HIV Infection Presenting as Haemophagocytic Syndrome

The authors describe a case of a 48-year-old man who presented with four weeks of fever, generalized malaise, weight loss, right upper quadrant abdominal pain and hepatosplenomegaly. He evolved with pancytopenia, bone marrow haemophagocytosis and hyperferritinaemia. Recent diagnosis of HIV infection, with the exclusion of other plausible causes, prompted the diagnosis of haemophagocytic syndrome (HPS) secondary to HIV. Despite intensive care support and initiation of antiretroviral therapy, the patient died. HPS diagnosis secondary to HIV alone demands the exclusion of all the other secondary causes. The best approach includes early diagnosis and specific treatment of the associated cause, whenever possible.

Guillain-Barré Syndrome with Absent Brainstem Reflexes: a Case Report

A 41-year-old man was admitted to an intensive care unit following respiratory arrest. One day prior to admission, he had complaints of nausea and pain involving lower limbs. On the night of admission he developed diplopia, dysphagia, and rapidly progressive quadriparesis. He developed respiratory failure requiring mechanical lung ventilation 24 hours later. On the fifth day of hospital stay the patient became comatose with absent brainstem reflexes and appeared to be brain dead. The cerebrospinal fluid showed albuminocytological dissociation. The electroencephalogram revealed an alpha rhythmical activity. The electrophysiological evaluation revealed an inexcitability of all nerves. Guillain-Barré syndrome was suspected. With supportive treatment the patient had a remarkable recovery and now is able to independently conduct his daily activities.

Severe Case of Thrombotic Microangiopathy with a Delayed Diagnosis of Atypical Haemolytic Uraemic Syndrome Successfully Managed with Eculizumab

Objectives: To describe the diagnosis and treatment of a severely ill patient presenting with thrombotic microangiopathy (TMA) of unknown cause. Case presentation: An adult female presented to intensive care with abdominal pain and haemorrhagic shock, requiring reanimation. Results: Features of TMA were present, but initial plasma exchange was ineffective. Treatment with the anti-C5 antibody, eculizumab, improved laboratory parameters and organ function, albeit slowly. Eculizumab remains effective and well tolerated after 30 months of treatment. Conclusion: This case demonstrates the complexities and importance of early identification of atypical haemolytic uraemic syndrome in patients presenting with TMA.