Leucemia linfocítica crónica con infiltración ocular / Chronic lymphocytic leukemia with eye infiltration

La incidencia de la leucemia linfocítica crónica (LLC) aumenta progresivamente con la edad; aproximadamente el 75 por ciento de los casos presentan 60 años o más. Este tipo de leucemia es más frecuente en varones y se desconoce su causa, existen casos que son de origen hereditario. Se presenta el caso de un paciente con el diagnóstico de LLC con infiltración ocular. Paciente blanco, masculino de 76 años de edad con antecedentes de salud de haber sido diagnosticado con LLC desde hace 5 años que se trata con Leukeran (Clorambucil) (2mg) 2 tabletas en el almuerzo y 3 tab en la comida, así como de prednisona (5mg) 1 cada 8 horas solo cuando está descompensado. Hace alrededor de 3 días comenzó con astenia, anorexia, mareos, dolor e inflamación del párpado superior derecho. El examen físico, la biopsia del párpado superior y el frotis de sangre revelaron la presencia de una recaída hematológica de la LLC con infiltración ocular(AU)

The incidence of chronic lymphocytic leukemia (CLL) increases progressively with age, approximately 75 percent of cases present 60 years or more. This type of leukemia is more frequent in men and its cause is unknown, there are cases that are of hereditary origin. A clinical case of a patient with the diagnosis of Chronic Lymphocytic Leukemia with ocular infiltration is presented. A 76-year-old white male patient with a health history of having been diagnosed with a Chronic Lymphocytic Leukemia for 5 years who is treated with Leukeran (Chlorambucil) ( 2mg) 2 tab at lunch and 3 at night; and prednisone (5mg) 1 tab every 8 hours. About 3 days ago begins with asthenia, anorexia, dizziness, pain and swelling of the upper right eyelid. Physical examination, upper eyelid biopsy and blood smears reveal the presence of a hematological relapse with ocular infiltration(AU)

Prevalence of ZAP-70 and CD 38 in Indian chronic lymphocytic leukemia patients.

Aim of Study: Chronic lymphocytic leukemia (CLL) is the most common chronic lympho-proliferative disorder. This study was undertaken to know the prevalence of ZAP-70 and CD 38 in the treatment naive patients of CLL seen at a tertiary care centre of north India. Materials and Methods: ZAP-70 and CD 38 were tested by flow cytometry on peripheral blood samples. ZAP-70 positive and CD 38 positivity was defined as positive expression on 20% and 30% of CLL cells, respectively. Clinico-hematological profile and its correlation with ZAP-70 and CD 38 were assessed in consecutive 80 CLL patients. Results: There were 64 males and median age of the group was 58 years. Sixteen patients (20%) were asymptomatic and diagnosed incidentally. Median total lymphocyte count (TLC) at presentation was 62 × 10 9 /L. Rai stage distribution was: Stage 0-6, stage I-20, stage II-36, stage III-5, and stage IV-13. ZAP-70 and CD 38 positivity were detected in 20 patients (25%) and 29 patients (36%), respectively. Eleven patients were positive and 34 were negative for both ZAP-70 and CD 38 yielding a concordance rate of 56%. There was no statistically significant difference between ZAP-70 and CD 38 positivity and negativity with regard to age, sex, Lymphocyte count, lymphadenopathy, organomegaly, and Rai staging. Conclusion: ZAP-70 and CD 38 positivity were detected 25% and 36%, respectively, with concordance rate of 56%, which is higher than Western literature. There was no correlation of ZAP-70 and CD 38 positivity with age, sex, lymphadenopathy, organomegaly, and Rai staging.

Monoclonal B-cell lymphocytosis: a brief review for general clinicians / Linfocitose monoclonal de células B: uma breve revisão para clínicos gerais

Monoclonal B-cell lymphocytosis (MBL) is a recently described medical condition that displays biological similarities to the most common subtype of adult leukemia in the Western world, i.e. chronic lymphocytic leukemia (CLL). Diagnostic criteria have been published with the aim of differentiating between these two entities. The overall prevalence of MBL is at least 100 times higher than that of CLL, which indirectly suggests that MBL is not necessarily a pre-leukemic condition, although in some circumstances, CLL cases can really be preceded by MBL. In view of this high prevalence rate, general clinicians and even non-hematological specialists have a high chance of being faced with individuals with MBL in their routine clinical practice. MBL is classified as "clinical MBL", "population-screening MBL" and "atypical MBL" and the clinical management of affected individuals depends greatly on this differentiation. The present review provides a guide to diagnosing and following up MBL patients.

A linfocitose monoclonal de células B (LMB) é uma condição médica recentemente descrita que exibe similaridades biológicas com o mais comum subtipo de leucemia em adultos de países ocidentais, qual seja, a leucemia linfocítica crônica (LLC). Critérios diagnósticos foram publicados com o intuito de separar as duas entidades. A prevalência global da LMB é pelo menos 100 vezes maior do que a da LLC, o que, indiretamente, sugere que a LMB não é necessariamente uma condição pré-leucêmica, embora, em algumas circunstâncias, casos de LLC possam realmente ser precedidos pela LMB. Em virtude dessa alta taxa de prevalência, clínicos gerais e mesmo outros especialistas não hematologistas têm grande chance de deparar-se com casos de LMB em suas rotinas clínicas. A LMB é classificada como "LMB clínica", "LMB de screening populacional" e "LMB atípica", sendo que o manuseio clínico dos indivíduos afetados depende substancialmente dessa diferenciação. A presente revisão fornece um guia para o diagnóstico e acompanhamento dos pacientes com LMB.

Aspectos epidemiológicos, biotipologia e evolução do tratamento da leucemia linfocítica aguda na infância e adolescência no Rio Grande do Sul / Epidemiologic aspects, biotypology and evolution of acute lymphocytic leukemia in childhood and adolescence in Rio Grande do Sul

A leucemia linfocítica aguda na infância e adolescência é umaneoplasia de precursores linfoides de natureza heterogênea. NoBrasil, os estudos cooperativos foram iniciados em 1980. Nasequência destes estudos foram observadas sobrevidas livre deeventos de 50%, 58% e 70% nos protocolos 80, 82 e 85 respectivamente.Neste trabalho foram avaliados 1.472 pacientes comLLA, 56,59% masculinos e 43,41% femininos, com idades entre 0e 20 anos, média de 7,40 anos, no período de 1980 a 2008 provenientesdo RS. Os dados foram colhidos dos prontuários dos pacientesdas principais instituições hospitalares que assistemneoplasias hematológicas pediátricas. Neste estudo, 487 pacientes(39,40%) foram registrados nos protocolos do GBTLI; 678(54,85%) receberam tratamento baseados nos regimes do grupoBFM e 71 (5,75%) por outros regimes. A sobrevida livre de eventosdos pacientes protocolados foi significativamente superiorcomparada aos não protocolados, 62,41% ± 2,43% e 53,86% ±2,04% respectivamente, em cinco anos. Pacientes com idade de 15a 19 anos tiveram um índice de SLE de 37,98% ± 4,72% em cincoanos, inferior quando comparado aos de 0 a 4 anos e 5 a 9 anosrespectivamente: 62,78% ± 2,28% e 62,43% ± 2,84%. Foi observada,na população estudada, uma SG de 63,73% ± 1,49% e SLEde 57,27% ± 1,57%. A incidência da LLA com progenitores Bseguiu o padrão observado em países desenvolvidos com um picode frequência absoluta entre as idades de 2 a 4 anos. Houve diferençasignificativa entre a população da região urbana ou rural:SLE em cinco anos de 61,76% ± 1,76% e 49,81% ± 4,28% respectivamente.A SLE e a SG em lactentes e portadores de síndrome deDown foi inferior aos resultados obtidos em instituições dos paísesdesenvolvidos.

Acute lymphocytic leukemia (ALL) in childhood andadolescence is a neoplastic disease of varied lymphoid precursors.In Brazil, cooperative studies on the treatment of ALL started in1980. According to these studies, the event free survival (EFS)was 50%, 58% and 70% in the protocols of 1980, 1982 and 1985,respectively. In this work, 1472 ALL patients were evaluated;56.59% were male and 43.41% were female with ages that rangedbetween 0 and 20 years old (mean age of 7.4 years old). Data wascollected from the records of patients with hematologic neoplasiasin medical institutions that offered treatment for pediatricneoplasias in the period from 1980 to 2008. In this study, 487patients (39.40%) were registered in the GBTLI protocol; 678(54.85%) received treatment based on the BFM group regimenand 71 (5.75%) were treated according to other regimens. TheEFS at five years of the patients on the GBTLI protocol wassignificantly higher than those who were not on this protocol(62.41% ± 2.43% and 53.86% ± 2.04%, respectively). In respectto the age range, the patients who were between 15 and 20 yearsold had an EFS of 37.98% ± 4.72% at five years, which is lowerthan the rate for 0 to 4-year-old and 5 to 9-year-old patients(62.78% ± 2.28% and 62.543± 2.84%, respectively). An overallsurvival of 63.73% ± 1.49% and EFS of 57.27% ± 1.57% wereobserved in the studied population. Epidemiologically, theincidence of ALL in B progenitors followed the pattern observedin developed countries with an absolute frequency peak in the agerange of 2 to 4 year olds. The outcome was better for patientsfrom urban areas compared to those from rural areas. EFS andoverall survival in infant and Down syndrome patients were worsethan the results obtained in developed countries.

Epidemiologia da leucemia linfocítica crônica e leucemia linfocítica crônica familiar / Epidemiology of chronic lymphocytic leukemia and familial chronic lymphocytic leukemia

A leucemia linfocítica crônica (LLC) é uma doença linfoproliferativa crônica (DLPC) que apresenta características epidemiológicas peculiares; acomete indivíduos com idade mais avançada (não ocorre em crianças e é rara abaixo dos 30 anos) e a sua incidência varia conforme a origem étnica dos pacientes. É a leucemia mais freqüente nos países ocidentais enquanto muito rara nos orientais. A sua etiologia não está ainda esclarecida, não se conhecendo fatores ambientais que mostrem forte associação com o surgimento da doença. Não tem relação com radiação ionizante (sobreviventes da bomba atômica não apresentaram aumento na incidência da LLC), assim como não foi demonstrada associação com agentes tóxicos ou virais específicos. A LLC familiar é assim denominada quando, pelo menos, dois membros de uma família apresentam LLC e o diagnóstico nestes pacientes costuma ocorrer em idade mais precoce. A ocorrência é maior em parentes de primeiro grau (irmãos, filhos), podendo, porém, afetar parentes mais distantes. Os familiares de pacientes com LLC apresentam maior freqüência de outras DLPC (risco relativo 30x maior) e de proliferação monoclonal de linfócitos B (13 por cento-18 por cento) e parece que o HLADR1.11 está implicado na LLC familiar, pelo menos em algumas populações.

Chronic lymphocytic leukemia (CLL) is a chronic lympho-proliferative desorder (CLPD) with peculiar epidemiologic characteristics. It is a disease of the elderly, which is very rare in under 30-year-old individuals and absent among children. Its incidence largely varies according to the ethnical origin: CLL is the most common leukemia in Western countries while it is rarely seen in Eastern countries. The etiology of CLL is still unknown. Environmental factors such as exposure to ionizing radiation (atomic bomb survivors did not show an increased incidence of CLL) or toxic or viral agents are not associated to the occurrence of CLL. Familiar CLL is characterized when at least two members of the same family develop CLL The diagnosis of familiar CLL seems to be made 10 to 20 years earlier than sporadic cases. CLL occurs more frequently among siblings and offspring although it may appear in other members such as cousins. The frequency of other CLPD (relative risk is 30 times greater) and monoclonal CD5+B cells proliferations (13.5 percent-18 percent) are higher among "healthy" members of familial CLL and HLADR1/11 is implicated in at least some populations with familial CLL.

Epidemiologia, tratamento e profilaxia das infecções na leucemia linfóide crônica / Epidemiology, treatment and prophylaxis of infections in chronic lymphocytic leukemia

Infecção é uma freqüente complicação em pacientes com leucemia linfocítica crônica (LLC). O risco de infecção é maior à medida que a doença avança, e guarda estreita correlação com a dosagem de imunoglobulinas no sangue. Embora o defeito imune primário na LLC seja deficiência de imunoglobulinas, as diversas modalidades de tratamento comprometem outros elementos do sistema imune, ampliando o espectro de agentes infecciosos. Este texto sumariza o conhecimento atual sobre os riscos de infecção com as diversas modalidades de tratamento e apresenta recomendações para o manejo destas infecções, seja na prevenção seja no tratamento. Tais recomendações são colocadas em graus de evidência tanto do ponto de vista de sua força quanto da qualidade da evidência.

Infection is a frequent complication in patients with chronic lymphocytic leukemia (CLL). The risk of infection is greater with advanced disease and with lower serum immunoglobulin levels. In newly diagnosed patients, the main immunodeficiency is hypogamaglobulinemia, but with different regimens of treatment, other immunodeficiencies occur and contribute to changing the spectrum and increasing the incidence of infection. This paper reviews the current knowledge of the risks and epidemiology of infection according to the different regimens given in the treatment of CLL, and presents recommendations for prophylaxis and treatment of these infections. Such recommendations are rated according to standard scoring schema used in other guidelines to illustrate the strength of the supporting evidence and the quality of the data.

Análisis de factores pronósticos en leucemia linfática crónica. Valor predictivo del inmunofenotipo, el CD23 soluble y la morfología / Predictive factors in chronic lymphocytic leukemia. Value of immunophenotype, SCD23 and morphology

En LLC se reconocen factores pronósticos útiles como la duplicación linfocitaria, el estadio clínico y el patrón de infiltración medular. Otros, como el porcentaje de células CD38+, están en estudio y requieren confirmación. El objetivo del presente trabajo fue evaluar si existe asociación entre morfología, inmunofenotipo linfocitario, CD23 soluble (SL) y sobrevida libre de eventos (SLE). Se evaluaron prospectivamente 36 pacientes sin tratamiento. Se determinaron: morfología típica, mixta y LLC-PL; inmunofenotipo linfocitario (score de Matutes); niveles plasmáticos de CD23 SL; estadio clínico, duplicación linfocitaria; ß2 microglobulina y alteraciones citogenéticas. Se consideró evento: progresión de enfermedad (necesidad de tratamiento, evolución a estadios avanzados, desarrollo de organomegalia voluminosa) y muerte por enfermedad. Mediana de seguimiento 24 meses. Resultados: estadio 0: 11/36, SLE 80 por ciento; I: 10/36 SLE 90 por ciento; II: 13/36: III y IV: 2/36. SLE >= II 37 por ciento. p= 0.023. Duplicación linfocitaria: <12m 7/31, >12m 24/31. SLE 28 por ciento vs 80 por ciento p<0.001. Citogenético: normal 13/28; anormal 15/28. SLE 92 por ciento vs 54 por ciento p=0.053. +12 positiva 7/30, negativa 23/30. SLE 65 por ciento vs 66 por ciento. ß2 microglobulina normal 9/35, elevada 26/35; SLE 100 por ciento vs 53 por ciento p=0.006. D23 SL < 350 Ul/ml 15/32, > 350 Ul/ml 17/32. SLE 92 por ciento vs 53 por ciento p=0.005. Inmunofenotipo: Score 5: 15/36, Score 4: 19/36, SLE 64 por ciento. Score 3: 2/36. p=0.516. Morfología típica 17/35, mixta 17/35. SLE 81 por ciento vs. 57 por ciento p=0.099. LLC-PL 1/35. El CD 23 SL resultó adecuado para predecir SLE, particularmente útil en estadios iniciales sin otros marcadores de actividad. La morfología y el fenotipo linfocitario, dos variables accesibles, no fueron útiles para el propósito del estudio.

Profil epidemiologique; clinique et biologique de la leucemie lymphoide chronique. Experience du service d'hematologie clinique du CHU de Yopougon : a propos de 50 cas colliges

L'etude retrospective du profil clinique et biologique de la leucemie lymphoide chronique portant sur 50 cas recrutes dans les services d'hematologie clinique du CHU de Yopougon et de Option : Medecine interne du CHU de Treichville a permis de retenir les resultats suivants. Au plan clinique et epidemiologique : il existe une legere predominance feminine avec un sex-ratio de 0;43; l'age moyen est de 60;46 ans; la tranche d'age predominante est celle de 61 a 70 ans; le groupe ethnique le plus atteint est le groupe KWA; 72 pour cent; les menageres sont les plus exposees. 60 pour cent; le syndrome tumoral domine le tableau clinique avec; au premier rang la splenomegalie dans 32 pour cent. Au plan biologique : il existe une hyperleucocytose moderee et constante dans 78 pour cent des cas; la lymphocytose medullaire est constante chez 70 pour cent de nos patents; la lymphocytose sanguine est moderee (inferieure a 50000 mm3) chez 86 pour cent de nos patients; leucemie lymphoide chronique; syndrome tumoral ; lymphocytose sanguine et medullaire; lymphocytes

Clinicoepidemiological features of adult leukemias in Pakistan

A total of 113 patients of leukemia, over 15 years of age, were seen in three different institutions from July, 1992 to June, 1994. There was an almost equal distribution of acute myeloid leukemia [AML] and acute lymphoblastic leukemia [ALL] [44 vs 43 cases respectively]. Chronic lymphocytic leukemia [CLL] was the least common, accounting for 5% of all cases. Mean age in CLL was 59 years. Chronic myeloid leukemia [CML] was three times commoner than CLL with a younger age distribution [median age was 34 years]. We conclude that the clinicoepidemiological features of adult leukemias differ considerably from that seen in the developed world. However, recruitment of patients needs to continue in order to define these features based on a larger patient population

Clasificación, frecuencia y distribución de las lesiones linfoides oculares y orbitarias en la A.P.E.C. (revisión de 30 años: 1959-1990) / Study of ocular and orbital lymphoid lesions in a 30 year period at the A.P.E.C

Se presenta un estudio retrospectivo de revisión de lesiones linfoides oculares y orbitarias en un periodo de 30 años en el Hospital de la A.P.E.C. En total se evaluaron 60 casos, de los cuales, el 41.66 por ciento correspondió a lesiones malignas, el 31.66 por ciento a lesiones benignas y el 21.66 por ciento a lesiones limítrofres constituídas por todos aquellos casos con diagnóstico histopatológico dudoso entre linfoma e hiperplasia linfoide. De las lesiones malignas, el 76 por ciento correspondió a linfoma con predominio del tipo linfocítico y el 94.74 por ciento de la patología benigna se debió a hiperplasi linfoide. Los linfomas se presentaron entre la 7a. y 8a. decádas de la vida; por lo contrario la hiperplasia prevaleció en pacientes con edades entre 20 y 40 años. Se encontró una mayor frecuencia de las lesiones conjuntivales y no se demostraron diferencias clínicas significativas que distingan a los procesos benignos de los malignos. Se realiza una revisión de los factores inmunogenéticos involucrados en las nuevas clasificaciones de lesiones linfoides y se concluye que es necesaria la utilización de inmunohistoquímica para la clasificaicón definitiva de las lesiones limítrofes y se propone un protocolo clínico para la adecuada evaluación de los pacientes con tumores linfoides.

Malignant lymphoma--a 15-year study report.

Analysis of incidence of different types of malignancies during 15 years showed occurrence of malignant lymphoma in 192 cases (4.1%). There were 82 cases (42.7%) of non-Hodgkin's lymphoma and 110 cases (57.3%) of Hodgkin's disease. Lymphocytic lymphoma, the major type observed among the non-Hodgkin's lymphoma with 58 cases (30.21%), showed a preponderance of well differentiated type (40 cases). Other cases in this group comprised mixed cellularity (2.08%), histiocytic (3.64%) and unclassified one (6.77%). Analysis of the Hodgkin's disease cases showed majority in it belonged to the mixed cellularity type (44.5%) followed by the lymphocytic predominant type (27.3%), lymphocytic depletion type (18.2%) and nodular sclerosing type (10%). The incidence of 14 cases of extranodal lymphomas was 7.3% among the lymphomas with maximum occurrence in the gastrointestinal tract (42.8%).

Incidence estimation of leukemia among Koreans

This study was undertaken in order to estimate the incidence of leukemia among Koreans. Medical records were studied of patients with diagnoses of either ICD-9 038 (septicemia), or 204-208 (leukemias), or 284 (aplastic anemia), or 289 (other diseases of the blood and blood-forming organs) in the claims sent in by medical care institutions throughout the country to the Korea Medical Insurance Corporation (KMIC) during the period from January 1, 1986 to December 31, 1987. These records were abstracted in order to identify and confirm new cases of leukemia among the beneficiaries of KMIC, which covers about 10% of the whole Korean population. Using these data from the KMIC, the incidence rates of leukemia among Koreans were estimated as of July 1st, 1986 to June 30, 1987. The crude incidence rate of all types of leukemia among Koreans is estimated to be 3.45 (95% CI; 0.77-9.55) and 2.29 (95% CI; 0.28-7.81) per 100,000 in males and females, respectively. The cumulative rate for the age span 0-64 is 0.25% in males and 0.18% in females, and for the age span 0-74, 0.35% in males and 0.23% in females. The adjusted rates for the standard world population are 3.90 and 2.48 per 100,000 in males and females, respectively. The relative frequencies by type are 51.5% for AML, 21.6% for ALL, 20.2% for CML, and only 1.5% for CLL. The incidence patterns of various types of leukemia, of which this is the first report in Korea, are analyzed and presented.(ABSTRACT TRUNCATED AT 250 WORDS)

Neoplasias malignas do globo ocular e demais conteúdos orbitários no Hospital Erasto Gaertner - (1973/1986) / Malignant neoplasms of ocular globe and others orbital contents at Erasto Gaertner Hospital (1973/1986)

Os autores apresentam a freqüência das neoplasias malígnas do Globo Ocular e Demais Conteúdos Orbitários. Estudam os 69 casos destas neoplasias malígnas atendidas no Hospital Erasto Gaertner, no período de 1973 a 1986, comparando-as com as freqüencias em relaçäo as outras regiöes anatômicas. Fazem um levantamento da literatura sobre a freqüência destes tumores em outros países e regiöes braileiras, procurando evidenciar algumas diferenças regionais

Leucemia linfocitica cronica: experiencia acumulada en el servicio de hematología del Hospital San Juan de Dios / Chronic lymphocytic anemia: cases seen in the Hematology Departament at the San Juan de Dios Hospital

En un estudio realizado en el servicio de hematologia del Hospital San Juan de Dios, durante un periodo de 20 años (1967-1988), se analizo los datos hematologicos, los diagnosticos y la evolucion de los pacientes con leucemia linfocitica cronica (LIC) con el proposito de conocer la frecuencia de esta entidad en Costa Rica y compararla con la que se presenta en otros paises. La revision de los archivos de 16.946 pacientes de los cuales se habia obtenido una muestra de medula osea por aspiracion, permitio escoger 20 casos que reunian criterios para calificarlos como de LLC. Se establecio la frecuencia de 1.18 casos de L.L.C,5,43 de leucemia granulocitica cronica (LGC) y 30 de leucemia aguda (LA), por cada mil estudios de medulas oseas realizadas en el servicio de hematologia. Se determino que la frecuencia de la LLC es similar a la reportada en estudios efectuados en Mexico, en Japon y en China, paises en los cuales la frecuencia es baja, a diferencia de la reportada en Estados Unidos de Norteamerica.