Myopathie inflammatoire post-méningococcémie aiguë chez un patient présentant un déficit en properdine : à propos d’un cas

IntroductionMyalgia is a classical sign in invasive meningococcal diseases (IMD), but severe and persistent myalgia following an IMD have never been reported to date.Case reportA 20-year-old man presented with purpura fulminans and meningitis caused by Neisseria meningitidis serogroup Y, revealing properdin deficiency. Although meningitis symptoms improved after antibiotherapy, initial myalgia of the lower limbs increased, associated with mild rhabdomyolysis. Magnetic resonance imaging (MRI) revealed an increased STIR (Short TI inversion recovery) signal of both quadriceps muscles, without abscess. After exclusion of other causes of myopathy, a post-infectious myositis was diagnosed. A four-week course of corticosteroids led to dramatic improvement.ConclusionPost-infectious inflammatory myopathy should be suspected in case of severe and persistent myalgia associated with rhabdomyolysis following an IMD, after exclusion of pyomyositis especially. A short course of corticosteroids seems to be effective.     

Réaction transfusionnelle hémolytique retardée due à un anti-U

Delayed hemolytic transfusion reactions due to anti-U are rare, only two (2) cases having been reported in the literature. We now report a third case : a multiparous black woman without any transfusion history was admitted to hospital for severe microcytic anemia (31 g/l). The patient was group AB negative, the direct antiglobuline test was negative and an anti P₁ cold allo-antibody was present in her serum. Five A, Rh negative, P₂ packed red cells were cross-marched with the sample obtained at admission on January 8, 1988. She was transfused on January 8, 9, 10, 11 and 12. On the 12th of January her hemoglobin level reached 125 g/l. On January 13, the patient presented clinical signs of hemolysis and her hemoglobin fell to 60 g/l within 24 hours. On january 15, the direct antiglobulin test was positive and an antibody found in her serum was reactive with all the red cells of the commercial panel. The sample was referred to our red cell serology reference laboratory. The phenotype of the pre-transfusion sample was found to be Fy(a-b-) M, N, S-s-U-. An anti-U was detected in the eluate and the serum. The patient was transfused with two (2) units of O-P₂, U-red cells obtained from the American Red Cross, Syracuse, and her hemoglobin reached 90 g/l within 48 hours. This is the third reported case of a delayed hemolytic transfusion reaction due to anti-U. This case illustrates the need to perform cross-matches with samples obtained within 48 hours of the scheduled transfusion for patients who have been transfused with blood in the preceding 3 months. Also, this case emphasises the need to recruit U negative blood donors for the Canadian rare donor file.     

Troponinémie faussement positive chez un patient présentant un pneumothorax : cas clinique

A 20-year-old patient is admitted to the emergency room for chest pain occurring in the context of recurrent left complete pneumothorax. Ultrasensitive troponinemia is elevated to 20 times normal. Myocardial distress is attributed to pneumothorax following the negativity of cardiological examinations (EKG, TTE, cardiac MRI). The pneumothorax is drained with a favorable evolution.This is the first reported case of pneumothorax associated with a significant elevation of troponin without ECG change, TakoTsubo syndrome, or myocardial inflammation. Several mechanisms are considered: rotation of the myocardium around its axis, increase in pulmonary vascular resistance with overload of right ventricular pressure, disturbance of coronary blood flow on significant mediastinal compression with decrease in systolo-diastolic myocardial perfusion.     

Hématome rétropéritonéal provoqué par un drain de Redon

The retroperitoneal hematoma is usually secondary in a traumatism. The spontaneous idiopathic shape is rare. We report the case of a 70-year-old woman, operated for calculi of biliary duct and in whom the postoperative consequences were marked by a bleeding of medium abundance exteriorized by the drain of Redon, a deglobulisation and a functional renal insufficiency. The surgical resumption revealed a retroperitoneal hematoma, retrocolique Secondary in a distribution of a bleeding of posterior abdominal wall provoked by a traumatism by the drain of Redon.     

Syndrome de Kasabach-Merrit compliquant un hémangiome géant du foie

Background

Giant hepatic hemangiomas are very rare. These hemangiomas can be complicated exceptionally by the Kasabach-Meritt syndrome. This syndrome associates a coagulopathy and a hemolysis. We report a new case of a giant hepatic hemagioma associated with the Kasabach-Merritt syndrome.

Case report

A 30-year-old-woman without medical history was admitted for right hypochondrium mass. Laboratory tests found pancytopenia, and reduction in the activity of prothrombin and factor V. An ultrasonography and abdominal computed tomography scan disclosed a giant hepatic mass pervading the whole peritoneum. Exploratory laparotomy revealed an hemangioma of almost the entirety of the right liver. A right hepatectomy was realized. Histological examination confirmed the benign nature of the hemangioma. The outcome was favorable, with regression of the Kasabach-Meritt syndrome.

Discussion and conclusion

The association of liver hemangioma and Kasabach-Merritt syndrome is uncommon. The physiopathology of this syndrome is still not understood. The thrombocytopenia is due to a consumption of platelets within the lesion with the absence of coagulation and fibrinolysis. Laboratory tests and imaging permit diagnosis. There is no consensus regarding the treatment which is surgical when the hemangioma is pedunculated, complicated, or when the diagnosis is uncertain.     

Masse cervicale douloureuse révélant un anévrysme de la veine jugulaire interne

Internal jugular vein aneurysms or phlebectasia of the internal jugular vein are considered a benign pathology. They are more and more diagnosed with the evolution of imaging techniques : ultrasonography, angioscanner and MRI. Clinically they are often by chance, however accompanying clinical signs can be seen such as pain, hoarseness or vocal cord paralysis. Several differential diagnoses can be mentioned such as laryngocoele, gill cyst, paraganglioma and hemangioma. They are of unknown etiology with several hypotheses on the etiopathogenesis and on the frequent localization on the right. Conservative treatment can be chosen for small aneurysms and in children. Surgical treatment finds its indication especially in the event of a complication such as thrombosis or for an aesthetic interest; other treatments such as endovascular treatment are being evaluated. We report the case of a 67-year-old woman admitted for a painful latero-cervical mass, and in whom the diagnosis of an aneurysm of the internal jugular vein was suspected and confirmed by ultrasound and CTscan. The patient received successful surgical treatment.     

Quand évoquer un syndrome myéloprolifératif en pathologie vasculaire ?

Thrombotic events are frequent in polycythemia vera (PV) and in essential thrombocythemia (ET). The frequency of thrombotic complications at presentation of PV and ET is nearly 50%. The spectrum of thrombotic complications is broad: thrombosis of arteries, veins and microvessels have been reported. Venous thrombosis can involve all territories but PV and TE are the commonest underlying etiology for Budd-Chiari Syndrome and splanchnic veins thrombosis. Endogenous erythroid-colony formation may be seen in up to 78% of patients thought to have Budd Chiari Syndrome and in 48% of splanchnic veins thrombosis. Major arterial thrombotic complications occur in 20%, especially in the extremities and in cerebral circulation. Microcirculatory disturbances are common in ET, occurring in 29% at presentation and 27% during follow up. In the extremities, erythromelalgia, a characteristic syndrome of red and congested extremities with raised temperature and painful burning sensations, is noticed in 30 to 50% of TE. Other microcirculatory manifestations like acrocyanosis, blue toes, digital gangrene can occur. All of these manifestations are highly sensitive to aspirin. Cerebral microcirculatory symptoms occur in about one-third of patients: migraine, transient visual symptoms like scotomata, blurred vision are characterized by a sudden onset, a short duration and a sequential course. Three kinds of leg ulcers have been described: leg ulceration as a consequence of microcirculatory thrombosis, exceptionally, pyoderma gangrenosum, and leg ulcers attributed to side effects of hydroxyurea. Microcirculatory leg ulcers are the most common: they are painful, inflammatory and sometimes, necrotic. They heal with treatment of SMP. Hydroxyurea-induced leg ulcers are painful, fibrous and multiple in 60%. Cessation of hydroxyurea typically leads to wound healing. The Polycythemia Vera Study Group (PVSG) established diagnostic criteria for PV and TE. Because SMP can have incompletely expressed disease, other authors have proposed determination of serum erythropoietin, examination of bone marrow histology, and spontaneous endogenous colony assays for diagnosis of PV or TE. The individual thrombotic risk depends on elevated hematocrit for PV, age (> 60) and prior thrombosis for PV and TE. Congenital and acquired (antiphospholipid syndrome) thrombotic states probably increase the risk of thrombosis.