[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.

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.

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.

[Immunoblastic sarcoma with autoimmune manifestations]. / Sarcome immunoblastique avec manifestations auto-immunes

The authors report a new case of immunoblastic sarcoma with autoimmune manifestations. -- The early stages suggest a virus infection of this lymphoma: -- There is a hemolytic anemia which improves on chemotherapy, -- The pathological appearances are quite special, owing to the absence of malignant characteristics in the early stages of the disease and the difficulty of differentiating the tumour from a Hodgkin lymphoma. Various pathogenic theories are recalled.