Anemia hemolítica autoimune na doença de Castleman multicêntrica: relato de caso / Autoimmune hemolytic anemia in multicentric Castleman's disease: case report

A doença de Castleman é um distúrbio linfoproliferativo raro, podendo se manifestar sob a forma de massas localizadas ou como doença multicêntrica. A doença de Castleman multicêntrica é caracterizada por adenopatias generalizadas, visceromegalias, manifestações autoimunes e infecções recorrentes. Este artigo apresenta o relato de caso de anemia hemolítica autoimune por anticorpos quentes em paciente com doença de Castleman multicêntrica. Resposta eficaz foi obtida com uso de corticoterapia sistêmica e tocilizumabe.

Castleman disease is a rare lymphoproliferative disorder that can manifest as localized masses or as multicentric disease. Multicentric Castleman disease is characterized by generalized adenopathies, visceromegaly, autoimmune manifestations, and recurrent infections. This article presents the case report of a patient with multicentric Castleman's disease and autoimmune hemolytic anemia by warm antibodies. Effective response was obtained with systemic corticotherapy and tocilizumab.

Clinicopathological characteristics of natural killer cell enteropathy: report of two cases and review of literature / 中华病理学杂志

Objective: To study the clinicopathological and genetic features of natural killer (NK)-cell enteropathy for better understanding of this rare disease and prevention of its misdiagnosis. Methods: Two cases of NK-cell enteropathy were diagnosed in the First Affiliated Hospital of Zhengzhou University, China from October 2017 to February 2021. The clinical characteristics, morphology, immunohistochemistry, Epstein-Barr virus-encoded RNA (EBER) in situ hybridization and T cell receptor gene rearrangement were analyzed. The patients were followed up by a telephone interview. Results: The patients were both male, aged 40 and 28 years, respectively. Both patients were admitted to the hospital for an annual checkup without obvious gastrointestinal symptoms. The endoscopy showed that the gastric body of case 1 had a mucosal bulge, small area of congestion and erosion, while the rectum of case 2 had congestion and erosion. Microscopically, the lesions of the 2 cases were relatively limited. Many lymphoid cells infiltrated within the lamina propria of the mucosa and into the muscularis mucosa in case 2. In case 1, the glands were reduced in the lesion, and the glandular cavity was slightly compressed and deformed. There was no infiltration or destruction of the glands in either case. Lymphoid cells were atypical, with medium-to-large cell sizes. Their cytoplasm was medium-to-slightly abundant and appeared eosinophilic or translucent. In case 2, characteristic eosinophilic granules were seen in the cytoplasm of a few cells. The nuclei in both cases were round, oval and irregular, with fine chromatin, inconspicuous nucleoli, and no mitotic figures were noted. Necrosis was seen in case 1 while both cases had no central growth or destruction of blood vessels. Immunophenotyping showed that CD56, granzyme B and TIA-1 were positive in both cases, part of the cells was CD3-positive, and some cells were weakly CD4-positive in case 2. The CD5, CD8, CD30, ALK and B-lineage markers (CD20, CD79α) were all negative. The Ki-67 proliferation index was about 60% and 30%, respectively. Both cases were EBER negative. TCR gene rearrangement was polyclonal. Follow-up showed that none of the 2 patients had any special treatments and stayed well. Conclusions: NK-cell enteropathy is rare, with biological behaviors similar to benign tumors, and occasional recurrence. Its histology and immunophenotype are easily confused with NK/T cell-derived lymphomas. Combination of its unique endoscopic features, EBER negativity, polyclonal TCR gene rearrangement and good prognosis can confirm the diagnosis and avoid misdiagnosis and overtreatment.

Linfoma primario no Hodgkin de mama / Primary Non-Hodgkin's Breast Lymphoma

Introducción: El cáncer de mama se ha convertido en un verdadero desafío a la calidad y expectativa de vida de la población mundial, con un aumento considerable en su incidencia y prevalencia. Cuba cuenta, desde 1987, con un Programa de Control de Cáncer Mamario. Objetivo: Ejemplificar el estudio de un caso con linfoma no Hodgkin primario de la mama, su cuadro clínico y los medios diagnósticos utilizados. Caso clínico: Paciente femenina de 70 años de edad, color de la piel negra, que acudió al Consultorio Médico de la Familia por presentar malestar general, molestias y aumento de volumen en la mama derecha. Se le remitió a consulta de cirugía y se le realizaron estudios complementarios. Se detectó nódulo de mediana densidad, contornos parcialmente definidos, distorsión del tejido vecino, otros nódulos de menos tamaño y densidad en el cuadrante inferior interno, calcificaciones vasculares en ambas mamas. Una biopsia corrobora linfoma no Hodgkin difuso de células grandes de alto grado. Conclusiones: El linfoma primario no Hodgkin de mama es muy raro y de difícil diagnóstico clínico porque no se dispone de signos, síntomas o criterios de imagen específicos para ello. El autoexamen de mama continúa siendo el principal método de diagnóstico del cáncer de mama y aunque el examen clínico, el ultrasonido de mama y la mamografía apoyan la presunción, es la biopsia quien solo brinda la confirmación diagnóstica precisa(AU)

Introduction: Breast cancer has become a real challenge to the quality of life and to life expectancy of the world population, with a considerable increase in its incidence and prevalence. Cuba has, since 1987, a breast cancer control program. Objective: To present a case with primary non-Hodgkin's lymphoma of the breast in a 70-year-old patient, its clinical picture and the diagnostic means used. Clinical case: 70-year-old female patient, with black skin, who came to the family medical office due to malaise, discomfort and increased volume in her right breast. She was referred to surgery and complementary studies were performed. Nodule of medium density was identified, with partially defined contours, distortion of the neighboring tissue, together with other nodules of less size and density in the lower internal quadrant, and vascular calcifications in both breasts. A biopsy confirms diffuse high-grade large-cell non-Hodgkin's lymphoma. Conclusions: Primary non-Hodgkin's lymphoma of the breast is very rare and difficult to diagnose clinically because there are no specific signs, symptoms or imaging criteria for it. Breast self-examination continues to be the main method of diagnosis for breast cancer and, although clinical examination, breast ultrasound and mammography support such diagnostic presumption, it is the biopsy that only provides the precise diagnostic confirmation(AU)

Leucemia linfoide crónica: aspectos citogenéticos y moleculares / Chronic Lymphoid Leukemia: cytogenetic and molecular aspects

Introducción: La leucemia linfoide crónica es un trastorno linfoproliferativo caracterizado por la acumulación de linfocitos pequeños de aspecto maduro en sangre periférica, médula ósea y tejidos linfoides con un período de vida prolongado. Presenta una gran variabilidad clínica y genética. Objetivo: Describir los aspectos citogenéticos y moleculares de la leucemia linfoide crónica. Métodos: Se realizó revisión de la literatura en inglés y español, a través del sitio web PubMed y el motor de búsqueda Google académico, de artículos publicados en los últimos 5 años. Se hizo un análisis y resumen de la bibliografía revisada. Desarrollo: En la leucemia linfoide crónica están presentes alteraciones citogenéticas frecuentes como la deleción de los cromosomas 13q, 11q y 17p, así como la trisomía 12, que unido al conocimiento del estado mutacional del gen de la región variable de la cadena pesada de la inmunoglobulina, y otras mutaciones somáticas en diferentes genes, así como a variables clínicas y de laboratorio permiten la estratificación pronóstica de los pacientes. Conclusiones: El diagnóstico a través de los estudios citogenéticos convencionales estimulados con mitógenos, la hibridación in situ por fluorescencia y la secuenciación génica permite una mayor comprensión de la biología de la enfermedad, así como tomar decisiones terapéuticas más personalizadas(AU)

Introduction: Chronic B lymphoid leukemia is a lymphoproliferative disorder characterized by the accumulation of small, mature-looking lymphocytes in peripheral blood, bone marrow and lymphoid tissues with a long life span. It has great clinical and genetic variability. Objective: To describe the cytogenetic and molecular aspects of the disease. Methods: A review of the literature in English and in Spanish was carried out, in the PubMed website and using the search engine of Google Scholar, for articles published in the last five years. We performed analysis and summary of the reviewed bibliography. Development: In chronic lymphoid leukemia, frequent cytogenetic alterations are present such as deletion of chromosomes 13q, 11q and 17p, as well as trisomy 12, which together with the knowledge of the mutational status of the gene for the variable region of the immunoglobulin heavy chain and other somatic mutations in different genes, as well as clinical and laboratory variables allows prognostic stratification of patients. Conclusions: Diagnosis through conventional mitogen-stimulated cytogenetic studies, fluorescence in situ hybridization and gene sequencing allow a better understanding of the biology of the disease, as well as making more personalized therapeutic decisions(AU)

Pérdida de agudeza visual progresiva en paciente con proptosis. Caso clínico y revisión de la literatura / Progressive visual impairment in a patient with proptosis. Case report and review of the literature.

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.

Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.

Oral Mucosal Diffuse Large B-cell Lymphoma Caused by Long-term Oral Methotrexate:Report of One Case / 中国医学科学院学报

A case of primary oral mucosal diffuse large B-cell lymphoma(DLBCL)due to long-term use of methotrexate(MTX)for the treatment of rheumatoid arthritis(RA)was admitted to the Department of Hematology,Fujian Medical University Union Hospital.We analyzed and discussed the clinical features,diagnosis and treatment,and prognosis of specific malignant lymphoma induced by MTX in this RA patient.Our purpose is to improve the awareness and knowledge of other iatrogenic immunodeficiency-associated lymphoproliferative disorders of clinicians and pathologists.This study provides a new reference for the clinical diagnosis and treatment of MTX-associated DLBCL.

Trastornos linfoproliferativos en una cohorte de pacientes adultos con trasplante hepático atendidos en un hospital de referencia en Bogotá, Colombia / Post-transplant lymphoproliferative disorders in a cohort of adult patients with a liver transplant from a reference hospital in Bogotá, Colombia

Introducción. Los trastornos linfoproliferativos después de un trasplante se caracterizan por la proliferación descontrolada de linfocitos como consecuencia del tratamiento inmunosupresor posterior a este. Objetivo. Caracterizar clínica y patológicamente los casos de trastornos linfoproliferativos después de trasplante (Post-Transplant Lymphoproliferative Disorders, PTLD) en una cohorte de pacientes adultos con trasplante de hígado atendidos a lo largo de 15 años en el Hospital Universitario Fundación Santa Fe de Bogotá. Materiales y métodos. Se hizo un estudio observacional retrospectivo a partir de la revisión de las bases de datos de la Unidad de Trasplante Hepático y del Departamento de Patología del Hospital en busca de los casos de PTLD diagnosticados durante el periodo de estudio. Se recolectó la información epidemiológica, clínica y patológica, y se adelantaron los análisis estadísticos. Resultados. Durante el periodo de estudio, hubo 572 pacientes con trasplante de hígado, la incidencia de trastornos linfoproliferativos fue de 2,44 %, el 79 % en hombres, y la edad promedio en el momento del diagnóstico fue de 62,5 años. El 71 % de los casos se presentó durante los primeros 12 meses después del trasplante y el mismo porcentaje fue seropositivo para el virus de Epstein-Barr (EBV). El fenotipo patológico más frecuente fue el monomorfo y la mayoría de los tumores se detectaron en el hilio hepático. La supervivencia al año fue del 50 %. Conclusiones. Llamó la atención el alto porcentaje de casos de presentación temprana, así como la gran frecuencia de seropositividad para el EBV tanto en los donantes como en los receptores. Deben adelantarse estudios más detallados para una mejor comprensión de esta enfermedad en el país. Este es el primer análisis clínico y patológico de PTLD en pacientes con trasplante de hígado adelantado en Colombia hasta la fecha.

Introduction: The post-transplant lymphoproliferative disorders (PTLD) are characterized by an uncontrolled pathological lymphoid proliferation as a consequence of transplant immunosuppression therapy. Objective: To characterize the clinical and pathological characteristics of PTLD in a cohort of adult patients with liver transplant during a 15 year period at the Hospital Universitario Fundación Santa Fe de Bogota. Materials and methods: We conducted an observational retrospective study by searching for the PTLD cases diagnosed during the study period in the databases of the Liver Transplantation Unit and the Pathology Department. We collected the epidemiological, clinical, and pathological information and performed the corresponding statistics analyses. Results: During the research period, 572 patients were transplanted; the incidence of PTDL was 2.44%; 79% of them were man and the average age at the time of diagnosis was 62.5 years; 71% of the cases were diagnosed during the first year after the transplant and the same percentage EBV-seropositive patients. The most frequent pathological phenotype was monomorphic and the majority of tumors was detected in the hepatic hilum. The one-year survival was 50%. Conclusion: The high proportion of early cases and the high frequency of Epstein-Barr virus seropositivity both in donors and receptors drewour attention. More studies are necessary to have a better understanding of this condition in Colombia. This is the first PTLD clinical and pathological analysis in liver-transplant patients from Colombia to date.

Polyclonal CD5+/CD19+ B1a lymphocytes after allogeneic stem cell transplantation: a potential diagnostic pitfall

In adults, B-lymphocytes comprise approximately 10% of circulating lymphocytes. The majority of peripheral B cells are B2 cells ("Mature" B-cells), which function as part of the humoral adaptive immune system. B1 cells ("Innate-like" B cells) are another sub-class of B lymphocytes, considered as innate immune cells with a characteristic phenotype (CD20+, CD27+, CD43+, CD70-, CD11b+, sIgM++, sIgD+) which can be divided into two subtypes; B1a (CD5+): spontaneously produce broadly reactive natural IgM, and B1b (CD5-): can generate T-cell independent, long-lasting IgM. There is very limited data available, indicating a correlation between allogeneic bone marrow transplantation and an increase in B1a cells. Here we present a case of a 17-year-old female with homozygous sickle cell disease (HbSS disease) who underwent hematopoietic stem cell transplant (HSCT). Approximately seven months post-transplant, she was found to have 16% immature mononuclear cells on complete blood count (CBC)-differential report. A follow-up peripheral blood flow cytometry showed that these cells were polyclonal CD5+/CD20+ B-cells, and comprised 66% of lymphocytes. Further workup and follow up failed to reveal any lymphoproliferative disorders. It is important not to misdiagnose these cells as an atypical CD5+ lymphoproliferative disorder. The presence of B1a cells has not been widely reported in non-neoplastic post-stem cell transplanted patients. This case also adds to and expands our knowledge regarding the presence of increased circulating B1a cells after stem cell transplant in a patient with no history of hematological malignancy.

Aplicaciones de la citometría de flujo en el estudio de los síndromes linfoproliferativos crónicos / Applications of flow cytometry in the study of chronic lymphoproliferative syndromes

Introducción: La citometría de flujo es una técnica de avanzada, objetiva y altamente sensible que permite el análisis y la cuantificación simultánea de múltiples parámetros celulares; es muy utilizada en el estudio de las hemopatías malignas. En los últimos años, ha demostrado ser de gran utilidad en la identificación y la caracterización inmunofenotípica de los síndromes linfoproliferativos crónicos. Estos constituyen un grupo heterogéneo de enfermedades caracterizadas por la expansión monoclonal de células linfoides de aspecto maduro. Objetivos: Analizar los aspectos generales de la aplicación de la técnica de citometría de flujo al estudio y clasificación inmunofenotípica de los síndromes linfoproliferativos crónicos. Métodos: Se realizó una investigación bibliográfica-documental acerca del tema. Se consultaron las bases de datos de SciELO y Pubmed. Análisis y síntesis de la información: Se describen los aspectos técnicos de la citometría de flujo, desde la obtención y procesamiento de las muestras hasta la generación del informe por el citómetro; así como la aplicación de la técnica a la caracterización inmunofenotípica de los síndromes linfoproliferativos crónicos. La citometría de flujo multiparamétrica se ha convertido en uno más de los métodos diagnósticos de este síndrome. Uno de los principales objetivos del estudio inmunofenotípico por citometría de flujo consiste en descartar si esa población de células B es clonal o no. Conclusiones: La citometría de flujo permite el análisis, la interpretación y la clasificación inmunofenotípica de los síndromes linfoproliferativos crónicos. Es una herramienta útil en las que se apoya el diagnóstico y el seguimiento de estos pacientes(AU)

Introduction: Flow cytometry is an advanced, objective and highly sensitive technique that allows simultaneous quantification and analysis of multiple cellular parameters. It is widely used in the study of malignant hemopathies. In recent years, it has proved very useful in the identification and immunophenotypic characterization of chronic lymphoproliferative syndromes. These conditions belong to a heterogeneous group of diseases characterized by monoclonal expansion of mature lymphoid cells. Objectives: To analyze the general aspects of flow cytometry application to the study and immunophenotypic classification of chronic lymphoproliferative syndromes. Methods: A bibliographic-documentary research about the topic was carried out. We consulted the SciELO and Pubmed databases. Information analysis and synthesis: The technical aspects of the flow cytometry are described, from obtaining and processing the samples to the cytometer's generating the report; as well as the technique's application to the immunophenotypic characterization of chronic lymphoproliferative syndromes. Multiparametric flow cytometry has become one of the diagnostic methods for this syndrome. One of the main objectives of the immunophenotypic study by flow cytometry is to rule out whether this population of B cells is clonal or not. Conclusions: Flow cytometry allows the analysis, interpretation and immunophenotypic classification of chronic lymphoproliferative syndromes. It is a useful tool that supports the diagnosis and monitoring of these patients(AU)

Two cases of rare HHV8-driven intravascular lymphoma with synchronous Kaposi sarcoma, both diagnosed at autopsy in renal transplant recipients

We present the first report of two rare yet remarkably similar autopsy cases of Kaposi sarcoma (KS) and intravascular human herpesvirus 8 (HHV8) positive lymphoproliferative disorder in renal transplant patients. It is well established that HHV8 infection causes Kaposi sarcoma (KS). More recently, it is recognized that HHV8 is also related to several lymphoproliferative conditions. These are poorly characterized and often difficult to diagnose. In both cases described herein, the diagnoses of multifocal hepatic KS and intravascular HHV8 positive (EBV negative) systemic diffuse large B-cell lymphoma, NOS were made at autopsy. Given the findings we describe in cases with fatal outcomes, we discuss the implications of HHV8 screening in solid allograft recipients.

Neoplasia blástica de células dendríticas plasmocitoides variante leucemia aguda: reporte de un caso / Blastic plasmacytoid dendritic cell neoplasm variant acute leukemia: case report

RESUMEN La neoplasia blástica de células dendríticas plasmocitoides (NBCDP) es una malignidad hematológica poco frecuente y generalmente agresiva, por lo cual se requiere su reconocimiento precoz. A continuación, se describe el curso clínico prolongado de un paciente masculino de 60 años con NBCDP procedente de Venezuela, en cuyos hallazgos más relevantes destacó la presencia de lesiones cutáneas, organomegalias, infiltración de la médula ósea y del sistema nervioso central. Posterior al diagnóstico se indicó quimioterapia sistémica, no obstante, el paciente falleció por complicaciones respiratorias durante la fase de inducción del tratamiento. En esta enfermedad es necesario establecer el diagnóstico diferencial con trastornos linfoproliferativos, leucemias linfoides y mieloides agudas, constituyendo el análisis morfológico de las células neoplásicas un aspecto importante para una adecuada orientación diagnóstica.

ABSTRACT Blastic plasmacytoid dendritic cell blast neoplasm (BPDCN) is a rare and generally aggressive hematologic malignancy, requiring early recognition. Below is a description of the prolonged clinical course of a 60-year-old male patient with BPDCN from Venezuela, whose most relevant findings highlighted the presence of skin lesions, organomegaly, infiltration of the bone marrow and central nervous system. Systemic chemotherapy was prescribed after diagnosis; however, the patient died of respiratory complications during the induction phase of treatment. In this disease, it is necessary to establish the differential diagnosis with lymphoproliferative disorders, acute lymphoid and myeloid leukemias. The morphological analysis of neoplastic cells is, thus, an important aspect toward proper diagnostic guidance.

Case for diagnosis. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Abstract: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare disease, with an indolent evolution and benign course. The classic presentation is a solitary nodule on the face or trunk. The disorder's rarity and clinical and histopathological characteristics, can make the diagnosis difficult. We present the case of a 36-year-old Caucasian woman with a purplish erythematous nodule, hardened, shiny, asymptomatic, on the left nasal ala, which had grown progressively for 45 days. Histopathological examination and immunohistochemistry panel demonstrated alterations consistent with primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. There was complete remission of the condition within 60 days of treatment with potent occlusive corticosteroids.

Treatment of primary cutaneous anaplastic large cell lymphoma

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.

Albumin-to-Fibrinogen Ratio as an Independent Prognostic Parameter in Untreated Chronic Lymphocytic Leukemia: A Retrospective Study of 191 Cases / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Chronic lymphocytic leukemia (CLL) is one of the most frequent type of B-cell chronic lymphoproliferative disorders and chronic inflammation takes part in the development of CLL. However, there has been no valid immune biomarker to predict the prognosis of untreated CLL patients. MATERIALS AND METHODS: In this retrospective study, we analyzed the clinical correlations and prognostic value of albumin-to-fibrinogen ratio (AFR) detected at diagnosis in 191 CLL patients. RESULTS: The cut-off value of AFR was 9.7 calculated by X-tile. Patients who were more than 65 years old were often accompanied by low level of AFR (p < 0.001). Survival analysis showed that patients with low level of AFR had shorter overall survival (OS) than patients with high level of AFR (p < 0.001). Multivariate analysis illustrated that AFR had a negative impact on OS (p=0.003) and was independent of parameters involved in CLL international prognostic index and other prognostic markers such as CD38 and ZAP-70. CONCLUSION: These data provide a comprehensive view of AFR and shows that AFR at diagnosis is an adverse prognostic factor in untreated CLL patients.

A Case of Primary Cutaneous CD4-positive Small/Medium T-cell Lymphoproliferative Disorder / 대한피부과학회지

No abstract available.

Clinical Analysis of B-CLPD with Cytopemia as the Predominant Characteristic / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical characteristics and therapeutic responte of patients with B-CLPD mainly manifested as cytopenia, so as to deeply understand this disease.@*METHODS@#The clinical data of 13 B-CLPD patients with hematocytopenia as main manifestation, and the absolute count of lymphocytes＜5×10/L, absence of hepatosplenic lymph-nodes and extramedullary invasion tin our department fron 2003 to 2018 were analyzed retrospectively. The clinical characteristics, therapeutic efficacy and adverse reactions of 3 patients were summarized.@*RESULTS@#The median age of patients was 59 (43-76) years old, the median of lymphocyte was 1.86 (0.69-4.8) ×10/L, the levels of LDH and β2-microglubulin were normal in most patients, the monolineage and multilencage hematopoietic failure of different degrees existed in most all patients. The lymphocyte ratio in patients was 18.5%-94.0%, CD20 was positive in all patients, and yet the CD5-positive and CD-negative existed in 7 and 6 cases respectively. There was no significant difference in ratio of lymphocyte invasion among different immunophemtype. The FISH detection showed that there were no high risk genetic types. 92.3% of patients received rituximab treatment, most of them received chemotherapy of rituximab combined with C0P/CHOP like regimen, only 2 patients received fludarabine for comparatively short course. The analysis indicated that 8 out of 13 patients showed a certain theropeutic efficacy, however the drug-related hematopoietic suppression occurred in both 2 patients treated with fludarabin.@*CONCLUSIONS@#The B-CLPD accompanied with hematocytopenia often displays bone marrow hypohematopoiesis of different degree and easily confuses with the congenital and acquired hemotopoietic faiture diseases. The rituximab treatment may be more appropreate for these patients, but for patients received chemotherapy containing fludarabin, the persistant hematopoietic failure must be especially watched out.

Follicular Bronchiolitis Mimicking Lung Cancer in a Patient with Primary Sjögren's Syndrome

Follicular bronchiolitis (FB) is an uncommon pulmonary lymphoproliferative disorder that is characterized by the presence of peribronchiolar hyperplastic lymphoid follicles with reactive germinal centers. FB could be associated with systemic illnesses including immunodeficiency, infection, and autoimmune diseases. In Korea, a single case of FB with rheumatoid arthritis was recently described but there has been no report on FB associated with other rheumatic diseases. Herein, we describe the first case of FB presenting nodular ground-glass opacities (GGO), which mimicked lung cancer, in patients with primary Sjögren's syndrome (SS). The differential diagnosis of nodular GGO lesions should include FB although it is a rare manifestation in SS patients.

EBV reactivation mimicking a lymphoproliferative disorder associated with ruxolitinib therapy for myelofibrosis

No abstract available.

A case of Castleman disease that improved after kidney transplantation / 대한이식학회지

This is a case of a 56-year-old man with Castleman disease (CD) who improved after kidney transplantation (KTP). CD is an uncommon lymphoproliferative disorder that was found incidentally on biopsy during dialysis in the current patient and was followed up without further treatment. However, the lesion showed improvement after KTP. Therefore, active KTP can be considered even if CD is one of the lymphoproliferative disorders that can occur as a complication after KTP.