ABSTRACT
Estudamos o envolvimento secundário pulmonar por leucemias em estudo retrospectivo de 12 anos de autópsias. Descrevemos o padrão de infiltração pulmonar em 77 linfomas e 50 leucemias. Os linfomas apresentaram maior freqüência do padrão peribrônquico/ perivascular de infiltração. O padrão intersticial foi mais freqüente nas leucemias. Mais de um padrão de infiltração estavam presentes por paciente. Linfomas de Hodgkin apresentaram maior freqüência do padrão nodular em relação aos linfomas B e T. Linfomas T apresentaram maior freqüência de infiltração intersticial do que os linfomas B / We have studied the secondary lung involvement by lymphomas and leukemias in a retrospective 12-year autopsy study. We have described histological infiltration patterns in lymphomas and leukemias. Lymphomas showed peribronchial/perivascular infiltration pattern. Leukemias had more frequently interstitial pattern. More than one infiltration pattern was present per patient. Hodgkin lymphomas had increased frequency of nodular infiltration pattern than B or T cell lymphomas. T cell lymphomas had more frequent interstitial pattern than B cell lymphomas. Pleural and intrapulmonary lymphnode involvement was more frequent in lymphomas than in leukemias...
Subject(s)
Autopsy , Hodgkin Disease/classification , Lymphoma, Non-Hodgkin/classification , Hodgkin Disease/pathology , Retrospective StudiesABSTRACT
Context: Chédiak-Higashi Syndrome (CHS) is a rare autosomal recessive disease characterized by recurrent infections, giant cytoplasmic granules, and oculocutaneous albinism. Objective: To describe clinical and laboratory findings from CHS patients. Design: Case report. Setting: The patients were admitted into the Allergy and Immunology Unit of the Instituto da Criança, a tertiary public care institution. Cases Report: Seven patients had oculocutaneous albinism, recurrent infections and giant cytoplasmic granules in the leukocyte. One patient had low IgG levels and three showed impaired bactericidal activity of neutrophils. Six patients died of infectious complications during the accelerated phase. Therapy included ascorbic acid and antibiotics. Chemotherapy was used for the accelerated phase in two patients. Bone marrow transplantation (BMT) was proposed for one patient. Discussion: The authors emphasize the need for early diagnosis and therapy of CHS. BMT should be indicated before the accelerated phase of the disease has developed.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Chediak-Higashi Syndrome/diagnosis , Retrospective Studies , Chediak-Higashi Syndrome/drug therapy , Chediak-Higashi Syndrome/physiopathologyABSTRACT
Os autores apresentam quatro individuos com a anomalia de Pelger-Huet, em duas familias. Dois dos pacientes foram encaminhados para diagnostico de infeccao devido a interpretacao erronea de hemograma. Os outros dois eram parentes de um dos casos-indices. Os autores enfatizam a importancia do diagnostico da anomalia para nao se incorrer em condutas diagnosticas e terapeuticas desnecessarias