Martial Koenig

Thrombotic microangiopathy with liver, gut, and bone infarction (catastrophic antiphospholipid syndrome) associated with HELLP syndrome

Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome is a thrombotic microangiopathy complicating pregnancy and shares many clinical and biological features with thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Thrombotic microangiopathy is also a pathological feature of catastrophic antiphospholipid syndrome (CAPS). An association between refractory HELLP syndrome and antiphospholipid syndrome (APS) has been reported in a few cases. We describe a 19-year-old woman with APS and multiorgan failure conforming to a diagnosis of CAPS who developed refractory HELLP syndrome.

An unusual cause of spontaneous bleeding in the intensive care unit – mastocytosis: a case report

We report the case of a 39-year old male patient who presented with anaphylactoid shock and diffuse bleeding with prolonged activated partial thromboplastin time at the emergency room. The diagnosis of aggressive mastocytosis was suspected and then confirmed by raised tryptase level and mastocytic infiltration of the bone marrow. The outcome was favorable with supportive measures, antihistamine drugs, and imatinib mesylate.

Acute camptocormia induced by olanzapine: a case report.

IntroductionCamptocormia refers to an abnormal posture with flexion of the thoraco-lumbar spine which increases during walking and resolves in supine position. This symptom is an increasingly recognized feature of parkinsonian and dystonic disorders, but may also be caused by neuromuscular diseases. There is recent evidence that both central and peripheral mechanisms may be involved in the pathogenesis of camptocormia. We report a case of acute onset of camptocormia, a rare side effect induced by olanzapine, a second-generation atypical anti-psychotic drug with fewer extra-pyramidal side-effects, increasingly used as first line therapy for schizophrenia, delusional disorders and bipolar disorder.Case presentationA 73-year-old Caucasian woman with no history of neuromuscular disorder, treated for chronic delusional disorder for the last ten years, received two injections of long-acting haloperidol. She was then referred for fatigue. Physical examination showed a frank parkinsonism without other abnormalities. Routine laboratory tests showed normal results, notably concerning creatine kinase level. Fatigue was attributed to haloperidol which was substituted for olanzapine. Our patient left the hospital after five days without complaint. She was admitted again three days later with acute back pain. Examination showed camptocormia and tenderness in paraspinal muscles. Creatine kinase level was elevated (2986 UI/L). Magnetic resonance imaging showed necrosis and edema in paraspinal muscles. Olanzapine was discontinued. Pain resolved quickly and muscle enzymes were normalized within ten days. Risperidone was later introduced without significant side-effect. The camptocormic posture had disappeared when the patient was seen as an out-patient one year later.ConclusionsCamptocormia is a heterogeneous syndrome of various causes. We believe that our case illustrates the need to search for paraspinal muscle damage, including drug-induced rhabdomyolysis, in patients presenting with acute-onset bent spine syndrome. Although rare, the occurrence of camptocormia induced by olanzapine must be considered.

Éruption et syndrome néphrotique, penser à la syphilis

Resume Introduction Parmi les manifestations cliniques de la syphilis secondaire figure la nephropathie, comme en temoigne cette observation. Observation Chez un homme de 63 ans, a l’occasion d’une syphilis secondaire confirmee par les examens serologiques, est survenu un syndrome nephrotique correspondant a une glomerulonephrite extra-membraneuse de type 1 a la biopsie renale. Commentaires La glomerulonephrite extra-membraneuse est une complication immunologique de la syphilis secondaire. D’evolution presque toujours favorable sous traitement, elle est souvent associee a des manifestations pouvant orienter a tort vers une connectivite.

Une hypergammaglobulinémie considérable

Un homme de 22 ans était adressé en médecine interne pour le bilan d’une fièvre prolongée avec frissons, altération de l’état général et pesanteur de l’hypochondre droit, évoluant depuis six mois environ. Son seul antécédent remarquable était la notion d’une splénectomie à l’âge de sept ans pour un abcès splénique à Salmonella sp. Les examens faits en ville avaient révélé une cytolyse modérée (transaminases à 2N) et une lymphocytose (5 à 6 G/l). L’examen révélait une micropolyadénopathie généralisée et une hépatomégalie sensible. Les examens biologiques initiaux montraient un taux d’hémoglobine à 12 g/dl, des lymphocytes à 4 G/l et des monocytes à 2 G/l. Les plaquettes et l’hémostase étaient normales, la créatininémie à 69 !mol/l, la protéine C réactive à 20 mg/l, les ALAT/ALAT à 8N, les phosphatases alcalines à 4N, les LDH à 3N, les triglycérides à 5 g/l, les gammaglobulines à 60 g/l sur l’électrophorèse des protéines (Fig. 1). De nombreuses perturbations immunologiques étaient détectées avec un test de Coombs positif de type IgG, une présence d’anticorps antinucléaires (1/80 mouchetés) et d’anticorps antimuscles lisses de type antiactine (1/160), un facteur rhumatoïde IgG, et une présence de cryoglobuline non typée. L’imagerie ne révélait qu’une hépatomégalie homogène. La sérologie du VIH était négative et aucun facteur d’immunodépression n’était retrouvé en dehors de la splénectomie.