RESUMO
Three full-term infants died in the first month of life with hypoplastic left heart syndrome (HLH) and persistent pulmonary hypertension (PPH). At postmortem examination, they were found to have alveolar capillary dysplasia with misalignment of pulmonary veins (ACD with MPV). The association of HLH syndrome, and ACD with MPV with intestinal malrotation and/or obstruction, is unique. Decreased blood flow in the ascending aorta in fetuses with left outflow tract obstruction might cause vasoconstriction of pulmonary arterioles to maintain cerebral perfusion. Vasoconstriction early during embryogenesis might lead to decreased growth and development of alveolar capillaries and pulmonary veins. This results in pulmonary hypertension, and the arterial blood is forced to bypass the deficient capillary bed and can drain only via the anomalous bronchial veins.
Assuntos
Síndrome do Coração Esquerdo Hipoplásico/patologia , Alvéolos Pulmonares/patologia , Veias Pulmonares/anormalidades , Veias Pulmonares/patologia , Capilares/patologia , Evolução Fatal , Feminino , Idade Gestacional , Humanos , Hipertensão Pulmonar/congênito , Hipertensão Pulmonar/patologia , Recém-Nascido , Obstrução Intestinal/congênito , Obstrução Intestinal/patologia , Masculino , Alvéolos Pulmonares/irrigação sanguíneaRESUMO
To develop a practical immunohistochemistry panel for distinguishing lymphoblastic lymphoma from Ewing sarcoma (ES), we evaluated 17 ES and 27 lymphoblastic lymphoma and leukemia cases with antibodies to CD99, terminal deoxynucleotidyl transferase (TdT), leukocyte common antigen (LCA), CD43, CD79a, CD20, CD3, vimentin, and neuron-specific enolase (NSE). Three cases were bone lymphomas, 2 initially misdiagnosed as ES. All cases were CD99+. All lymphomas and leukemias were TdT+ compared to none of the ESs. None of the ESs expressed other lymphocytic markers, which were inconsistently expressed in the lymphomas and leukemias: CD43, 33%; LCA, 30%; CD79a, 19%; CD3, 19%; and CD20, 7%. Of the ESs, 88% were vimentin positive compared with 23% of lymphomas and leukemias. Vimentin was stronger and more diffuse in ES. NSE did not reliably stain any cases. When faced with the differential diagnosis of ES vs lymphoblastic lymphoma, an immunohistochemical panel that includes antibodies to CD99 and TdT is useful. Both epitopes are well preserved in fixed and decalcified tissue. A panel composed of antibodies to CD99 and TdT, in conjunction with other lymphocytic markers and vimentin, is highly sensitive and specific.
Assuntos
Imuno-Histoquímica , Leucemia-Linfoma Linfoblástico de Células Precursoras/metabolismo , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Sarcoma de Ewing/metabolismo , Sarcoma de Ewing/patologia , Antígeno 12E7 , Adulto , Antígenos CD/metabolismo , Moléculas de Adesão Celular/metabolismo , Criança , DNA Nucleotidilexotransferase/metabolismo , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Estudos Retrospectivos , Vimentina/metabolismoRESUMO
We describe the clinical course, angiography, and histopathology of a newborn male with pulmonary atresia and intact ventricular septum who succumbed to a myocardial infarction. Angiography demonstrated right ventricular-dependent coronary circulation and focal areas of coronary narrowing. His clinical course was characterized by attacks of sudden irritability, consistent with ischemia. Histology demonstrated significant coronary artery narrowing secondary to fibromuscular dysplasia as well as evidence of new and old infarction. This case illustrates the severity of coronary lesions in pulmonary atresia and the potential for progression of coronary obstruction and insufficiency, and it provides correlation between angiography, ischemic symptoms, and pathology.
