Case records of the Massachusetts General Hospital. Case 31-1966.
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An autopsy case of right-sided infective endocarditis combined with mitral valvular involvement in a 20-year-old male Japanese with ventricular septal defect (VSD) was reported. The vegetations were found on the endocardium bordering VSD, tricuspid valve, mural endocardium of the right ventricular outflow tract, and even the pulmonic valve, resulting in forming infective aneurysm of the pulmonary trunk. Streptococcus was morphologically identified in the vegetations obtained at autopsy. On the other hand, smaller vegetations were also noted on the mitral valve. The mechanisms of the mitral extending were discussed when right-sided infective endocarditis associated with VSD preceded that on the mitral valve. The authors think that mitral regurgitation in relation to VSD and right to left shunt through VSD which occur even temporarily may be the most important mechanism responsible for the mitral valvular involvement. Several differences between right-sided and left-sided infective endocarditis were also reviewed.
Patients with either homozygous or heterozygous sickle cell disease may have frequent episodes of abdominal pain and/or fever of uncertain cause. While many of these episodes represent a so-called sterile crisis, the possibility of gross organ infarction and rupture, infection, or other complication cannot be ignored. Computed tomography (CT) was used to evaluate 30 such patients. Virtually all patients had splenic abnormalities, which could be considered clinically significant (rupture, hemorrhage) or insignificant (calcified spleen). In four patients hepatic abnormalities were found, including one case of multiple liver infarction, one of hepatic abscess, and two of retained intrahepatic gallstones after cholecystectomy. Two patients had significant acute renal abnormalities, including one case of interstitial nephritis and one of renal vein thrombosis. One patient had a ruptured periappendiceal abscess and one a pericolonic abscess. One patient had an abscess around a total hip replacement. CT was found to be an excellent and relatively noninvasive means of both initial investigation and subsequent follow-up.
99mTc-diphosphonate bone scans of 13 patients in acute sickle crisis were analyzed. Twelve of the 13 patients had abnormal scans, with increased activity in joints and skull being a common finding. One patient with splenic calcification and two patients without splenic calcification had splenic uptake of diphosphonate. Most of the abnormal scans showed hot areas, with cold areas seen only in four cases. There was no correlation between the site of pain clinically and the abnormal areas on scanning. The pathophysiology of the abnormal uptake of radioactivity and the role of bone scanning on sickle cell disease are discussed.
A focal splenic defect was revealed during the investigation of a possible intra-abdominal abscess. Focal splenic defects have been described on 99Tcm-sulphur colloid liver-spleen scintigraphy in cases of infarction, lymphoma, metastatic carcinoma and cyst. This case is presented and the differential diagnosis discussed.
Ultrasonographic (US) examinations of the upper abdomen were performed in 80 patients with Gaucher disease. Of the 49 patients that had not undergone splenectomy, 47 had splenic enlargement. Sixteen patients had multiple lesions in the spleen. Most patients had discrete hypoechoic lesions that corresponded pathologically to focal homogeneous clusters of Gaucher cells. Several patients had similar lesions that were hyperechoic and were composed of Gaucher cells and fibrosis or infarction. A few patients had a geographic pattern of irregular areas of involvement of Gaucher cells among normal splenic parenchyma. The liver was often enlarged but otherwise sonographically unremarkable. Patients with Gaucher disease often have US examinations of the left upper quadrant for abdominal pain. A variety of US findings in the spleen typical for Gaucher disease should be recognized and not interpreted as acute changes.
METHODS: To evaluate the series of advanced ovarian cancer patients who underwent splenectomy as part of their surgery, a retrospective file review of 258 ovarian cancer cases were examined. RESULTS: There were 13 cases that underwent splenectomy. These patients constitute 5% of all and 8.8% of the advanced ovarian cancer patients. Seven cases had splenectomy as part of their initial cytoreductive surgery while 6 had surgery for recurrent disease. Splenectomy was performed to resect hilar and/or capsular involvement in 9 (69%) cases, while in 3 cases (23%) splenic injury during adjacent tumoral resection required splenectomy. One patient had splenectomy for infarcts and died after the surgery with systemic complications nonrelated to the surgery. No other serious morbidity was detected. Median survival of the patients was 34 months (1-120 months) from the initial diagnosis. Median survival was 18 months (1-78 months) after the splenectomy. Three patients were still alive with no evidence of disease 2, 5 and 6.5 years after the splenectomy. CONCLUSION: Splenectomy is a feasible and safe procedure. However, it should be reserved for patients in whom optimal cytoreductive results could be achieved.
Cystic lesions of the spleen include parasitic and nonparasitic cysts. Parasitic cysts are due almost exclusively to echinococcal disease representing 50 to 80 per cent of splenic cysts. However, splenic echinococcosis is not so common as hepatic or pulmonary location (only 3.5 per cent described in literature). Nonparasitic cysts are classified as primary or true cysts, which have an epithelial or endothelial lining, and pseudocysts. True cysts of the spleen are very rare and include epidermoid and dermoid cysts, cystic hemangiomas and cystic lymphangiomas. Pseudocysts are much more common and may be post-traumatic (haemorrhagic or serosal), degenerative (infarction) or inflammatory. Such conditions can be occasionally observed or detected in emergency in case of splenic rupture. Four cases of splenic cystic pathology are reported. Correlated diagnostic problems are analyzed. The four cases (a cystic hydatidosis, a cystic lymphangioma and two post-traumatic haemorrhagic cysts), successfully treated with splenectomy are discussed.
Examinations of the clinical and immunological state of 68 patients with the acute stage of cerebral stroke were carried out, and so were immunomorphological examinations of the spleen and the subclavicular lymph nodes in 36 of them whose disease ended in death. An analysis of the results obtained has shown that the immunological reactivity increases from the very first day of the disease. The authors believe that the early immunological response, especially in cases of brain infarction, is due to development of the cerebral stroke in a sensitized body. A rise of selective antibody titres depending on the character of the cerebrovascular disorder was noted: there were: anti-encephalic antibodies targeted at the malacia focus in cases of brain infarction, and antivascular, and to a lesser extent anti-encephalic antibodies in cases of a hemorrhagic stroke. The informative value of the complement fixation test was found to be greater than that of the hemagglutination inhibition test. High degrees of plasmatization of the splenic tissue and the presence of the anti-encephalic immune complexes in it, mainly in cases of brain infarction, also point to the activity of the immunogenesis in the acute stage of the cerebral stroke.
Splenic rupture associated with anticoagulant therapy is a rare entity. We report a case of spontaneous splenic rupture in a 62-year-old man who was treated by acenocoumarol orally after an aortic-valve replacement. Unrecognised, minor trauma may lead to splenic haemorrhage in patients receiving anticoagulant therapy. The symptoms and signs may mimic those of acute myocardial infarction and cardiogenic shock. Early diagnosis is essential. Reversal of the anticoagulant and emergent splenectomy are the treatments of choice.
A very unusual cause of splenic rupture is described in a 73-year-old man with severe coronary artery disease. A transthoracic intra-aortic balloon pump (IABP) was inserted during coronary artery bypass grafting and was used for 3 days. However, his hypotension continued, and ischemic changes in his hand were thought to be due to the use of the pump. He died 7 days later of cardiac arrest. Autopsy revealed multiorgan failure associated with a preoperative myocardial infarct. Numerous systemic arterial atheroemboli, likely resulting from IABP-related aortic trauma and hypotension, had given rise to severe acute pancreatitis. The necrotizing process within the pancreatic tail had extended to the splenic hilum, eroding its capsule and causing splenic rupture and hemoperitoneum.
Thrombosis of the portal-mesenteric axis is an infrequent cause of intestinal ischemia or infarction. In addition to the multiple acquired factors that contribute to the development of this entity, hereditary risk factors, especially the factor V Leiden mutation and the G20210A mutation of the prothrombin gene, have been implicated. The G20210A mutation of the prothrombin gene is found in up to 40% of patients with splenic-portal-mesenteric thrombosis. The present case illustrates the unusual and nonspecific presentation of this mutation in the form of diarrhea and images of thrombosis of the superior mesenteric-portal vein and cavernous transformation of the portal vein. Delayed diagnosis is highly frequent since the clinical signs, laboratory investigations and radiological tests do not suggest the diagnosis. The patient received anticoagulant treatment and showed clinical improvement with complete portal-mesenteric recanalization. Currently the diagnostic technique of choice is magnetic resonance angiography or computerized tomography angiography and treatment consists of indefinite anticoagulation. This case illustrates that an unusual or atypical localization of venous thrombosis may be a manifestation of thrombophilia, emphasizing the importance of genetic screening in these cases.
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Subchronic and chronic toxicities of hexachlorobenzene (HCB) were studied in both sexes of Swiss mice, Syrian golden hamsters and Sprague-Dawley rats, at dietary dosages of 0, 100 and 200 ppm (mice), and 0, 200 and 400 ppm (hamsters and rats) for 90 days. At day 91, 25/50 animals in each of 18 groups were killed for histology studies. The rest were killed at 6-week intervals until the study was ended. Marked hepatosplenomegaly, enlarged thymuses and lymph nodes, or swollen and granular-looking renal cortices with depressions or nodulary areas were commonly observed. Dose- and sex-dependent progressive changes included toxic-degenerative hepatitis, chronic cirrhosis, hepatomas, bile-duct adenomas and a few hepatocarcinomas in older animals. A generalized lymphohaematopoietic response led to thymic, splenic and nodal lymphosarcomas, especially in female mice. Toxic-tubular nephritis with cortical infarcts developed into regenerative foci and renal adenomas in low incidences. Liver lesions were more prominent in females, while renal changes were most common in male rats. HCB was retested in both sexes of rats at oral doses of 0, 75 and 150 ppm for up to 2 years. At the start, each group contained 94 rats, and four randomly selected rats were killed at weeks 0, 1, 2, 3, 4, 8, 16, 32, 48 and 64 for microscopy. Progressive liver lesions started as hyperaemia and degenerations (4 weeks), and developed into toxic hepatitis, cirrhosis and formation of pre- and neoplastic foci (36 weeks), with hepatomas, bile-duct adenomas and hepatocellular carcinomas (64 weeks) in very high incidences in females and renal adenomas in male rats.
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Sarcoidosis involves the spleen in a significant percentage of patients afflicted with this disease. The most commonly applied modality for studying such patients is the (99m)Tc-sulfur colloid scan. Different patterns have been recognized: "normal" spleen and nonspecific splenic enlargement, with or without focal areas of photon deficiency, which was thought to represent an infarction in a previous report. We have encountered two patients showing splenomegaly and multifocal photon deficiencies secondary to sarcoid granulomata. These cases are presented to inform clinicians of a new pattern. Our patients were managed with systemic corticosteroids and palliation of pain. If symptoms persist or worsen, splenectomy may be necessary to avoid spontaneous rupture. An abbreviated differential diagnosis of the enlarged spleen with multiple focal defects is discussed.
The Authors report a case of spontaneous splenic rupture in a patient with Cytomegalovirus infection, stress the main characteristics of this infection and describe the mechanism that, during the viral infection, causes morphological and functional alterations of the spleen. The hypersplenism secondary to hyperfunctioning, the formation of immunocomplexes with secondary infarction, mainly of the white pulp, and the disseminated intravascular coagulation are responsible, as in the observed case, of the rupture of splenic capsula. The Authors conclude that the wide diffusion of Cytomegalovirus infection needs the knowledge of all the pathologic signs of this infection to make a timely diagnosis and treatment.
Cutaneous lesions of hypereosinophilic syndrome (HES) may display various presentations. These are important to recognize to allow early diagnosis. We report an unusual case of a young man with HES secondary to a splenic T-cell lymphoma, revealed by diffuse necrotic cutaneous lesions. Later on, brain and heart infarctions developed, leading to the patient's death. Analysis of skin biopsy specimens showed occluded dermal vessels surrounded by activated eosinophils. Because a complete analysis of hemostasis was unremarkable, these findings suggest that dermal vessel thrombosis was the direct consequence of the migration of activated eosinophils. Cutaneous lesions of HES are protean and probably reflect the differences between the lesional events induced in situ by activated eosinophils.