Mahadevan Navin R, Morgan Elizabeth A, Mitchell Richard N
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
Cardiovasc Pathol. 2016 Sep-Oct;25(5):371-4. doi: 10.1016/j.carpath.2016.05.007. Epub 2016 May 30.
Pericardial effusion can cause cardiac tamponade physiology with resultant cardiogenic shock and death. Myelofibrosis, the replacement of marrow cavity by fibrous connective tissue, is a secondary complication of a group of disorders known as myeloproliferative neoplasms, which are clonal processes characterized by abnormal proliferative growth of one or more hematopoietic lineages. One consequence of myelofibrosis is the development of hematopoiesis at other anatomic sites, most commonly the spleen and liver, a phenomenon known as extramedullary hematopoiesis (EMH). Herein we report a case of a man who died from pericardial tamponade due to a subacute pericardial effusion secondary to EMH in the pericardium in the setting of myelofibrosis. This case highlights an unusual etiology for pericardial effusion and tamponade that should be considered in cases of myelofibrosis and stimulates a discussion regarding the mechanisms and anatomic distribution of EMH.
心包积液可导致心脏压塞生理状态,进而引发心源性休克和死亡。骨髓纤维化是指骨髓腔被纤维结缔组织替代,是一组称为骨髓增殖性肿瘤的疾病的继发性并发症,这些疾病是克隆性过程,其特征是一个或多个造血谱系的异常增殖性生长。骨髓纤维化的一个后果是在其他解剖部位发生造血,最常见的是脾脏和肝脏,这种现象称为髓外造血(EMH)。在此,我们报告一例男性患者,其在骨髓纤维化背景下,因心包髓外造血继发亚急性心包积液导致心包压塞而死亡。该病例突出了心包积液和压塞的一种不寻常病因,在骨髓纤维化病例中应予以考虑,并引发了关于髓外造血的机制和解剖分布的讨论。