RECURRENT SEPSIS IN A PATIENT WITH BILIARY HAMARTOMAS.

Biliary hamartomas or von Meyenburg complexes (VMCs) are hepatic tumour-like lesions related to congenital malformation of the ductal plate, and are part of the ciliopathy spectrum of disorders. The exact pathogenesis of VMCs is unclear and it remains controversial whether they have the potential for malignant transformation. Patients are often asymptomatic and VMCs are usually encountered as an incidental finding on imaging. We report a case of recurrent sepsis with an unidentified focus. It was later confirmed that biliary hamartomas were acting as a sanctuary for the persistent pathogenic agent. The authors hope to draw attention to the existence of this unusual focus of recurrent sepsis. LEARNING POINTS: Hepatobiliary sepsis is an unusual clinical presentation of biliary hamartomas.Clinicians should be aware of the infectious complications of these diffuse structural biliary ductal abnormalities.Early recognition of this atypical life-threatening clinical presentation is important for the prognosis.

Gaucher Disease: One of the Few Causes of Massive Splenomegaly.

Gaucher disease (GD) is a rare, autosomal recessive genetic disease caused by deficiency of a lysosomal enzyme (glucocerebrosidase/ß-glucosidase) that leads to the accumulation of its substrate in lysosomal macrophages. GD remains rare and delayed diagnosis is common due its gradual onset. It is important to include this differential diagnosis in cases of massive splenomegaly and/or thrombocytopenia, in order to avoid potentially harmful splenectomy. This case report describes a 25-year-old female patient with a 10-year medical history of anaemia and thrombocytopenia, who presented with symptoms of haemorrhagic dyscrasia, pancytopenia and massive splenomegaly. The differential diagnosis of massive splenomegaly included several conditions which were considered but ruled out. Because of a lack of resources, the patient was forwarded to a reference centre where the diagnosis of GD was made. LEARNING POINTS: Many diseases are associated with splenomegaly but massive splenomegaly is seen in only a few conditions.While some causes of splenomegaly are obvious (malaria, infection), the aetiological diagnosis of splenomegaly may involve extensive history taking, serum testing and imaging studies.Infiltrative disorders such as Gaucher disease are a rare cause of splenomegaly and should be considered when other more common causes have been ruled out.The authors hope to raise awareness of this diagnosis in order to encourage early treatment.

Ceftriaxone-induced Agranulocytosis.

Ceftriaxone is a widely used antibiotic regarded as safe and effective. Drug-induced agranulocytosis is a life-threatening adverse reaction and few reports related to ceftriaxone were found in a review of the literature. The authors present a case of ceftriaxone-induced agranulocytosis, in which a brain abscess was diagnosed and ceftriaxone was commenced. Neutropenic fever occurred on the 29th day of therapy with a cumulative dose of 116 g ceftriaxone and a neutrophil nadir of 0.1×109/l. Ceftriaxone was withheld, filgrastim was administered for 3 days and neutrophil normalization was achieved. Although rare, ceftriaxone-induced agranulocytosis may occur in patients on a long course of ceftriaxone therapy. Prompt recognition and drug withdrawal are required. LEARNING POINTS: Ceftriaxone-induced agranulocytosis is rare but may occur in patients with high cumulative doses.Prompt recognition, drug withdrawal and administration of recombinant human granulocyte colony-stimulating factor (rhG-CSF) are the mainstay approach.