, shows a relatively low incidence of CLL when compared to other chronic B-cell tumors and a high representation of ATLL associated to HTLV-1 infection, compared with other Western countries. The lower incidence of CLL in our study might be due to patient's selection and/or underdiagnosis of this disease as a substantial proportion of CLL are asymptomatic

Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile / Clinical significance and frequency of the 11q23/MLL genetic/molecular alteration in chilean infants with acute leukemia

Background: Acute leukemia (AL) in infants generally shows distinctive biologic features and has a poor prognosis. Aim: To study the frequency of the cytogenetic alteration of11q23 chromosome or the recombination of MLL gene in infants less than 18 months old, with acute leukemia. Patients and methods: We analyzed 37 cases of AL in infants less than 18 months of age diagnosed in Chile from 1989 to 1999. The clinical features and cytogenetic/molecular defects of 11q23MLL gene rearrangement and their influence in prognosis were determined. Results: There were 18 cases of acute Lymphoblastic leukemia (ALL) characterized by female sex (67 per cent) high presenting leukocyte count (median 99 x109/L), blast cells with a CD10 negative phenotype (50 per cent) and 11q23/MLL rearrangement (39 per cent). Molecular abnormalities of 11q23 were significantly associated with adverse prognosis, with an event free survival (EFS) of only 14 ñ 12 per cent. Interestingly, infants with germ line 11q23 had a very good outcome with an EFS of 73 ñ 11 per cent (p<0.025). There were 19 cases of acute myeloblastic leukemia (AML) characterized by male sex (63 per cent) high leukocyte count (median 93 x 109/L), FAB-MS morphology (53 per cent) and 11q23/MLL rearrangement (53 per cent). EFS was very poor, 20 ñ 9 per cent and 33ñ4 per cent for rearranged and germinal group respectively (p=NS), due to a high mortality rate during the first month of diagnosis. Conclusions: These findings demonstrate that Chilean ALL infants with 11q23 abnormalities have a very poor prognosis. However those with germinal state can enjoy a prolonged disease free survival with the current treatment protocols

Leucemia mieloide aguda del adulto: resultado del protocolo nacional de drogas antineoplásica: Hospital del Salvador 1990-1998 / Acute myeloid leukemia in the adult: results of the national antineoplastic drug protocol (Panda) at Hospital del Salvador, Chile

Background: The incidence of acute myeloid leukemia is 3 cases per 100.000 inhabitants/year and its five years event free survival is 15 to 20 percent. Since the incorporation of trans retinoic acid, event free survival of M3 acute myeloid leukemia is 80 percent. Aim: To report the results of acute myeloid leukemia treatment at the Hospital del Salvador, between 1990 and 1998. Patients and methods: The medical records of 117 patients (66 female, mean age 48.2 years), treated between 1990 and 1998 using PANDA protocol, were retrospectively reviewed. Immunophenotyping was done in 69 patients and cytogenetic studies were done in 65. Results: Sixteen percent of patients had M3 acute myeloid leukemia. The most frequent phenotype was the association of DR, CD34 plus a panmyeloid marker. DR and CD34 were negative in seven of nine patients with M3 acute myeloid leukemia. Cariotype was abnormal in 78 percent of patients. Complete remission was achieved in 65 percent of cases with a 13 percent of failures. Early mortality was 21.3 percent and decreased to 6.1 percent in the last three years. Infections and coagulation disorders were the main causes of death. Mean survival was 10.5 months. Five years event free survival was 11 percent. In M3 acute myeloid leukemia, the figure is 50 percent. Conclusions: Treatment results are less effective than protocols that consider more aggressive chemotherapeutic protocols or bone marrow transplantation. The reduction in early mortality is due to a better management of febrile neutropenia

Mejoría en el pronóstico de la leucemia linfoblástica aguda en niños de un país en desarrollo: resultados del protocolo nacional chileno PINDA 87 / Improved outcome for acute lymphoblastic leukemia in children of a developing country: results of the chilean national trial PINDA 87

Un grupo oncológico pediátrico nacional, PINDA, reporta el primer protocolo prospectivo, no randomizado, para tratamiento de la leucemia linfoblástica (LLA), usando una versión modificada del protocolo de Berlín-Frankfurt-Munster (LLA BFM 86). Los objetivos de este estudio fueron clasificar inmunofenotipos, disminuir radioterapia de cráneo y comprobar si este protocolo podía mejorar la sobrevida de nuestros pacientes. Procedimiento: desde junio 1987 a junio 1992 se registraron 444 pacientes, no seleccionados; de ellos 425 fueron evaluables. La terapia fue estratificada según riesgo: riesgo bajo (RB), riesgo alto (RA) y riesgo muy alto (RMA). Los pacientes en RB y RA recibieron inducción con protocolo I, consolidación con protocolo M (RMA usó protocolo E), reinducción con protocolo II y mantención. Todos recibieron tratamiento de prefase con prednisona oral y metotrexato (MTX) intratecal. Radioterapia de cráneo solo en RA y RMA (12-18 Gy). Los siguientes cambios se introdujeron al protocolo LLA BFM 86: en protocolo M 1 g/m² en vez de 5 g/m²; en protocolo E, 1 g/m² de citarabina en vez de 2 g/m², la mitoxantrona e ifosfamida fueron sustituidas por teniposido y ciclofosfamida. Resultados: inmunofenotipo: LLA común 67,4 por ciento, LLA proB 14 por ciento, LLA T 10 por ciento, LLA preB 4,3 por ciento. La frecuencia de sobrevida libre de eventos (SLE) global a 5 años fue 60 por ciento ñ 2 por ciento error standard; según riesgo fue: RB 75 por ciento, RA 62 por ciento, RMA 28 por ciento con una mediana de seguimiento de 6,5 años (rango 4,5 - 9,5 años). La incidencia acumulada de recaída en sistema nervioso central SNC fue 5,4 por ciento. Conclusión: hemos tenido éxito en realizar un estudio a nivel nacional. Nuestra estrategia para adaptar el protocolo BFM fue efectiva para mejorar la SLE. La distribución por fenotipos es similar a otras series

Leucemia linfoma T del adulto en Chile: estudio clínico patológico y molecular de 26 pacientes / Adult T cell leukemia lymphoma in Chile: a clinical-pathological and molecular study of 26 patients

Background: Adult T cell leukemia lymphoma is a lymphoproliferative syndrome etiologically associated to human T cell lymphotropic virus type I. Aim: To describe the clinical and laboratory features of 26 caucasian chilean patients, with HTLV-I positive adult T-cell leukemia lymphoma (ATLL). Material and methods: Diagnostic criteria included clinical features, cell morphology, immunophenotype, HTLV-I serology and/or DNA analysis by southern blot or PCR. Results: According to the clinical presentation, 12 cases had the acute ATLL form, 6 had a lymphoma, 4 the chronic form and 4 had smoldering ATLL. The median presentation age was 50 years, younger than the Japanese patients, but significantly older than patients from other south american countries (eg Brasil, Jamaica, Colombia). The main clinical features: lymphadenopathy, skin lesions and hepatosplenomegaly, were similar in frequency to those of patients from other countries, except for the high incidence of associated neurological disease. Tropical spastic paraparesis (TSP) in our series of ATLL, was seen in one third of the patients (8/26). A T-cell immunophenotype was shown in all 26 cases and HTLV-I serology was positive in 25/26 patients. Molecular analysis on the seronegative patient showed clonal integration of proviral HTLV-I DNA into the lymphocytes DNA, and thus he may have been a poor responder to the retroviral infection. Proviral DNA integration was also demonstrated in 15/16 patients being clonal in 10, polyclonal in 3 (all smoldering cases) and oligoclonal in one. Conclusions: ATLL in Chile has similar clinical and laboratory features than the disease in other parts of the world, except for a younger age than japanese patients but older than those from other latin american countries and a high incidence of patients with associated TSP. Detailed morphological and immunophenotypic analysis of the abnormal circulating lymphocytes, together with the documentation of HTLV-I by serology and/or DNA analysis are key tests for the identification of this disease

Leucemia mieloide aguda MO: características clínicas y de laboratorio / Acute myeloid leukemia MO: clinical and laboratory features

Patients and methods: Eight patients, our of 368, with acute myeloid leukemia that were studied in the Hematology Laboratory of a public hospital in Santiago, were classified as LMA-MO. Results: Blast cell morphology was undifferentiated or of subtype FAB-L2 lymphoblastic leukemia with medium sized blasts, agranular basophilic cytoplasm, reticular nuclear chromatin and a prominent nucleolus. Cytochemical staining was negative for peroxidase and esterases, immunophenotyping showed the expression of one or more myeloid antigens (CD13, CD33) and was negative for lymphoid antigens. Immunocytochemical expression of myeloperoxidase was positive in the three cases where it was performed. Only one patient achieved complete remission and is free of disease after 36 months of follow up. All other patients died without obtaining remission, six shortly after the onser and one 12 months after. Conclusions: The diagnosis of LMA-MO is essential considering its dismal prognosis

Inmunofenotipo: características clínicas y laboratorio de la leucemia linfoblástica aguda en Chile: estudio de 500 niños y 131 adultos / Immunophenotype: clinical and laboratory characteristics of acute lymphoblastic leukemia in Chile: study of 500 children and 131 adults

We describe the clinical features and immunophenotype of 500 children and 131 adults with acute lymphoblastic leukemia (ALL), diagnosed between 1984 and 1993. Cases were classified according to immunophenotype in B-cell ALL accounted for 74 percent of cases and pro-B ALL was more common in children of less than 1 year (14 percent). B ALL was observed in 2 percent of children. Ten percent of children, mostly males, had T-cell ALL. The third part of these children had high leukocyte counts and a mediastinal mass. Children from Mapuche origin, compared with Caucasian had a lower proportion of common ALL (36 and 74 percent respectively) and a higher proportions of T-cell ALL (41 and 10 percent respectively). Among adults common ALL was the most common phenotype (72 percent) followe by T-cell ALL (15 percent), pro-B ALL (11 percent) and B-cell ALL (2 percent). There was a lower incidence of children with common ALL with positive cytoplasmatic immunoglobulin, compared to North American or European studies (2 and 15-33 percent respectively) and a higher proportion of adults with common ALL compared with pro-B cell ALL, in contrast to European studies that show a higher proportion of patients with pro-B cell ALL. No other immunophenotypic, clinical or laboratory differences were observed with ALL. No other immunophenotypic, clinical or laboratory differences were observed with ALL from developed countries. It is concluded that the immunophenotyping of ALL, allows a more precise diagnosis of this disease

Toxocariasis sistémica en el paciente adulto / Systemic toxocariasis in adult patient

We report a retrospective review of 8 patients (one male), aged 19 to 31 years old, with systemic toxocariasis (visceral larva migrans), seen at the Salvador Hospital of Santiago. 4 cases had heart involvement, three had lung involvement and in 2, several organs were affected. Seven cases had leukocytosis (range 14, 5 to 160 x 10 9/1) and all had eosinophilia (35 to 90 percent). Serum ELISA titers for toxocara sp were positive in all cases, ranging from 64 to 1000. Six patients were treated with thiabendazole and one with albendazole. Steroids or hydroxiurea were administered to patients with heart failure or hyperleukocytosis. Subjects with lung involvement recovered quickly but those with cardiac compromise had a partial recovery with frequent decompensations of their cardiac failure and one death. Eosinophilia lasted up to 20 years, in spite of specific treatment. We emphasize the importance of infection prevention, accuracy of diagnosis and the severity of cardiac damage in the adult patient