[Extensive bone marrow necrosis and major sickle cell syndromes]. / Nécrose médullaire étendue et syndromes drépanocytaires majeurs.

Three cases of extensive bone marrow necrosis in patients with sickle cell disease are reported. All three patients presented severe bone pains with severe anaemia (haemoglobin value less than 5 g/dl) and high increased of LDH serum values (upper than 20 fold normal value). Bone marrow aspirate and biopsy showed typical signs of necrosis. The extent of necrosis was evaluated by reticuloendothelial scan obtained with 111In chloride. Treatment required transfusions of phenotyped red blood cell concentrates. Favourable outcome was observed in all patients.

[Clinical and epidemiological aspects of Horton disease based on survey methods in ophthalmology or internal medicine]. / Aspects cliniques et épidémiologiques de la maladie de Horton selon le milieu de recrutement, ophtalmologique ou de médecine interne.

We conducted a retrospective study of 29 consecutive cases of temporal arteritis, all with definite histological diagnostic criteria. The epidemiological aspects of the main clinical and biological features were compared according to their mode of recruitment, the patients being hospitalized either in an ophthalmological unit (n = 15), or in an Internal Medicine unit (n = 14). The level of fever and of sedimentation rate were significantly less in the ophthalmological group (respectively p < 0.001 and p < 0.02), a third of which is represented by the purely ophthalmological manifestation called "occult temporal arteritis". Among the ophthalmological manifestations, despite the fact that the recruitment bias prevented any strict epidemiological comparison, we were surprised to find no significant difference between the two groups concerning the frequency of transient ophthalmological manifestations, which confirmed in half the patients their reputation of forerunners of irreversible lesions. Thus, the presentation of temporal arteritis differs according to its ophthalmological or Internal Medicine recruitment. We were unable to determine a precise chronology difference in the principal manifestations between these two groups. The severity of the ocular manifestations justifies looking for temporal arteritis in all cases of transient ophthalmological manifestations, even if clinically totally isolated.

Improvement of microcirculation abnormalities in sickle cell patients upon buflomedil treatment.

The efficacy of the vasoactive compound buflomedil on previously described microcirculation abnormalities observed in sickle cell disease (SCD) was investigated using bulbar conjunctival angioscopy (BCA) and laser doppler flux metering. Eleven sickle cell disease patients at steady state were studied in a double-blind randomized parallel-versus-placebo study (5 patients were given placebo and 6 patients received 600 mg buflomedil per day for 21 days after a washout period of 15 days). The blood flux score measured by BCA improved, indicating reduced entrapment of sickle red blood cells. There was a striking improvement of reactive alterations of vasomotion in the buflomedil group, particularly in postocclusive reactive hyperemia; the half return time was also significantly reduced. These results encourage further studies with buflomedil to investigate long-term effects of this compound in SCD, potential prevention of acute vaso-occlusive events, and degenerative complications.

[Acute aseptic arthritis in major sickle cell syndromes]. / Arthrites aiguës aseptiques au cours des syndromes drépanocytaires majeurs.

We report 9 cases of febrile aseptic arthritis which occurred in 6 adults presenting with major sickle cell disease. The monoarticular arthritis affected the knee in 8 cases and the ankle in 1 case. Body temperature ranged from 38 degrees C to 39.5 degrees C in 7 cases, and neutrophilia (12.7 to 24 x 10(9)/l) was noted on six occasions. The always sterile synovial fluid contained 100 to 1700 cells/mm3 in 6 cases and 5200 to 12,500 cell/mm3 in 3 cases. Uricacidaemia was normal, and a search for crystals in the synovial fluid gave negative results. Bone scintigraphy and magnetic resonance imaging, performed in 3 patients, revealed the presence of a juxta-articular cortical bone infarct, whereas standard radiography was normal. In all patients, a brief immobilization and treatment with non-steroidal anti-inflammatory drugs resulted in complete cure in less than 10 days. These cases illustrate the characteristics of sickle cell arthritis, a rare complication of major sickle cell disease. Clinically, the condition resembles septic arthritis, being febrile and showing granulocytosis, but articular needle aspiration corrects the diagnosis. The mechanisms underlying these disorders have not yet been elucidated, but the bone scintigraphy and MRI performed in our patients are in favour of a reaction to a juxta-articular bone infarct.

Comparative evaluation of laser Doppler flux metering, bulbar conjunctival angioscopy, and nail fold capillaroscopy in sickle cell disease.

Eleven adult patients with stable sickle cell disease (SCD) were investigated twice at a 15-day interval by nail fold capillaroscopy (NFC), bulbar conjunctival angioscopy (BCA), and laser Doppler flux metering (LDF). NFC and BCA are essentially descriptive. NFC revealed, in some patients, network patterns similar to those observed in vasculitis. BCA provides a reproducible method for the visual assessment of the microcirculation. When using a rating scale with five criteria it identified abnormalities related to both altered red blood cells and vascular morphology but failed to discriminate between patients. LDF, in contrast, is quantifiable and showed reproducible characteristic reactive alterations of vasomotion in response to ischemia after 3 min of occlusion. The SCD patients had a delayed response, best measured by the recovery half time, which was four to five times longer than that of normal subjects. In addition, the cold provocative test was always abnormal, as evidenced by a significant drop in the contralateral flux, and was often poorly tolerated. The observed trends suggest that BCA and LDF should be performed in a large cohort of patients, both in the steady state and during vasoocclusive crises. These methods seem to be useful complementary noninvasive procedures to cellular and clinical evaluation monitoring for therapeutic trials.

Severe pneumococcal sepsis and meningitis in human immunodeficiency virus-infected adults with sickle cell disease.

Pneumococcal infection is frequent in children with sickle-cell disease (SCD) but is less common in adults. Infection due to human immunodeficiency virus (HIV) is also a risk factor for pneumococcal infection. The purpose of this study was to determine the clinical course of HIV infection in adults with SCD. During a 6-year period, 283 adults suffering from SCD were screened for HIV type 1 and HIV type 2 infections. The clinical course of HIV infections in these patients was retrospectively analyzed at the term of the study. HIV type 1 infection was detected in eight patients. The mean follow-up period after diagnosis of HIV infection was 4.6 years. None of the patients had AIDS. Five episodes of severe pneumococcal infection were observed in four of these eight patients (septic shock in two patients and three episodes of meningitis in two patients); two patients died of meningitis. Only one severe pneumococcal infection (meningitis) was observed in the 275 non-HIV-infected patients with SCD (P = .000001). Our results show that pneumococcal infection appears to occur frequently and is often serious in HIV-infected adults with SCD.

[Value of a fraction of low molecular weight heparin, enoxaparine, in patients at high risk of hemorrhage and thrombosis]. / Intérêt d'une fraction d'héparine de bas poids moléculaire, l'énoxaparine, chez des patients à hauts risques hémorragique et thrombotique.

Treatment of patients with both thromboembolic manifestations and acute bleeding episodes is poorly defined. The use of low molecular weight heparin fractions has been shown to provide prophylactic antithrombotic activity in humans, with the possibility of fewer hemorrhagic complications because of their low anticoagulant activity and weaker action on platelets. An open trial was carried out in 50 patients. 47 of whom had a recent history of thromboembolic accident (34 venous thrombosis, including 13 with pulmonary embolism, 9 systemic arterial episodes, 4 cardiac emboli) and 3 who required prophylaxis. Conventional heparin therapy was contraindicated in all patients: 30 because of major digestive or muscle hemorrhage and 20 with high hemorrhagic risk (neurosurgery, deep thrombocytopenia). Enoxaparine (Laboratoire Pharmuka, Gennevilliers) was administered as 1 mg/kg/24 hours as 2 divided injections with adjustment to maintain an anti-Xa activity of 0.1 to 0.4 IU/ml. Antithrombotic efficacy as function of type of pathology was rated excellent in all patients, while tolerance, evaluated similarly by tests chosen as a function of type of hemorrhage, was good: one patient had moderate bleeding after accidental overdose and 4 patients minor bleeding. Enoxaparine by subcutaneous injection could represent a new therapeutic approach by improving the efficacy/risk ratio in this type of patients.

Allopurinol hypersensitivity syndrome as a cause of hepatic fibrin-ring granulomas.

Hepatic fibrin-ring granulomas were found in a 35-yr-old man who developed fever, myalgias, rash, eosinophilia, and abnormal liver function tests 4 wk after the beginning of allopurinol treatment. All clinical and biochemical abnormalities spontaneously resolved within 6 wk after cessation of therapy. There was no evidence for Q fever or Hodgkin's disease, which are the recognized causes of hepatic fibrin-ring granulomas. It is suggested that allopurinol hypersensitivity might be an additional cause of these peculiar granulomas.

[Idiopathic peliosis hepatis: case report and review of the literature (author's transl)]. / Péliose hépatique idiopathique: analyse d'un cas et revue de la littérature.

Peliosis hepatis is a very uncommon disease defined by pathological lesions located in the liver and in the spleen characterized by blood filled lakes irregularly disseminated throughout parenchyma, without abnormalities of the hepatic veins or the vena cava system. A new case, regularly followed 3 years after the initial diagnosis is reported. Recent reports of the literature have pointed out etiologic factors, particularly iatrogenic factors. None of the reported factors could be found out in the present case. This emphasizes the need of epidemiological studies of such a rare disease (case-control study in a multicentric basis). Finally a successful diagnosis approach is proposed: transvenous (transjugular) liver biopsy. This procedure prevents hemorrhagic risk which could be a complication of routine transcutaneous liver biopsy in this disease.

[Paroxystic hypertension after ingestion of gruyere cheese during isoniazide treatment: a report on two cases (author's transl)]. / Hypertension artérielle paroxystique provoquée sous isoniazide par l'ingestion de gruyère: deux cas.

Paroxystic hypertension was observed in two patients receiving isoniazide treatment, after they had ingested gruyere cheese. It is highly probable that isoniazide was responsible, as hypertension did not reoccur under the same conditions after interrupting treatment. One patient developed a vasomotor reaction identical to that which sometimes occurs after the eating of foods rich in tyramine by patients taking monoamine oxydase inhibitors. It could be that in our patients this phenomenon was consecutive to the inhibition of plasma monoamine oxydase by the isoniazide. It was not due to an overdose of isoniazide. This unusual reaction suggest that patients taking isoniazide should be questioned whether they have had post-prandial vasomotor flushes, and if so, advised to avoid foods rich in tyramine.