Primary bilateral and symmetric MALT lymphoma of the lacrimal sac mimicking chronic dacryocystitis / Linfoma MALT primário, bilateral e simétrico do saco lacrimal simulando dacriocistite crônica

Abstract We report a case of primary bilateral mucosa associated lymphoid tissue (MALT) lymphoma of the lacrimal sac. MALT lymphoma is a subtype of Non-Hodgkin's Lymphoma of the ocular adnexa. When the primary site of the lymphoma is the lacrimal sac, it mimics chronic dacryocystitis. This may delay diagnosis, with potentially lethal results.

Resumo Descrevemos um caso de um linfoma MALT bilateral, simétrico e primário de saco lacrimal. O linfoma MALT é um subtipo do Linfoma Não-Hodkin dos anexos oculares. Quando o local primário do linfoma é o saco lacrimal, ele pode simular uma dacriocistite crônica. Essa situação pode atrasar o diagnóstico e ter consequências fatais.

Outcome of adjuvant chemotherapy with lomustine, vinblastine and chlorambucil on management of canine mast cell tumour of high to intermediate risk / Resultado da quimioterapia adjuvante com lomustina, vimblastina e clorambucil no manejo do mastocitoma canino de risco alto a intermediário

In spite of the many available protocols, the use of chemotherapy for the management of canine mast cell tumours (MCT) remains empirical, and there is lack of criteria for the choice of protocol and definition of patients who may benefit from treatment. The objective of this study was to evaluate the outcome of dogs with MCT after adjuvant chemotherapy according to the risk of recurrence or metastasis proposed on the literature. This prospective study included 89 followed up dogs with prognosis assesment including clinical, histological, immunohistochemical and genetic features of canine MCT. Patients were grouped according to risk of recurrence and metastasis and recommended treatment with lomustine followed by chlorambucil if considered at high-risk, or vinblastine followed by chlorambucil if a patient was at intermediate risk. Outcome was defined by disease-free interval (DFI) and overall survival (OS) estimated by Kaplan-Meier curve. Adjuvant lomustine was useful for control of canine MCT of high-risk of recurrence or metastasis, but only when sequentially associated to chlorambucil with a DFI of 686 days and not reached OS. There was no difference in outcome in the intermediate-risk group despite choosen treatment. Patients at intermediate-to-low risk may not require adjuvant treatments, even in the absence of free surgical margins.(AU)

Apesar dos inúmeros protocolos disponíveis, o uso da quimioterapia permanece empírico para o mastocitoma canino e faltam critérios para escolha do protocolo e da definição dos pacientes que poderiam se beneficiar do tratamento. O objetivo deste estudo foi avaliar o resultado de cães com mastocitoma após a quimioterapia adjuvante, de acordo com o risco de recorrência ou metástase proposto na literatura. Este estudo prospectivo incluiu 89 cães com acompanhamento clínico e avaliação prognóstica, incluindo características clínicas, histológicas, imuno-histoquímicas e genéticas dos mastocitomas. Os pacientes foram agrupados segundo o risco de recorrência ou metástase, sendo recomendado tratamento com lomustina seguida de clorambucila, se considerados sob alto risco, ou vimblastina seguida de clorambucila, se estivessem sob risco intermediário. O resultado final foi definido pelo intervalo livre de doença (ILD) e pela sobrevida global (SG), estimados pela curva de Kaplan-Meier. Na adjuvância, a lomustina foi útil no controle do mastocitoma canino de alto risco, mas apenas quando associada ao clorambucila, com um ILD de 686 dias, sem atingir a mediana para SG. Não houve diferença no grupo de risco intermediário, independentemente do tratamento escolhido. Pacientes de risco intermediário podem não necessitar de tratamentos adjuvantes, mesmo na ausência de margens cirúrgicas livres.(AU)

Action Mechanism of Chlorambucil against Mantle Cell Lymphoma Cell Line Jeko-1 / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To explore the action mechanism of chlorambucil against mantle cell lymphoma cell line Jeko-1.</p><p><b>METHODS</b>The effect of chlorambucil on Jeko-1 cell proliferation was measured by MTT method. The effect of chlorambucil on the apoptosis of Jeko-1 cell was detected by Hoechst staining and Annexin V-FITC dual staining. The activation of PI3K/AKT signaling pathway and the expression of BAX, BCL-2, procaspase 3, procaspase 8 and procaspase 9 were detected by Western blot.</p><p><b>RESULTS</b>0, 5, 10, 20 µmol/L chlorambucil could inhibit Jeko-1 cell proliferation at 24, 48, 72 h in a time- and dose-dependent manner. Chlorambucil of 0, 5, 10, 20 µmol/L increased the apoptotic rate of Jeko-1 cells, upregulated the expression of BAX, procaspase 3, procaspase 8, procaspase 9 and PI3K, increased the phosphorylation of AKT and down-regulated the expression of BCL-2.</p><p><b>CONCLUSION</b>The chlorambucil can induce the apoptosis of mantle cell lymphoma Jeko-1 cells via blocking PI3K/AKT signaling pathway.</p>

Cholorambucil versus cholorambucil plus prednisolone as first-line therapy of chronic lymphocytic leukemia in west of Iran

Chronic lymphocytic leukemia [CLL] has been the most common type of leukemia in adults worldwide, and then more common in the elderly, markedly more common in patients over the age of 65 years. Seventy patients with CLL have referred to Clinic of Hematology-Oncology, Kermanshah, Iran, between Jan 2000 and Jun 2014. We have analyzed age, sex, survival, kind of chemotherapy and type of response in all of the patients with chronic lymphocytic leukemia. Survival curves of complete response patients have compared with partial response, by log-rank test using the Prism 5 GraphPad Software for the five-year period with two years follow up. The mean age of patients was 61.57 +/- 8.88 years that 55.7% were males. Between the 70 patients, 40 patients [57.1%] have started treatment with chlorambucil and 30 patients [42.9%] with chlorambucil plus prednisolone. Among the forty patients that have treated with chlorambucil, overall response rate was 95% that 9 patients [22.5%] had complete response. Among the 30 patients that have treated with chlorambucil plus prednisolone, overall response rate was 96%, that 9 patients [30%] had complete response after six months of treatment. The mean of five-year overall survival for treated patients with chlorambucil and chlorambucil plus prednisolone in the first-line of therapy was 38.5 and 40.5 months, respectively. Combination of prednisolon to chlorambucil has increased survival rate in the patients more than mono-therapy with chlorambucil and also the complete response rate to chlorambucil in West of Iran was better than other areas of world

Efficacy Analysis of MAC Regimen as Salvage Treatment Protocol for Acute Myeloid Leukemia Patients Older Than 55 Years / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of MAC regimen in the treatment of acute myeloid leukemia(AML) patients older than 55 years.</p><p><b>METHODS</b>A total of 33 relapsed or non-remission AML patients older than 55 years were enrolled in this research. MAC regimen was given as the salvage treatment. Complete remission rate(CR), partial remission rate(PR), overall survival(OS), relapse-free survival(RFS) and adverse effect were analysed.</p><p><b>RESULTS</b>CR rate after the salvage therapy with MAC was 51.1%, partial remission (PR) rate was 6.1%, the overall response rate (ORR) was 57.6%, the median OS was 8 months (1.0-66.0 months), the median relapse-free survival (RFS) was 10.1 months (2.3-40.4 months). Mortality related with salvage treatment in 30 days was 9.1%. Low incidence of severe organ damage were found.</p><p><b>CONCLUSION</b>MAC can be used as a relative effective and safe regimen for the salvage treatment of the older AML patients.</p>

Chlorambucil and ascorbic acid-mediated anticancer activity and hematological toxicity in Dalton's ascites lymphoma-bearing mice.

Chlorambucil is an anticancer drug with alkylating and immunosuppressive activities. Considering various reports on the possible antioxidant/protective functions of ascorbic acid (vitamin C), it was aimed at to explore the modulatory effect of ascorbic acid on therapeutic efficacy and toxicity induced by chlorambucil. Dalton’s ascites lymphoma tumor serially maintained in Swiss albino mice were used for the present experiments. The result of antitumor activity showed that combination treatment with ascorbic acid and chlorambucil exhibited enhanced antitumor activity with 170% increase in life span (ILS), which is significantly higher as compared to chlorambucil alone (ILS 140%). Analysis of apoptosis in Dalton’s lymphoma tumor cells revealed a significantly higher apoptotic index after combination treatment as compared to chlorambucil alone. Blood hemoglobin content, erythrocytes and leukocytes counts were decreased after chlorambucil treatment, however, overall recovery in these hematological values was noted after combination treatment. Chlorambucil treatment also caused morphological abnormalities in red blood cells, majority of which include acanthocytes, burr and microcystis. Combination treatment of mice with ascorbic acid plus chlorambucil showed less histopathological changes in kidney as compared to chlorambucil treatment alone, thus, ascorbic acid is effective in reducing chlorambucil-induced renal toxicity in the hosts. Based on the results, for further devel­opment, hopefully into the clinical usage, the administration of ascorbic acid in combination with chlorambucil may be recommended.

Acute lymphoblastic leukemia and chronic lymphocytic leukemia in dogs / 동물의과학연구지

An Australian cattle dog (case 1: 6-year-old castrated male) and a Shih-Tzu dog (case 2: 8-year-old castrated male) were referred to the Gyeongsang Animal Medical Center due to anorexia and depression. Physical examinations, complete blood counts, serum chemical analysis, radiography, ultrasonography, and bone marrow biopsy were performed. Upon physical examinations of cases 1 and 2, enlargement of superficial lymph nodes was not identified. Hematologic findings in these dogs included leukocytosis with severe lymphocytosis, anemia, and thrombocytopenia. Upon radiography, both dogs showed splenomegaly. Upon examination of a peripheral blood smear in case 1, immature lymphoid cells, featuring decreased nuclear chromatin condensation and nuclear pleomorphism, were present. Biopsy samples of the bone marrow in case 1 revealed hypercellularity as well as a large number of immature lymphoblastic cells similar in shape to cells in the peripheral blood. The characteristic morphological features of peripheral blood and bone marrow samples in case 2 were small lymphocytes. Thus, the dogs were tentatively diagnosed with acute lymphoblastic leukemia (ALL) and chronic lymphocytic leukemia (CLL), respectively. After diagnosis, the CLL patient was administered chlorambucil and prednisolone therapy. Due to its similarity to human leukemia, the canine leukemia model provides a valuable model for research into human leukemia.

Chronic Lymphocytic Leukemia-Era of Targeted Therapy / 대한내과학회지

Chronic lymphocytic leukemia (CLL) is a unique indolent B-cell leukemia which is rare in Korea. Many patients with early stage CLL do not require immediate treatment, while those with advanced stage or symptoms need systemic chemotherapy. As our understanding about the pathophysiology of CLL increases, significant advances have been achieved in the treatment of this disease. Modern molecular genetics have been revealing remarkable heterogeneity of various genetic alterations and the corresponding prognostic stratification in CLL. The treatment of CLL had been developed from nitrogen mustard alkylating agent like chlorambucil to combination therapy including purine analogues like pentostatin and fludarabine until early 2000s. Since the introduction of targeted agent like anti-CD20 and anti-CD52 monoclonal antibodies in the treatment of CLL, the treatment outcome of CLL has leaped further. In conclusion, one of the current standard regimens in patients with untreated CLL is the combination of rituximab, cyclophosphamide and fludarabine. We recently passed the entrance for the era of targeted therapy, and are exploring various new agents and their combinations.

Aspectos clinicoepidemiológicos de la leucemia linfoide crónica / Clinical epidemiological aspects of chronic lymphoid leukaemia

Se hizo un estudio descriptivo y retrospectivo de 71 pacientes con leucemia linfoide crónica, atendidos en el Servicio de Hematología del Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, desde enero del 2001 hasta noviembre del 2006, con vistas a identificar algunas variables clinicoepidemiológicas en ellos, mostrar las terapéuticas más empleadas, así como evaluar la supervivencia, las principales causas de la entidad clínica y la mortalidad. En la serie predominaron los ancianos, el sexo masculino y la categoría de alto riesgo correspondiente a los estadios avanzados. El esquema terapéutico de clorambucil y prednisona fue el más empleado, con buenos resultados en la mayoría de los integrantes de la casuística. La supervivencia de los pacientes, en general, osciló entre 1-5 años, en tanto las muertes ocurridas fueron causadas por progresión de la enfermedad, procesos infecciosos respiratorios, transformación prolinfocítica, segundas neoplasias y accidentes vasculares encefálicos.

A descriptive and retrospective study of 71 patients with chronic lymphoid leukemia, attended at the Hematology Service from Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba was carried out from January, 2001 to November, 2006, in order to identify some clinical epidemiological variables on them, to show the therapeutical variables more used, as well as to assess survival, mortality, and the main causes of the clinical entity. Elderly, male sex, and high risk category related to advanced stage were predominant in the series. The therapeutical schedule of chlorambucil and prednisone was the most used, achieving good results in the majority of the case material. The survival of patients, in general, ranged among 1-5 years, whereas deaths occurred due to disease progression, infectious respiratory processes, pro-lymphocytic transformation, second neoplasias, and strokes.

Chlorambucil and cyclosporine A in Brazilian patients with Behçet's disease uveitis: a retrospective study / Clorambucil e ciclosporina A em pacientes brasileiros com doença de Behçet e uveíte: estudo retrospectivo

PURPOSE: To assess the efficacy and side effects of immunosuppressive therapy in patients with Behçet's disease uveitis. METHODS: A nonrandomized retrospective case-series study analyzed data from 22 patients with Behçet's disease uveitis, from a single Uveitis Service, São Paulo, Brazil (period 1978-2007), under systemic chlorambucil and/or cyclosporine A, for at least 6 months with a minimum one-year follow-up. Drug efficacy was measured by reduction in relapse rate and reduction of prednisone dose. RESULTS: Patients (10M/12F) mean age was 29 (range 10-43) years-old at the onset of uveitis. The median duration of followup was 11 (range 1-29) years-old. Chlorambucil (2-6 mg/day) was used in 13 patients and cyclosporine A (3-5 mg/kg/day) in 9 patients at initiation. Drugs were switched because of no effectiveness or side-effects. Chlorambucil was effective in 78.5 percent (11/14) and induced disease remission in 43 percent (6/14) of patients, whereas cyclosporine A was effective in 57 percent (8/14) of patients. Chlorambucil and cyclosporine A were discontinued due to side effects in 21 percent (leucopenia) and in 57 percent of patients (nephrotoxicity, 36 percent and gastrointestinal complications, 21 percent), respectively. No case of late malignancy was observed. 36 percent (16/44) of eyes had final visual acuity <0.1, among which 69 percent (11/16) had already this visual acuity at the first visit. CONCLUSION: This study reiterates previous data that chlorambucil can induce long-term remission of Behçet's disease uveitis, whereas cyclosporine is effective but side effects limit its use. Chlorambucil therapy may still be a reasonable option in patients with intractable, sight-threatening Behçet's disease uveitis.

OBJETIVOS: Avaliar a eficácia e efeitos colaterais da terapia imunossupressora em pacientes com uveíte associada à doença de Behçet. MÉTODOS: Estudo retrospectivo não randomizado no qual são analisados dados de 22 pacientes com uveíte associada à doença de Behçet que utilizaram clorambucil e/ou ciclosporina A sistêmica por período mínimo de 6 meses, acompanhados pelo período mínimo de 1 ano, num único Serviço de Uveíte, São Paulo, Brasil (período 1978-2007). A eficácia do tratamento foi avaliada pela redução no número de recidivas da inflamação e pela redução na dose diária de prednisona. RESULTADOS: Vinte e dois pacientes (10 M/12 F), com idade média de 29 (variação 10-43) anos, apresentaram tempo mediano de acompanhamento de 11 (variação 1-29) anos. Clorambucil (2-6 mg/dia) foi utilizado em 13 pacientes e ciclosporina A (3-5 mg/kg/dia) em 9 pacientes como droga de primeira escolha. As drogas foram substituídas quando não eficazes ou não toleradas devido a efeitos colaterais. Clorambucil foi eficaz em 78,5 por cento (11/14) e induziu remissão da doença em 43 por cento (6/14) dos pacientes; ciclosporina foi eficaz em 57 por cento (8/14) dos pacientes. Clorambucil e ciclosporina A foram suspensos devido a efeitos colaterais em 21 por cento (leucopenia) e em 57 por cento (nefrotoxicidade, 36 por cento e complicações gastrointestinais, 21 por cento) dos pacientes, respectivamente. Nenhum caso de malignidade foi observado. 36 por cento (16/44) olhos tiveram acuidade visual final <0,1, sendo que 69 por cento (11/16) já apresentavam esta acuidade na primeira consulta. CONCLUSÃO: Este estudo reforça dados de literatura que o clorambucil induz remissão da doença de Behçet enquanto a ciclosporina A é efetiva, porém efeitos colaterais limitam o seu uso. A terapia com clorambucil pode ser uma opção a ser considerada em pacientes com uveíte refratária associada à doença de Behçet.

Options of the treatment in steroid resistant nephrotic syndrome in children in central child teaching hospital-Baghdad

The therapy of steroid resistant nephritic syndrome [SRNS] is still a matter of controversy. To assess the options of treatment in SRNS. A retrospective study to 50 patient randomly selected in the Central Child Teaching Hospital during study period from Jan. 2006 till July 2008. The patient age was between 6 months - 18 years. All patients who had failed to achieve an improvement in proteinuria after minimum of 4 weeks [up to 8 weeks] of prednisolone [PDN] in a dosage 2 mg/kg/day were taken. Only the patients with idiopathic nephritic syndrome [45 patients] were involved in the study but the patients with secondary nephritic syndrome and congenital neprosis were excluded from the study. Each patient were individualized to the type of pathology and to the type of medication used. Forty five patients were included in the study, the age range between 6 months -18 years. Twenty eight patients were male and 17 were female, M: F ration 1.64: 1 regarding the type of pathology, 20 patients with focal segmental glomerulosclerosis [FSGS], 11 patients with minimal change nephritic syndrome [MCNS], 8 patients with diffuse mesagnial proliferation [DMP] and 6 patients were unknown biopsy [not down biopsy]. The drugs that used were methyl prednisolone [MP] in 17 patients. Every other day steroid [EODS] in 10 patients, cyclosporine A [CsA] plus EODS in 16 patients [10 patients as first option and 6 patients as second option, cyclophosphamide [CYS] used in 8 patients [6 patients as first option and 2 patients as second option] and chlorambucil wee used in 2 patients only. The response was higher in patients who received EODS [50%], followed by the patients who received CsA plus EODS [25%] then the patients who received MP [23.5%] and the patients who received CYS [12.5%] and chlorambucil [zero%]. The response to treatment was higher in females than males, 11 out of 28 males [39.28%] responded to treatment while 7 of 17 female [41.17%] responded to treatment. The patients with early presentation responded to treatment higher than those with late presentation, so 12 of 19 patients [63.15%] presented early while 10 of 26 patients [38.46%] presented ate. According to histopathology, the patients with unknown etiology had higher rate of response, 3 out of 6 patients [50%] responded to treatment followed by 7 of 20 [35%] patients with FSGS, then 2 of 8 [25%] patients with DMP, then 2 of 11 [18.18%] patients with MCNS. The drugs used are the common drugs and EODS is preferable type of medication used in SRNS

Health Effects of Chemicals used in hospitals among Healthcare Workers

Healthcare workers are exposed to a variety of chemical agents used in many different areas and purposes. The chemicals could cause health problems to healthcare workers using them. Glutaraldehyde is a kind of disinfectant and used for endoscopes, catheters, and many kinds of operating apparatus. It may cause allergic contact dermatitis. Formaldehyde is another disinfectant and can be used for fixing tissues. Formaldehyde was classified to a Group 1 carcinogen by IARC and it may cause lung or nasal cancer. Ethylene Oxide gas is the most popular disinfectant these days and may be applied to many health care sets or linens. EO gas may cause allergic contact dermatitis and breast cancer or leukemia. It is also classified as Group 1 carcinogen despite limited evidence for human cancers. Anesthetics are related to genotoxicities, sister chromatid exchange, and might be related to spontaneous abortion, stillbirth or birth defects. Some of the anti-neoplastic drugs such as Busulfan, Chlorambucil, cyclophosphamide, melphalan are Group 1 carcinogens. They could cause nausea, pruritus, or decreasing leukocytes or platelets. Other miscellaneous chemical agents are heavy metals such as elementary mercury or lead and organic solvents such as toluene, xylene and acetone. Although some of these chemical agents including EO gas have occasionally exceeded to permissible level, air levels of most above chemicals in Korean hospitals were relatively low. However, we have to make every effort to reduce the exposure level of these chemicals.

Pneumonitis due to cytomegalovirus in an immunocompromised patient

Cytomegalovirus [CMV] pneumonia is one of the most important infections in immunocompromised host. Immunosuppressive therapy plays a major role in reactivation of CMV. The patient was a 56-year old lady, known case of chronic lymphocytic leukemia [CLL], had been taking prednisolone and chlorambucil, who presented with dyspnea and productive cough. After bronchoalveolar lavage [BAL], transbronchial lung biopsy [TBLB] and CT-guided biopsy, CMV pneumonia was diagnosed. CMV should be suspected as a cause of pneumonia in immunocompromised patient and diagnosis may require invasive procedures

Linfoma renal primario en paciente con gammapatía monoclonal IgM / Primary renal lymphoma in a patient with IgM monoclonal gammapathy

Son infrecuentes los casos de linfoma renal primario, ya que la afectación renal por un proceso linfoproliferativo es, por lo general, secundaria a una enfermedad sistémica. Presentamos el caso de una paciente mujer de 48 años que acude por dolor lumbar y masa abdominal. Después de realizar estudios (TC), se práctica nefrectomía cuyo resultado anatomopatológico fue de linfoma no-hodking B primario renal. Asimismo el paciente presentaba una gammapatía monoclonal IgM asociada, por lo que precisó tratamiento quimioterápico sistémico. Realizamos una revisión bibliográfica centrándonos en los criterios diagnósticos y terapéuticos actuales.

Reports on primary renal lymphoma are scarce in the urological literature, the most part f them are secondary on a lymphomatous infiltration of the kidneys. We report the case of a 48 year old women with lumbar pain and adominal mass. After radiological studies (CT), we practise nephrectomy with a pathological result of a non-hodking B primary lymphoma. The patient present a IgM monoclonal gammapathy who need complementary treatment with chemotherapy. A literature review on currently recommended diagnostic and treatment practices in presented.

Small lymphocytic lymphoma that developed in a patient with long-standing cold agglutinin disease / 대한내과학회지

Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia (AIHA) induced by cold antibodies. CAD constitutes 16~32% of AIHA cases and the causative cold autoantibodies are commonly specific against blood group I or i antigens. CAD is closely related to lymphoid neoplasia and infections such as Mycoplasma and Epstein-Barr virus (EBV). Primary CAD has a tendency to develop into malignant lymphoma over its course. We experienced a case of small lymphocytic lymphoma during the treatment of primary CAD over 11 years. The patient was a 67-year-old man who complained of a neck mass. Node biopsy revealed diffuse neoplastic proliferation of small monotonous lymphoid cells with polygonal or oval nuclei;these cells were CD20- and CD5-positive. Follow-up chest and neck computed tomography after treatment with chlorambucil and prednisolone showed partial resolution and stable disease status for over 2 years. We report a case of small lymphocytic lymphoma that developed in a patient with long-standing cold agglutinin disease for 11 years and review the literature.

Acometimento pulmonar na doença de Behçet: uma boa experiência com o uso de imunossupressores / Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy

OBJETIVO: A doença de Behçet (DB) representa uma patologia sistêmica, cuja sobrevida se relaciona com a presença de acometimento pulmonar. Entretanto, sugere-se que pacientes com diferentes tratamentos podem apresentar diferentes prognósticos. O objetivo deste estudo foi avaliar a evolução clínica e tomográfica, bem como a sobrevida deste pacientes com acometimento pulmonar relacionado à DB acompanhados em nosso serviço. MÉTODOS: Uma análise retrospectiva de nossa experiência com pacientes com acometimento pulmonar relaionado a DB acompanhados de 1 de Janeiro de 1988 a 30 de Abril de 2006. Os dados clínicos, radiológicos, terapêuticos e de sobrevida foram obtidos dos prontuários médicos. RESULTADOS: Foram identificados 9 pacientes, com idade média de 34 ± 11,5 anos, sendo 7 deles do sexo masculino. Os achados radiológicos foram aneurisma de artéria pulmonar (AAP) em 8 pacientes, embolia pulmonar em 3 (resultando em uma incidencia de 5,11 casos/100 paciente-anos), hemorragia alveolar em 1 e hipertensão pulmonar em 1 de 9 doentes. O tratamento consistiu-se de prednisona mais clorambucil (ou ciclofosfamida ou micofenolato de mofetil) em todos os 9 pacientes, com resolução total ou parcial dos AAP. O paciente com AAP e hipertensão pulmonar também recebeu sildenafil e warfarina, com boa resposta clínica e tomográfica. A sobrevida de nossos pacientes foi de 88,8 por cento em 3 e 5 anos, com acompanhamento médio de 6,52 anos. CONCLUSÕES: Pacientes com acometimento pulmonar relacionado à DB podem apresentar boa sobrevida com tratamento imunossupressor, e a DB deve ser lembrada como uma possível causa de hipertensão pulmonar e hemorragia alveolar.

OBJECTIVE: Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement. METHODS: A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts. RESULTS: We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 ± 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8 percent, as was the five-year survival rate. CONCLUSIONS: Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.

Hepatitis B virus reactivation during chlorambucil and prednisolone treatment in an HBsAg-negative and anti-HBs-positive patient with B-cell chronic lymphocytic leukemia / 대한간학회지

It is generally accepted that seroconversion of hepatitis B virus (HBV) surface antigen (HBsAg) to an antibody to HBsAg (anti-HBs) indicates clearance of HBV. Here we report a case of severe hepatitis that manifested during chemotherapy in a female patient with chronic lymphocytic leukemia (CLL) who had been initially seronegative for HBsAg and seropositive for anti-HBs. The patient received chlorambucil and prednisolone for the treatment of CLL. After 6 months the serum levels of aminotransferases were increased, and HBsAg and HBV DNA were present in serum. Lamivudine was administered immediately after confirming the HBV reactivation, which considerably improved jaundice and aminotransferase levels after 3 weeks. The patient was able to resume the chemotherapy whilst continuing lamivudine treatment. This case report highlights the need for physicians to be aware of the potential risk of HBV reactivation even in an HBsAg-negative person but with detectable anti-HBc and/or anti-HBs, underscoring the need for future studies that explore the role of antiviral prophylaxis in this setting.

Castleman's disease in a patient with celiac disease.

A 72-year-old man with celiac disease was doing well on gluten-free diet. Five years later, he developed dyspepsia, fever and weight loss. CT scan and laparoscopic biopsies of small mesenteric lymph nodes clinched the diagnosis of Castleman's disease. He was started on chlorambucil; unfortunately he died few months after the diagnosis was made.

[B-cell chronic lymphocyte leukemia [B-CLL]]

The past two decades have seen a major progress in the filed of pathphysiology and treatment of chronic lymphocytic leukemia [CLL]. New findings suggested that CLL comprises two separate types of tumor with different prognosis outcome. CLL formerly was considered an incurable "oldman's disease" caused by slowly accumulating, incompetent lymphocytes. Because elderly patients were expected to die with CLL rather than from it, the mainstay of therapy was to palliate symptoms with oral, alkylating drugs, such as chlorambucil. However, treatment with such drugs not only could not cure the disease, but also could shorten survival if given to patients with early-stage disease. This article summarizes some of the recent advances that have shaped a new way of thinking about this disease including epidemiology, diagnosis and prognostic factors. Treatment strategies, chemotherapy, monoclonal antibodies have been briefly discussed