A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number ...A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number of macrophages with hemophagocytosis and liver biopsy showed pure centrilobular cholestasis with necrosis and some absence of portal bile ducts. Furthermore, a serological test for Epstein-Barr virus was positive. Under treatment by liver dialysis and administration of steroids led to rapidly defervescence and clinical improvement. However, liver enzymes were still markedly elevated with persistent anemia, even after immunosuppressive treatment. The patient is currently waiting for liver transplantation. This is the ?rst description of vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis, with underlying causes including infection, drug-induced factors and untreated autoimmune disorder.展开更多
Hemophagocytic lymphohistiocytosis(HLH)is a cytokine storm syndrome caused by an overactive but ineffective immune reaction.Without prompt diagnosis and treatment,HLH is life-threatening.However,presenting symptoms ar...Hemophagocytic lymphohistiocytosis(HLH)is a cytokine storm syndrome caused by an overactive but ineffective immune reaction.Without prompt diagnosis and treatment,HLH is life-threatening.However,presenting symptoms are often nonspecific,with fatigue and fever being the most common.A high index of suspicion is therefore critical for early diagnosis and timely management.A previously healthy,65-year-old female who initially presented with fever and abdominal pain developed abdominal compartment syndrome(ACS)requiring decompressive laparotomy on hospital day 6.Intraoperative frozen sections of biopsied liver showed intense portal lymphohistiocytic infiltrates.Epstein-Barr virus DNA copy numbers escalated from 600 copies/mL after admission to 134,000 copies/mL before death.The diagnostic criteria of HLH-2004 were met.Patient expired on hospital day 12.It is important to raise awareness of ACS being an unusual presentation of HLH.Recent changes in diagnostic criteria tailored to adult HLH cases are reviewed.展开更多
文摘A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number of macrophages with hemophagocytosis and liver biopsy showed pure centrilobular cholestasis with necrosis and some absence of portal bile ducts. Furthermore, a serological test for Epstein-Barr virus was positive. Under treatment by liver dialysis and administration of steroids led to rapidly defervescence and clinical improvement. However, liver enzymes were still markedly elevated with persistent anemia, even after immunosuppressive treatment. The patient is currently waiting for liver transplantation. This is the ?rst description of vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis, with underlying causes including infection, drug-induced factors and untreated autoimmune disorder.
文摘Hemophagocytic lymphohistiocytosis(HLH)is a cytokine storm syndrome caused by an overactive but ineffective immune reaction.Without prompt diagnosis and treatment,HLH is life-threatening.However,presenting symptoms are often nonspecific,with fatigue and fever being the most common.A high index of suspicion is therefore critical for early diagnosis and timely management.A previously healthy,65-year-old female who initially presented with fever and abdominal pain developed abdominal compartment syndrome(ACS)requiring decompressive laparotomy on hospital day 6.Intraoperative frozen sections of biopsied liver showed intense portal lymphohistiocytic infiltrates.Epstein-Barr virus DNA copy numbers escalated from 600 copies/mL after admission to 134,000 copies/mL before death.The diagnostic criteria of HLH-2004 were met.Patient expired on hospital day 12.It is important to raise awareness of ACS being an unusual presentation of HLH.Recent changes in diagnostic criteria tailored to adult HLH cases are reviewed.
