Autopsy findings in a patient with primary systemic AL (kappa light chain) amyloidosis

First described by Rokitansky in 1842, and further characterized by Virchow in 1854, amyloidosis is a disorder caused by amyloid deposition, a fibrillary insoluble protein. The clinical spectrum of amyloidosis is broad, as the amyloid deposition may virtually occur in all tissues. Herein, we report the case of a 66-year-old man with a long-lasting emaciating disease, diagnosed, at autopsy, with primary systemic amyloidosis. Amyloid protein deposition was found in many tissues and organs. The involvement of the vessels’ wall rendered ischemic injury most prominent in the intestinal loops causing mesenteric ischemia. Despite the thorough organic involvement, the immediate cause of death was aspiration bronchopneumonia. Massive amyloid deposition was found in virtually all major organs, such as the heart, liver, kidneys, spleen, pancreas, adrenals, prostate, skin, and thyroid: the latter, a complication of the amyloidosis known as amyloid goiter. Post-mortem review of the deceased’s laboratory workup showed a slightly abnormal kappa:lambda ratio in the blood; however, no clonal lymphoplasmacytic disorder was confirmed in the bone marrow and other lymphoreticular system organs either by the microscopic examination and immunohistochemical staining. Laser-capture microdissection and tandem mass spectrometry of the splenic tissue detected a peptide profile consistent with an immunoglobulin Kappa light chain. The presence of amyloid purpura favors the diagnosis of primary systemic amyloidosis

Sudden death in infective endocarditis

The case fatality rate of infective endocarditis (IE) is high and is associated with varying causes. Among them, acute myocardial infarction due to an embolism in a coronary artery is rare; the incidence of this complication in the setting of IE is reported to be up to 1.5%. We report a case of sudden death in a 22-year-old woman diagnosed with systemic lupus erythematosus who was referred to the Cardiology Center for the treatment of mitral valve incompetence due to IE. She was hemodynamically stable with antibiotic therapy and vasoactive drugs, despite severe mitral valve regurgitation. Unexpectedly, she presented cardiac arrest and died. The autopsy showed total occlusion of the left main coronary artery by septic embolus, which originated from the mitral vegetation, as the cause of death. Thus, although a rare complication, it should always be kept in mind that a coronary embolism can be a lethal complication of IE, and the possibility of surgical treatment combined with the underlying antibiotic therapy should be raise

Peripheral pulmonary artery aneurysm presenting as a solitary pulmonary nodule

We report the case of a 63-year-old female patient who was evaluated due to a solitary pulmonary nodule. The final diagnosis was a solitary peripheral pulmonary artery saccular aneurysm. The patient was submitted to a pulmonary lobectomy with excellent recovery. Peripheral pulmonary artery aneurysms that arise from segmental or intrapulmonary branches are extremely rare, and their management is still controversial

Sudden death in infective endocarditis

The case fatality rate of infective endocarditis (IE) is high and is associated with varying causes. Among them, acute myocardial infarction due to an embolism in a coronary artery is rare; the incidence of this complication in the setting of IE is reported to be up to 1.5%. We report a case of sudden death in a 22-year-old woman diagnosed with systemic lupus erythematosus who was referred to the Cardiology Center for the treatment of mitral valve incompetence due to IE. She was hemodynamically stable with antibiotic therapy and vasoactive drugs, despite severe mitral valve regurgitation. Unexpectedly, she presented cardiac arrest and died. The autopsy showed total occlusion of the left main coronary artery by septic embolus, which originated from the mitral vegetation, as the cause of death. Thus, although a rare complication, it should always be kept in mind that a coronary embolism can be a lethal complication of IE, and the possibility of surgical treatment combined with the underlying antibiotic therapy should be raise

Fatal adenoviral necrotizing bronchiolitis case in a post-cardiac surgery intensive care unit

We report a case of a 67 year-old-male patient admitted to the intensive care unit in the post-coronary bypass surgery period who presented cardiogenic shock, acute renal failure and three episodes of sepsis, the latter with pulmonary distress at the 30th post-operative day. The patient expired within five days in spite of treatment with vancomycin, imipenem, colistimethate and amphotericin B. At autopsy severe adenovirus pneumonia was found. Viral pulmonary infections following cardiovascular surgery are uncommon. We highlight the importance of etiological diagnosis to a correct treatment approach

Occurrence of mesothelial/monocytic incidental cardiac excrescences in material from open-heart procedures: case reports and literature review

Mesothelial/monocytic incidental cardiac excrescences (MICE) are unusual findings during the histological analysis of material from the pericardium, mediastinum, or other tissues collected in open-heart surgery. Despite their somewhat worrisome histological appearance, they show a benign clinical course, and further treatment is virtually never necessary. Hence, the importance of recognizing the entity relays in its differential diagnosis, as an unaware medical pathologist may misinterpret it for a malignant neoplasm. Other mesothelial and histiocytic proliferative lesions, sharing very close histological morphology and immunohistochemistry features with MICE, have been described in sites other than the heart or the mediastinum. This similarity has led to the proposal of the common denomination “histiocytosis with raisinoid nuclei.” We report three cases from the pathology archives of the Heart Institute of São Paulo University (Incor/HC-FMUSP), diagnosed as “mesothelial/monocytic incidental cardiac excrescence,” with immunohistochemical documentation, and provide a literature review of this entity

Human hemorrhagic pulmonary leptospirosis: pathological findings and pathophysiological correlations.

Abstract\ud Background: Leptospirosis is a re-emerging zoonosis with protean clinical manifestations. Recently, the importance of\ud pulmonary hemorrhage as a lethal complication of this disease has been recognized. In the present study, five human\ud necropsies of leptospirosis (Weil‘s syndrome) with extensive pulmonary manifestations were analysed, and the antibodies\ud expressed in blood vessels and cells involved in ion and water transport were used, seeking to better understand the\ud pathophysiology of the lung injury associated with this disease.\ud Principal Findings: Prominent vascular damage was present in the lung microcirculation, with decreased CD34 and\ud preserved aquaporin 1 expression. At the periphery and even inside the extensive areas of edema and intraalveolar\ud hemorrhage, enlarged, apparently hypertrophic type I pneumocytes (PI) were detected and interpreted as a non-specific\ud attempt of clearence of the intraalveolar fluid, in which ionic transport, particularly of sodium, plays a predominant role, as\ud suggested by the apparently increased ENaC and aquaporin 5 expression. Connexin 43 was present in most pneumocytes,\ud and in the cytoplasm of the more preserved endothelial cells. The number of type II pneumocytes (PII) was slightly\ud decreased when compared to normal lungs and those of patients with septicemia from other causes, a fact that may\ud contribute to the progressively low PI count, resulting in deficient restoration after damage to the alveolar epithelial\ud integrity and, consequently, a poor outcome of the pulmonary edema and hemorrhage.\ud Conclusions: Pathogenesis of lung injury in human leptospirosis was discussed, and the possibility of primary noninflammatory\ud vascular damage was considered, so far of undefinite etiopathogenesis, as the initial pathological\ud manifestation of the disease

Endocarditis due to Coxiella burnetii (Q fever): a rare or underdiagnosed disease? Case report

A febre Q é uma zoonose de distribuição mundial causada por Coxiella burnetii, sendo raros os registros da doença no Brasil. Estudos soroepidemiológicos mostraram uma freqüência relativamente elevada de anticorpos contra Coxiella burnetii em populações com exposição ocupacional. Em humanos, pode se manifestar clinicamente como doença aguda ou crônica, sendo que a endocardite é a forma crônica mais freqüente da febre Q e de maior morbi-mortalidade. Relatamos um caso grave de endocardite por Coxiella burnetii adquirida no Brasil com desfecho fatal, apesar de antibioticoterapia adequada e tratamento cirúrgico valvar.Q fever is a zoonosis of worldwide distribution that is caused by Coxiella burnetii. However, reports of this disease in Brazil are rare. Seroepidemiological studies have shown relatively high frequencies of antibodies against Coxiella burnetii in populations with occupational exposure. In humans, it can be manifested clinically as acute or chronic disease. Endocarditis is the most frequent chronic form of Q fever and the form with the greatest morbidity and mortality. We report a severe case of endocarditis due to Coxiella burnetii acquired in Brazil that had a fatal outcome, despite specific antibiotic therapy and valve surgery treatment