North American Journal of Medical Sciences最新文献

Context: Hemophagocytic Lymphohistiocytosis or the "Hemophagocytic Syndrome" is a spectrum of disorders of regulatory immunomodulatory pathways inciting phagocytosis of hematopoietic cells resulting in end-organ damage. The condition appears in both heritable and non-heritable forms from a multitude of possible environmental triggers, most notably infection. The condition often results in a fatal outcome without prompt diagnosis and treatment. Cases in children have been reported much more frequently and classically than in adult patients.

Case report: In this case series we examined five such cases in adult patients that were found at our institution in a window as small at 2 years with more cases having presented since the time of this writing. In these cases, likely triggers were found ranging from infectious, drug-inducing and even underlying malignancy. The condition can be diagnosed by a set of laboratory and physical criteria (Hemophagocytic Lymphohistiocytosis -2004). Treatment ranges from immunosuppressive agents to chemotherapeutic approaches with variable success.

Conclusion: Clinicians must maintain a higher index of suspicion in cases presenting with ominous symptomatology to ensure a prompt diagnosis and effective treatment of this potentially deadly condition.

Context: Splenic aplasia is seen when the spleen is congenitally absent, has been surgically removed, or becomes atrophic secondary to episodes of arterial/venous occlusion, which result in splenic infarction. This rare condition is caused by a heterogenous group of diseases, which may present a wide spectrum of clinical manifestations. Splenic hypoplasia is defined as reduction in splenic mass and or functions caused by incomplete splenic development or secondary parenchymal involution. Splenic infarction may be clinically silent and only discovered incidentally during abdominal exploration for other conditions.

Case report: We present an unusual case of hypoplastic spleen with calcifications, which was preoperatively found during radiologic workup for gastric carcinoma. An 88-year-old woman presented with coffee-ground emesis. Her past medical history was only significant for atrial fibrillation. Esophagogastroduodenoscopy demonstrated gastric carcinoma, for which a subtotal gastrectomy was planned. Preoperative computed tomography scan showed a hypoplastic spleen with calcifications in the left upper quadrant. Symptoms of immunologic deficiency were not present. During laparotomy, an atrophied and calcified spleen was identified and left in situ. The patient made an uneventful postoperative recovery. Splenic hypoplasia is an unique entity, which may be seen in the setting of atrial fibrillation and abdominal malignancy.

Conclusion: Splenic hypoplasia may be detected incidentally during radiologic workup or abdominal exploration. Abdominal symptoms or immunologic deficiency are not always present.

Context: Transient global amnesia (TGA) is an intriguing condition that classically presents with an abrupt onset of temporary complete anterograde amnesia and partial retrograde amnesia. Most individuals who experience such a form of amnesia usually have only one attack but recurrent attacks are possible. Most attacks last for a few minutes or few hours and the ability to lay down new memories may also be affected but gradually improves, leaving only a dense amnestic gap for the duration of the episode. There has been some discussion about the etiology behind TGA; however, there has yet to be a consensus with regard to any significant association.

Case report: We report the case of a 65-year-old male presenting with a sudden onset of memory loss that is typical of TGA and who was found to have elevated homocysteine levels. There has only been one other case previously reported that discussed a possible correlation between hyperhomocysteinemia and TGA. It is yet to be determined if increased homocysteine level is a significant risk factor for attacks of TGA.

Conclusion: Although it was first described more than half a century ago, it can still be misdiagnosed frequently as many physicians are not familiar with this condition. Furthermore, there are quite a few medical conditions that may cause sudden memory impairment, such as epilepsy and stroke, which make it difficult to distinguish them from this form of amnesia. The knowledge of these clinical identities is necessary for a high index of suspicion, which may lead to a meticulous medical evaluation as required for proper diagnosis.