Hemophagocytic lymphohistiocytosis with jaundice as first manifestation:A case report

BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening condition.It is an immune-mediated disease that has a wide range of causes,elicits a hyperinflammatory response,and results in multiple organ damage.Clinical presentations vary,and in some cases,jaundice occurs as the first symptom.CASE SUMMARY We report the case of a 71-year-old female patient who presented with jaundice.She was admitted to our hospital because of the occurrence of“jaundice for half a month”,and upon examination,obstructive jaundice with choledocholithiasis and gallstones was suggested.Cholecystectomy and choledocholithotomy were performed.However,the jaundice did not improve after surgery.We found splenomegaly,cytopenia,hypertriglyceridemia,hypofibrinogenemia,and elevated ferritin.Bone marrow biopsy revealed hemophagocytosis.Later,cardiac arrest occurred when she returned 3 wk after the surgery.We considered that HLH was triggered by septic shock.The patient’s condition deteriorated rapidly,with multiple organ dysfunction and severe gastrointestinal bleeding.Corticosteroid therapy and symptomatic treatment failed to save her life.CONCLUSION Jaundice rarely presents as the first symptom in HLH patients.The HLH in this case was triggered by septic shock with jaundice as the first symptom.Clinicians should try hard to reduce missed diagnoses and misdiagnoses.

Malignant biliary obstruction due to metastatic non-hepatopancreato-biliary cancer

Malignant biliary obstruction generally results from primary malignancies of the pancreatic head,bile duct,gallbladder,liver,and ampulla of Vater.Metastatic lesions from other primaries to these organs or nearby lymph nodes are rarer causes of biliary obstruction.The most common primaries include renal cancer,lung cancer,gastric cancer,colorectal cancer,breast cancer,lymphoma,and melanoma.They may be difficult to differentiate from primary hepato-pancreatobiliary cancer based on imaging studies,or even on biopsy.There is also no consensus on the optimal method of treatment,including the feasibility and effectiveness of endoscopic intervention or surgery.A thorough review of the literature on pancreato-biliary metastases and malignant biliary obstruction due to metastatic non-hepato-pancreato-biliary cancer is presented.The diagnostic modality and clinical characteristics may differ significantly depending on the type of primary cancer.Different primaries also cause malignant biliary obstruction in different ways,including direct invasion,pancreatic or biliary metastasis,hilar lymph node metastasis,liver metastasis,and peritoneal carcinomatosis.Metastasectomy may hold promise for some types of pancreato-biliary metastases.This review aims to elucidate the current knowledge in this area,which has received sparse attention in the past.The aging population,advances in diagnostic imaging,and improved treatment options may lead to an increase in these rare occurrences going forward.

Endoscopic ultrasound-guided biliary drainage-current status and future perspectives

Endoscopic retrograde cholangiopancreatography(ERCP)with stenting is the treatment modality of choice for patients with benign and malignant bile duct obstruction.ERCP could fail in cases of duodenal obstruction,duodenal diverticulum,ampullary neoplastic infiltration or surgically altered anatomy.In these cases percutaneous biliary drainage(PTBD)is traditionally used as a rescue procedure but is related to high morbidity and mortality and lower quality of life.Endoscopic ultrasound-guided biliary drainage(EUS-BD)is a relatively new interventional procedure that arose due to the development of curvilinear echoendoscope and the various endoscopic devices.A large amount of data is already collected that proves its efficacy,safety and ability to replace PTBD in cases of ERCP failure.It is also possible that EUS-BD could be chosen as a first-line treatment option in some clinical scenarios in the near future.Several EUS-BD techniques are developed EUS-guided transmural stenting,antegrade stenting and rendezvous technique and can be personalized depending on the individual anatomy.EUS-BD is normally performed in the same session from the same endoscopist in case of ERCP failure.The lack of training,absence of enough dedicated devices and lack of standardization still makes EUS-BD a difficult and not very popular procedure,which is related to life-threatening adverse events.Developing training models,dedicated devices and guidelines hopefully will make EUS-BD easier,safer and well accepted in the future.This paper focuses on the technical aspects of the different EUS-BD procedures,available literature data,advantages,negative aspects and the future perspectives of these modalities.