Discrepant Results for BRAF V600E Mutation Among Different Specimens in a Case of Hairy Cell Leukemia

No abstract available.

Rituximab, 2a línea de tratamiento en leucemia de células vellosas / Rituximab 2nd line of treatment for hairy cell leukemia

La leucemia de células vellosas (LCV) es un desorden linfoproliferativo crónico caracterizado por: esplenomegalia, pancitopenia con linfocitos vellosos e infiltración de médula ósea y de bazo. Rituximab demuestra eficacia terapéutica y en pacientes con LCV refractaria/recaída.

Splenic lymphoma with villous lymphocytes.

Splenic lymphoma with villous lymphocytes (SLVL) is a rare disorder that comprises less than 1% of lymphoid neoplasms. It is the leukemic counterpart of splenic marginal zone lymphoma (SMZL) and is characterized by splenomegaly, often with no lymphadenopathy, moderate lymphocytosis and villous lymphocytes on peripheral blood smear. Here, we report a case of SLVL in a 56-year-old male with very high leukocyte counts, massive splenomegaly and relatively few leukemic cells with subtle villous projections on the surface. This disorder is often confused with other chronic lymphoproliferative disorders, especially chronic lymphocytic leukemia (CLL) and hairy cell leukemia and should be differentiated from them. We are reporting this case to highlight the diagnostic pitfalls associated with this disorder.

Hairy cell leukaemia in a tertiary hospital in southern Nigeria: 10 year survey

The clinico-pathologic features, incidence and pattern of presentation of patients with the rare hairy cell leukaemia in University of Benin City Teaching Hospital, a major tertiary health centre in Niger Delta region of Nigeria have not been previously documented. All cases that presented from 1993 to 2003 were reviewed and this rare leukaemia constituted 1.5% of all haematological malignancies. Median age at presentation was 40 years. Typical clinical signs of pancytopenia and massive splenomegaly were found. Lymphadenopathy was rare. Normalization of pancytopenia was achieved by splenectomy followed by cytostatic agents but increased susceptibility to infection was not reduced. Four patients received interferon-alpha two patients, cladribine with prednisolone following splenectomy

Hairy-cell leukemia: a rare blood disorder in Asia.

We report a 68-year-old Indian man who was referred to the Hematology Unit for investigation for thrombocytopenia, an incidental finding during a pre-operative screening for prostatectomy. Physical examination was unremarkable. There was no splenomegaly, hepatomegaly or lymphadenopathy. Complete blood counts showed normal hemoglobin and total white cell count with moderate thrombocytopenia. Hairy-cell leukemia was diagnosed based on peripheral blood film, bone-marrow aspirate and trephine biopsy findings, supported by immunophenotyping results by flow cytometry. The purpose of this report is to create awareness of this uncommon presentation and to emphasize that a single-lineage cytopenia or absence of splenomegaly does not exclude the diagnosis of hairy-cell leukemia. Careful attention to morphological detail is important for early diagnosis, especially when low percentages of "hairy" cells are present in the peripheral blood and bone marrow. Early diagnosis is important to ensure that patients obtain maximum benefit from the newer therapeutic agents that have greatly improved the prognosis in this rare disorder.

Detection of CD5 in B-cell chronic lymphoproliferative diseases by flow cytometry: a strong expression in B-cell chronic lymphocytic leukemia

OBJETIVOS: CD5 é um marcador normalmente expresso nas células T e de forma aberrante nas células B da leucemia linfocítica crônica (LLC) e no linfoma de células do manto (LCM). Outras doenças linfoproliferativas crônicas como a hairy cell leukemia (HCL) e leukemia prolinfocítica de células B (LPL-B), são geralmente CD5 negativas ou expressam fracamente este antígeno. Neste trabalho investigou-se o padrão de expressão do CD5 em 42 pacientes com doenças linfoproliferativas crônicas de células B (DLC-B). METODOS: Investigamos a expressão de CD5 em células leucêmicas de 42 pacientes com DLC-B através da citometria de fluxo. Dados demográficos, tais como idade e sexo, bem como dados clínicos e laboratoriais também foram analisados. RESULTADOS: A imunofenotipagem mostrou que 35 casos foram LLC, 3 LPL-B, 3 HCL e um caso de LMC. O CD5 mostrou-se fortemente expresso em todos os casos de LLC e LMC. Baixa expressão desse antígeno foi observada em um caso de LPL-B, mostrando-se negativamente expresso em todos os casos de HCL. CONCLUSÃO: Nossos resultados demonstram que o padrão de expressão do CD5 pode auxiliar na distinção entre LLC da HCL e LPL-B, sendo no entanto similares na HCL e LCM.

Vasculitis leucocitoclásicas en leucemias a linfocitos vellosos / Leukocytoclastic vasculitis and hairy cell lymphocystic leukemia

Se presentan tres pacientes en quienes se observa la asociación de leucemia a linfocitos vellosos (LLV) y vasculitis leucocitoclásica (VL) (la vasculitis más frecuente en procesos linfoproliferativos). La LLV es una enfermedad rara que afecta con mayor frecuencia a hombres de edad media, siendo su signosintomatología esplenomegalia, pancitopenia, fatiga, infecciones recurrentes y anemia. Nuestros hallazgos a nivel dermatológico fueron en todos los casos de púrpura palpable. Algunas lesiones se ulceraron y transgredieron los límites inferiores. Se efectúa una revisión de la literatura y se comenta ésta rara asociación. Consultada la bibliografía éstos serían los únicos casos publicados a nivel nacional

Tratamento da leucemia de células cabeludas com interferon alfa / Treatment of hairy cell leukemia with alpha interferon

O presente trabalho descreve cinco casos de pacientes portadores de leucemia de células cabeludas (LCC) ou tricoleucemia, que foram tratados com interferon alfa, um modificador da resposta biológica com grande atividade nesta doença crônica linfoproliferativa. Novos conceitos considerando seu uso como terapêutica de primeira linha säo discutidos, bem como aspectos marcantes de sua etiopatogenia, diagnóstico clínico-laboratorial e histopatológico.

Leucemia de células vellosas: complicación dermatológica / Hairy-cell leukaemia: dermatologic complication

Se comunica un paciente de 49 años, sexo masculino, con lesión erosiva impetiginizada en región pubiana y ulceración no infiltrada en prepucio. Masa ganglionar inguinal derecha flegmásica de 15 x 5 x 2,5 cm. Micropoliadenopatías inguinales izquierdas y adenopatía asintomática axilar derecha. Hepatoesplenomegalia. Astenia, Pancitopenia. Los estudios realizados ratificaron el diagnóstico de L. C. V., cuya frecuencia es del 2% en el total de las leucemias del adulto. La C. V. tiene su origen en una célula linfoide B y se sitúa en el sistema de diferenciación en un estadio algo más evolucionado que la de la leucemia linfática crónica. Se caracteriza por tener citoplasma extenso dotado de típicas prolongaciones o pelos. La maior parte del cuadro clínicos es consecuencia de la pancitopenia y la explenomegalia. las lesiones dermatológicas asociadas más frecuentemente son las inespecíficas, y se expresan por infecciones recurrentes, equimosis o las resultantes de vasculitis. Las específicas se observan en un 8% de los casos y se traducen por erupciones máculo-pápulo eritematosas. predomina en el sexo masculino, pero el pronóstico es mejor en el femenino. Los tratamientos instituídos segun la sintomatología y curso evolutivo son la esplenectomía, el alfa Interferón, y 2 - Deoxycoformicina