Infiltración del nervio óptico como forma inicial de recaída de la leucemia linfoblástica aguda / Optic nerve infiltration as an initial form of relapse of acute lymphoblastic leukemia

Introducción: La infiltración del nervio óptico como forma inicial de recaída de la leucemia linfoblástica aguda es rara, aunque altamente indicativa de que el sistema nervioso central está involucrado. Objetivo: Actualizar el conocimiento sobre infiltración del nervio óptico como forma inicial de recaída de la leucemia linfoblástica aguda. Métodos: Se realizó una revisión de las publicaciones más relevantes relacionadas con el tema durante los últimos años. La búsqueda y la localización de la información se apoyaron en la elección de palabras clave/descriptores que configuraron el perfil de búsqueda. Se utilizó el MeSH Database de PubMed. Se realizó una extensa revisión en Google Académico y otros megabuscadores de revisión sistemática mediante TripDatabase y Cochrane. Conclusiones: La infiltración directa de células tumorales y las alteraciones hematológicas propias de la enfermedad constituyen los mecanismos fundamentales de la fisiopatogenia. El edema del disco óptico es su signo oftalmoscópico más distintivo. La imagen por resonancia magnética de cráneo, el análisis citológico del fluido cerebroespinal y la biopsia de médula ósea negativas no deben excluir el diagnóstico. La terapia combinada que incluye la radiación localizada constituye una buena opción de tratamiento. Un número considerable de ojos recuperan su agudeza visual y muestran resolución del cuadro infiltrativo(AU)

Introduction: Optic nerve infiltration as an initial form of relapse of acute lymphoblastic leukemia is rare, although it is highly indicative of central nervous system involvement. Objective: To update the knowledge about optic nerve infiltration as an initial form of relapse of acute lymphoblastic leukemia. Methods: A review of the most relevant publications related to the subject during the last years was carried out. The search and localization of information was supported by the choice of keywords/descriptors that configured the search profile. The MeSH Database of PubMed was used. An extensive review was performed in Google Scholar and other systematic review mega search engines using TripDatabase and Cochrane. Conclusions: Direct tumor cell infiltration and disease-specific hematologic alterations are the fundamental mechanisms of pathophysiology. Optic disc edema is the most distinctive ophthalmoscopic sign. Negative cranial magnetic resonance imaging, cytologic analysis of cerebrospinal fluid and bone marrow biopsy should not exclude the diagnosis. Combination therapy including localized radiation is a good treatment option. A considerable number of eyes recover visual acuity and show resolution of the infiltrative picture(AU)

Perfil neuropsicológico y efectos de la rehabilitación cognitiva en la leucemia linfoblástica aguda: a propósito de un caso / Neuropsychological profile and cognitive rehabilitation training effects on acute lymphoblastic leukaemia: a case study

La Leucemia Linfoblástica Aguda (LLA) infantil es el cáncer pediátrico más frecuente. Actualmente cuenta con un alto porcentaje de supervivencia, pero dichos pacientes presentan secuelas cognitivas secundarias a la enfermedad debidas, principalmente, al tratamiento médico recibido para evitar la recidiva del cáncer. Por lo tanto, resulta necesaria la implementación de programas de rehabilitación cognitiva específicos para este tipo de población. Es por ello que el objetivo del presente estudio fue describir los déficits cognitivos en un varón de 17 años que fue diagnosticado con LLA a los 9 años. Tras la valoración neuropsicológica inicial se desarrolló un programa de rehabilitación cognitiva intensivo durante dos años consecutivos. Realizamos un estudio pre-post en el que administramos el Conners Continuous Performance Test (CPT-II) y la Escala de Inteligencia de Wechsler para niños (WISC-IV). Los resultados, antes de la intervención, mostraron que el paciente manifestaba una menor velocidad de procesamiento y dificultades de atención sostenida y alternante, comprensión verbal y razonamiento perceptivo. Además, también presentó un número considerable de errores perseverativos, signo de dificultades de flexibilidad cognitiva y control inhibitorio. Dichos déficits mejoraron notablemente tras el programa de rehabilitación cognitiva. En conclusión, nuestro estudio pone de manifiesto la necesidad de aplicar programas de rehabilitación cognitiva tempranos para paliar las secuelas cognitivas derivadas de la LLA y de su tratamiento médico, así como mejorar la calidad de vida del paciente, ya que las mejoras cognitivas redundarán en su rendimiento académico y en su funcionamiento cotidiano.

Childhood Acute Lymphoblastic Leukemia (ALL) is the most common pediatric cancer. It currently has a high survival rate, but these patients have cognitive sequelae secondary to the disease, mainly due to the medical treatment received to prevent cancer recurrence. Therefore, it is necessary to implement specific cognitive rehabilitation programs for this type of population. Hence, the main objective of this study was to describe cognitive deficits in a 17-year-old male who was diagnosed with ALL when he was 9 years old. After the initial neuropsychological evaluation, an intensive cognitive rehabilitation program was developed during two consecutive years. We conducted a pre-post study in which we administered the Conners Continuous Performance Test (CPT-II) and the Wechsler Intelligence Scale for Children (WISC-IV). Results, before the intervention, showed that the patient presented a lower processing speed and difficulties of sustained and alternating attention, verbal comprehension and perceptive reasoning; in addition to a large number of perseverative errors, sign of self-monitoring difficulties and inhibitory control. These deficits improved markedly after a program of cognitive rehabilitation. In conclusion, our study highlights the need to apply early cognitive rehabilitation programs to alleviate the cognitive sequelae derived from ALL and its medical treatment. In addition, any improvement in their cognitive capabilities will have a positive impact in their academic performance and quality of life.

Efeitos de osmólitos na L- asparaginase II de Erwinia chrysanthemi em meio aquoso / Effects of omolytes in L- asparaginase II from Erwinia chrysanthemi in aqueous medium

A L- asparaginase é uma enzima aplicada no tratamento de Leucemia Linfoide Aguda, que atua na hidrólise da L- asparagina, privando a célula tumoral de um aminoácido essencial para o seu crescimento. A L- asparaginase, como outros biofármacos, deve ser estável, manter sua atividade específica e formar poucos agregados. A fim de manter a integridade do biofármaco, são utilizados adjuvantes nas formulações farmacêuticas, e dentre os mais importantes estão os osmólitos. Essas moléculas protegem a estrutura nativa da proteína, sendo capazes de interferir na formação de agregados e garantir a estabilidade proteica. O presente trabalho teve o objetivo de estudar o efeito dos osmólitos sacarose, sorbitol, arginina e glicina na atividade específica, estabilidade, cinética e caracterização de agregados na solução de L- asparaginase II de Erwinia chrysanthemi. Os resultados mostraram que a maioria dos osmólitos testados aumentou a atividade específica e a estabilidade da enzima, o que pode estar relacionado com o aumento da velocidade máxima e do kcat observados no ensaio cinético realizado com sacarose e sorbitol. Um perfil diferente de agregados foi encontrado para cada tipo de osmólito. A presença de sacarose ou sorbitol resultou na menor quantidade de agregados na faixa de, respectivamente, 100 a 200 e 200 a 300 nm em relação a enzima sem osmólito. Por outro lado, aumento no número total de agregados e presença de moléculas de alto peso molecular (300 a 500 nm) foram observados nas soluções enzimáticas contendo, respectivamente, glicina e arginina. Dessa forma, os resultados obtidos neste trabalho poderão auxiliar na produção e escolha da formulação de biofármacos, e, consequentemente, melhorar o tratamento medicamentoso de pacientes.

L L-Asparaginase is an enzyme applied in the treatment of Acute Lymphoblastic Leukemia, which acts on the hydrolysis of L- asparagine, depriving the tumor cell of an essential amino acid for its growth. L-asparaginase, as other biopharmaceuticals, must be stable, maintain its specific activity and form few aggregates. In order to maintain the integrity of the biopharmaceutical, adjuvants are used in the pharmaceutical formulations, and among the most importants adjuvants are the osmolytes. These molecules protect the native structure of the protein, being able of interfering in the formation of aggregates and guarantee protein stability. The present work had the objective of studying the effect of the osmolytes sucrose, sorbitol, arginine and glycine in the specific activity, stability, kinetic and aggregates characterization, in L- asparaginase II solution of Erwinia chrysanthemi. The results showed that the majority of the tested osmolytes increased the specific activity of the enzyme and its stability, which may be related to the augment of maximum velocity and kcat observed in the kinetic assay performed with sucrose and sorbitol. A different profile of aggregates was found for each type of osmolyte. The presence of sucrose or sorbitol resulted in the least amount of aggregates in the range of, respectively, 100-200 and 200-300nm in relation to the enzyme without osmolyte. On the other hand, increase in the total number of aggregates and the presence of high molecular weight molecules (300 to 500 nm) were observed in the enzymatic solutions containing, respectively, glycine and arginine. Thus, the results obtained in this work may help in the production and choice of the formulation of biopharmaceuticals and, consequently, improve the drug treatment of patients.

Outcome of children and adolescents with lymphoblastic lymphoma / Evolução de crianças e adolescentes com linfoma linfoblástico

SUMMARY Introduction: lymphoblastic lymphoma (LBL) is the second most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course of children and adolescents with LBL treated at a tertiary center. Methods: this is a retrospective cohort study of 27 patients aged 16 years or less with LBL admitted between January 1981 and December 2013. Patients received intensive chemotherapy regimen derived from acute lymphoblastic leukemia (ALL) therapy. Diagnosis was based on biopsy of tumor and/or cytological examination of pleural effusions. The overall survival was analyzed using the Kaplan-Meier method. Results: the median age at diagnosis was 11.6 years (interquartile range, 4.6-13.8). LBL had T cell origin in 16 patients (59%). The most common primary manifestation in T-cell LBL was mediastinum involvement in 9 patients (56%). Intra-abdominal tumor was the major site of involvement in patients with pB-LBL. Most patients had advanced disease (18 patients - 67%) at diagnosis. Twenty-four patients (89%) achieved complete clinical remission. After a median follow-up of 43 months (interquartile range, 6.4-95), 22 patients (81%) were alive in first complete remission. Five children (18.5%) died, three of them soon after admission and two after relapsing. The probability of survival at five years for 20 patients with de novo LBL was 78% (SD 9.4). Conclusion: our findings confirm the favorable prognosis of children with LBL with an intensive chemotherapy regimen derived from ALL therapy.

RESUMO Objetivos: linfoma linfoblástico (LL) é o segundo subtipo mais comum de linfoma não Hodgkin em crianças. O objetivo deste estudo foi caracterizar a evolução clínica de crianças e adolescentes com LL em um centro terciário. Métodos: estudo de coorte retrospectivo de 27 pacientes com idade de até 16 anos com LL admitidos entre janeiro de 1981 e dezembro de 2013. Os pacientes foram tratados de acordo com o protocolo de tratamento para leucemia linfoblástica aguda (LLA). O diagnóstico foi baseado em biópsia do tumor e/ou no exame citológico de derrame pleural. A sobrevida global foi analisada pelo método de Kaplan-Meier. Resultados: a média de idade ao diagnóstico foi de 11,6 anos (variação interquartil, 4,6-13,8). LL de células T foi identificado em 16 pacientes (59%) e a manifestação primária mais comum foi o acometimento mediastinal em 9 pacientes (56%). Tumor intra-abdominal foi a manifestação clínica principal nos pacientes com LL de células pré-B. A maioria dos pacientes apresentava doença avançada (18 pacientes - 67%) ao diagnóstico. Vinte e quatro pacientes (89%) alcançaram remissão clínica completa. Após um período de acompanhamento médio de 43 meses (intervalo interquartil, 6,4-95), 22 pacientes (81%) continuam vivos em primeira remissão clínica completa. Cinco crianças (18,5%) morreram, três delas logo após a admissão e duas após recidiva. A probabilidade de sobrevida em cinco anos para 20 pacientes com LL de novo foi de 78% (SD 9.4). Conclusão: nossos resultados confirmam o prognóstico favorável de crianças com LL tratadas com regime de quimioterapia intensiva derivado da terapia de LLA.

Physical problems among leukemic adolescent patients undergoing chemotherapy in Erbil city

Leukemia is a significant public health and life-threatening problem for pediatric cancer patients. Adolescents leukemic may face long periods of treatment; may describe the irritability, fatigue, bone pain, mouth ulcer, alopecia, and loss of appetite. The aim of the study was to assess the common physical problems among leukemic adolescent patients undergoing chemotherapy, and identify the association between their socio-demographic characteristics with physical problem. A descriptive study was carried out in Nanakali Hospital for Blood Diseases / Erbil city from the period of /1[st] Nov. /2010 to/ 1[st] of Feb. /2011/. Eighty adolescent who are receiving chemotherapy in face to face interview, were selected regarding the study. The study shows that there were highly significant associations between socio-demographic characteristics with some of physical problems such as pain, fatigue, loss of appetite, and oral ulcer [mucositis]. The study shows that there were significant association between adolescent patient and some physical problems. The study recommends giving more support and attention by medical and nursing staff manage to reduce their physical problems

Efficacy of primary hepatitis B immunization in children with acute lymphoblastic leukemia.

BACKGROUND: Children with acute lymphoblastic leukemia (ALL) carry a high risk of hepatitis B virus (HIV) infection. The present study was conducted to see if prior routine hepatitis B vaccine received as a part of national immunization program could prevent HBV infection in these children. METHODOLOGY: Ninety-six children with ALL were screened for HBV. Children were divided into three groups according to their initial HBV serology; previously vaccinated children (Group I) (n=34) previously unvaccinated and seronegative children (Group II) (n=56),and unvaccinated but HBsAg negative and anti-HBs positive children (group III) (n=6). Sixty-seven of 96 (69.7%) children received vaccination. The schedule was initiated during the third month of maintenance therapy and each course consisted of three doses given at one month interval. RESULTS: Anti-HBs seroconversion following the first course of three doses of hepatitis B vaccination in group I, II and III was 57%, 33% and 100%, respectively. It increased to 97% in Group I, 62.5% in Group II, 100% in Group III. HBsAg positivity was found in 11 children (11.5%) and all of them developed chronic hepatitis B. Ten of them were in Group II whereas only one child was in Group I (P<0.04). CONCLUSION: This data reveals that routine HBV vaccination within the national immunization program plays an important role in decreasing subsequent hepatitis B infection in children with ALL.

Densitometría ósea en niños leucémicos al completar el primer mes de quimioterapia / Bone mineral density in leukemic children after completing one month of chemotherapy

Background: An important loss of bone mineral density, associated to pain and fractures, has been reported in children with acute lymphoblastic leukemia (ALL). Aim: To measure bone mineral density among children with acute lymphoblastic leykemia (ALL) that completed the remission induction phase with chemotherapy, that lasts 30 days. Patients and methods: children with ALL, admitted to the oncology unit of a general hospital were considered eligible for the study. body composition and bone mineral density were measured by dual energy x ray absorptiometry (DEXA). each child with ALL was paired with a healthly control. Results: Fourteen children age 1 to 11 years, completed the study, Spine and femoral bone mineral desities were significantly lower than in their matched controls. No differences in total body bone mineral density or content were observed. Children with ALL had a lower fat free mass and a higher fat mass than their matched controls. There was a significant correlation between fat free mass and bone mineral content. Conclusions: After one month of chemotherapy, children with ALL had a lower bone mineral density in the spine and femur and a lower fat free mass.

Metaloproteinasas como marcadores de progresión tumoral y futuros blancos de terapias en patologías linfoides / Metalloproteinases as markers of tumoral progression and future targets for therapies in neoplasms of lymphoid tissue

En las últimas décadas la medicina evolucionó hacia un enfoque molecular en la búsqueda de blancos específicos que permitan seguir adecuadamente la progresión de las patologías neoplásicas y desarrollar terapias exitosas. El conocimiento y comprensión de que la matriz extracelular (MEC) que rodea a un tumor influye sobre el comportamiento del mismo, reveló la importancia que cumplen las metaloproteinasas (MMPs) en la regulación de los componentes de la MEC, y por lo tanto en el desarrollo tumoral. Es por ello que actualmente se está evaluando la eficacia de inhibidores sintéticos de estas enzimas en ensayos clínicos, algunos ya en fase clínica III de investigación. También se propone que estas MMPs podrían ser utilizadas como marcadores bioquímicos, permitiendo evaluar la progresión de la enfermedad en pacientes que sufren patologías neoplásicas, e incluso dar un valor pronóstico. Es en este punto en que el laboratorio de análisis clínicos cumplirá un papel fundamental y deberá contar con los conocimientos y las herramientas necesarias para evaluar la presencia y actividad de estas enzimas. En este trabajo se expone el conocimiento actual de la estructura y funciones biológicas de las MMPs así como los antecedentes que muestran el papel que cumplen en las enfermedades neoplásicas, en especial en leucemias y linfomas

Nutritional assessment and serum zinc and cooper concentration in leukemic children

Context: Malnutrition in childhood cancer is commonly a serious problem. Changes in blood zinc and copper have also been found in malignant diseases. Objective: To describe the protein-energy nutritional status and serum zinc and copper of children with newly diagnosed leukemia. Design: Cross-sectional study. Setting: University referral center. Participants: 23 children with newly diagnosed acute lymphocytic leukemia (ALL) or acute non-lymphocytic leukemia (ANLL) between the ages of 1 and 10 years. The control subjects were 31 healthy school children of similar age from local schools. Main measures: Anthropometric measurements of height/age and weight/height, food intake and serum levels of zinc and copper. Results: Almost the entire group of children were eutrophic. Zinc and copper intake were below the recommended values. Serum zinc levels were significantly lower and serum copper levels were significantly higher in the leukemic group when compared to normal children. Conclusion: At the time of diagnosis the children suffering from leukemia were not overtly malnourished but blood analysis showed alterations in concentrations of the trace elements zinc and copper.

Serum adenosine deaminase and arylsulfatase A as an index of early infiltration of central nervous system in acute lymphoblastic leukemia

The level of adenosine deaminase [ADA] and arylsulphatase A [ASA] in the sera of 22 patients with acute lymphoblastic leukemia [ALL] were significantly increased when compared with the control healthy group. Also, there is a significant increase in the activity of these enzymes in patients with ALL with central nervous system [CNS] infiltration when compared with patients of ALL without CNS infiltration. We conclude that the estimation of ADA and ASA in the serum may be useful for detection of the early infiltration of the central nervous system by leukemic cells in acute lymphoblastic leukemia

Tres casos de necrosis de médula ósea en leucemia aguda / Three cases of bone marrow necrosis in acute leukemia

La necrosis de la médula ósea (NMO) es rara: frecuentemente es un hallazgo post-mórtem que ocurre en pacientes afectados por neoplasias hematológicas, en especial leucemia aguda. Descubrimos aquí NMO en dos pacientes con leucemia aguda mielobástica (LAM) y uno con leucemia aguda linfoblástica (LAL), en quienes se realizó el diagnóstico de NMO en vida. Dos pacientes fallecieron por hemorragia intracraneal; el paciente con diagnóstico de LMA M5 desarrolló NMO una semana después de recibir el segundo ciclo de quimioterapia: su recuperación fue total y está en remisión completa después de casi cinco años del diagnóstico. El diagnóstico de NMO puede ser difícil por lo que se requiere sospecharla para establecer un diagnóstico temprano y brindar tratamiento de apoyo, ya que no necesariamente se asocia a un resultado fatal

Regulation of gamma T-cell antigen receptor expression by intracellular calcium in acute lymphoblastic leukemia cell line DND41

The calcium inophore, ionomycin, promotes an increase of intracellular calcium and regulates mRNA expression of gamma/delta-TcR gene in human T lymphocytes. The mechanism of this regulation is not yet clear. Thus, the regulation by intracellular calcium requires elucidation. We studied the gamma-TcR gene expression in acute lymphoblastic leukemia cell line DND41 (CD4-CD8-) by Northern blot and flow cytometric analysis. The mRNA levels of gamma-TcR increased by ionomycin, anti-CD3, and with TPA. TPA had an antagonistic effect to both ionomycin and anti-CD3. Also, TPA inhibitis the increased intracellular calcium promoted by ionomycin but not the increase promoted by antiCD3 and ionomycin. Our results suggest that intracellular calcium induces mRNA and protein expression of gamma-TcR chain. This effect is antagonized by protein kinase C-activation. Thus, we conclude that the target cells of the differential regulation on gamma-TcR mRNA expression by intracellular calcium modulators are the CD4-CD8- cells, and this is due to cytosolic calcium mobilization

Manifestaciones osteoarticulares en la presentación inicial de la leucemia linfoblástica aguda en el niño / Osteoarticular manifestations in the inicial presentation of acute lymphoblastic leukemia in children

Se describen retrospectivamente las manifestaciones óseas y articulares de 70 pacientes de leucemia linfoblástica aguda, menores de 15 años, atendidos a lo largo de 9 años (1982 a 1991). Se registró dolor osteoarticular en 37,5 por ciento de ellos, en 8 (11,4 por ciento) eran muy intensos, ensombrecían los síntomas hematológicos y constituían el motivo de consulta; en 9 (12,9 por ciento) el dolor era importante y relatado espontáneamente, pero era un acompañante de los síntomas hematológicos que motivaban la consulta; en otros 8 (11,4 por ciento) casos de dolor era leve, ocasional o no referido espontáneamente. Las manifestaciones hematológicas eran poco marcadas en los primeros, aun en el momento de identificar la leucemia, después de varias semanas de dolor oseoarticular, lo que explica los errores en los diagnósticos iniciales (enfermedad reumática, artritis reumatoidea, sinovitis transitoria, enfermedad de Perthes, osteomielitis o trastorno psiquiátrico) en todos estos pacientes y el retraso del diagnóstico de leucemia. En los niños cuyos dolores osteoarticulares eran significativos pero en carácter de acompañantes de otro síntoma, no hubo errores iniciales en el diagnóstico, enfocándose el estudio a una afección hematológica, ya que los signos principales la sugerían. En los pacientes con poco dolor la situación era aún más clara. El dolor osteoarticular no modificó, sin embargo, el pronóstico, pues la sobrevida en los pacientes que lo sufrían fue similar que en los sin dolor. Los síntomas osteoarticulares sin explicación clara, sumados a alguna alteración hematológica, obligan a incluir leucemia en el diagnóstico diferencial

Leucemia linfoblástica aguda del adulto: experiencia del Instituto Nacional de Cancerología de 1983-1987 / Adult acute lymphoblastic leukemia: experience at the Instituto Nacional de cancerología, 1983 - 1987

De enero 1983 a diciembre de 1987 se diagnosticaron en el INCan, 40 casos nuevos de leucemia linfoblástica aguda. Correspondieron a 20 hombres y 20 mujeres con edad media de 20.5 años (extremos 13-41 años). Seis pacientes se excluyeron del análisis, tres pacientes fallecieron sin recibir tratamiento y tres murieron en las primeras 96 horas de la inducción. Veintiocho recibieron tratamiento de inducción con esquema HOP: doxorrubicina, vincristina, prednisona y seis recibieron el esquema LSA2L2: ciclofosfamida, vincristina, adriamicina y prednisona, La profilaxis al SNC fue irregular. Se administró un tratamiento de mantenimiento convencional por dos ó tres años con pulsos mensuales de vincristina, prednisona, metotrexato semanal y 6-mercapturina diariamente. En los pacientes que recibieron LSA2L2, se intensificó con ciclos mensuales y alternos de Ara-C, ciclofosfamida y adriamicina. En 34 casos evaluables, 19 (56 por ciento) alcanzaron respuesta completa, seis (16.5 por ciento) respuesta parcial y nueve (26.5 por ciento) fallecieron durante la inducción. La respuesta completa en 9/19 pacientes, se alcanzó en las primeras cuatro semanas con una mediana de seguimiento de 319 días (141-1736+). Sólo dos pacientes continúan vivos en primera respuesta completa. Un paciente, falleció por causa no relacionada con la LLA y otro está perdido para seguimiento, ambos con respuesta completa. Se analizan las causas de muerte durante la inducción y el bajo porcentaje de sobrevivientes libres de enfermedad a largo plazo

El cromosoma Filadelfia 30 años después / Chromosome Philadelphia thirthy years later

Se analiza la evolución histórica del conocimiento sobre el cromosoma Filadelfia durante los últimos 30 años, el papel que juega la biología molecular en su identificación y su importancia en el diagnóstico y pronóstico de la leucemia mieloide crónica y la leucemia linfoblástica aguda

Leucemia linfoblástica aguda CD10 (calla) negativo: grupo de mal pronóstico / Acute lymphoblastic leukemia CD10 (calla) negative: bad pronostic group

Se describen por primera vez en Venezuela 8 casos de LLA, CD10 Negativo, Dr y CD19 positivo encontrados en un estudio de 188 casos de LLA, realizado entre 1985 y 1990. La negatividad de CD10 indica indiferenciación celular y es de mal pronóstico. La edad de los pacientes estuvo comprendida entre 2 y 30 años, con un promedio de 11 años. La relación masculina-femenina fue 5/3 Todos los casos presentaron edenomegalias y hepatomegalia. Con excepción de un caso con leucopenia, el contaje de glóbulos blancos fue siempre mayor de 30.000/dl. En cuatro pacientes se practicó estudio cromosómico con resultado de hiperdiploidia. Se enfatiza la importancia de la Inmunología y la Genética en los síndromes linfoproliferativos para el estudio de grupos de mal pronóstico