[Refractory thrombotic thrombocytopenic purpura revealing an epiglotis neoplasia]. / Purpura thrombotique thrombocytopénique réfractaire révélant un cancer de l'épiglotte.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy with neurological disorder which incidence is relatively rare. Since the discovery of the ADAMTS 13 in 1996 and the changes in the treatment (plasma exchanges and rituximab), the prognosis of the TTP has considerably improved. We report the case of a 60-year-old man with a refractory TTP. The subsequent discovery of an epiglottis neoplasia and its treatment saved the patient.

[Extrahepatic symptoms in the course of GBV-C/HGV infection]. / Manifestations extrahépatiques au cours de l'infection par le GBV-C/HGV.

The GBV-C/HGV virus has clearly established transmission modes, mainly blood contamination, and occasionally sexual transmission. It is frequently found among transfused patients, intravenous drug abusers, and hemodialysis patients and often associated with HCV. Its hepatic pathogenicity is very weak, marked by a moderate and transitory cytolysis. Chronic carriage is possible, but does not lead to chronic hepatitis. Carriage can be maintained before the virus disappears. The authors report the case of a patient presenting with pleuropericarditis after a blood transfusion without any other etiology than infection by GBV-C/HGV virus. The possible extrahepatic pathogenicity of the virus is suggested. This hypothesis was rarely put forward.

[Acute adrenal insufficiency during unilateral adrenal hemorrhage secondary to the antiphospholipid syndrome]. / Insuffisance surrénale aiguë et hématome surrénalien unilatéral au cours d'un syndrome des antiphospholipides.

INTRODUCTION: Acute adrenal insufficiency during antiphospholipid syndrome is usually due to a bilateral adrenal hemorrhage. For a patient, an unilateral involvement has induced an acute adrenal insufficiency. CASE REPORT: A patient with an antiphospholipid syndrome has developed an acute adrenal insufficiency with an unilateral adrenal hemorrhage. This complication was caused by a concurrent steroid therapy, for an associated lupus, which resulted in a suppression of the contralateral adrenal gland. CONCLUSION: In patients with antiphospholipid syndrome and concurrent steroid therapy, usually for lupus, unilateral adrenal hemorrhage can cause acute adrenal insufficiency. Withdrawal of steroid might be difficult in case of undetected bilateral hemorrhage.

Discrepancy in virological and biochemistry response of patients with chronic hepatitis HCV positive on treatment with PEG-IFN plus ribavirin.

Combination therapy of PEG-IFN alpha-2a o alpha-2b plus ribavirin represents a further improvement in treatment of chronic hepatitis HCV+ with a sustained virological response (SVR) either in monotherapy (25-39%) either in association with ribavirin (59-56%). SVR is highly predictable: 75% of all patients who achieve viral clearance at week 12 (EVR), if they had an adherence > 80% of planned therapy, they become sustained viral responders. In spite of virological response, 16-34% of patients on PEG-IFN monotherapy have high value of ALT, and this make them to reduce adherence. 62 patients whith chronic hepatitis HCV+ and no corrhosis, have been treated for 48 weeks with PEG-IFN and ribavirin to evaluate discrepancy incidence between virological (HCVRNA < 200UI) and biochemical (normal value of ALT) response of patients treated with PEG-IFN plus ribavirin and to verify the impact that stuch discrepancy can produce on SVR of treated patients. Our preliminary data confirm that PEG-IFN bring a superior virological response than biochemical one, either on naive patients either on experienced ones even with ribavirin in association. It will be useful to verify if this discepancy cause a superior SVR as already reported by several studies. Even the follow-up of our 5 discordant patients confirm this trend.

Infusional CDE with rituximab for the treatment of human immunodeficiency virus-associated non-Hodgkin's lymphoma: preliminary results of a phase I/II study.

Infusional CDE (cyclophosphamide, doxorubicin, etoposide; iCDE) is one of the most effective chemotherapeutic regimen for human immunodeficiency virus (HIV)-associated non-Hodgkin's lymphoma (NHL), with a complete remission rate of 46% and a median overall survival of 8.2 months (Sparano JA, Blood 1993; 81:2810). Since the majority of HIV-associated NHL are CD20-positive we reasoned that the addition of rituximab to iCDE (R-iCDE) could also improve the poor outcome of these patients. As a first step we investigated the safety of R-iCDE in a phase I/II study. Thirty patients with aggressive HIV-associated NHL were enrolled between June 1998 and October 2000. Characteristics of 29 evaluable patients were: median age: 38 years (range 29-65 years); male sex 24/29; histology: DLCL 16 (55%), Burkitt 10 (35%), ALCL 2 (7%), unclassified 1 (3%); stage: I (35%), II (10%), III (10%), IV (45%); International Prognostic Index: 0, 1 (59%), 2 (24%), 3 (17%), 4, 5 (0); CD4 count: median 132/ mm3 (range 3-470/mm3). Patients received rituximab (375 mg/m2) in conjunction with iCDE (five or six cycles). All patients were treated with G-CSF and highly active antiretroviral therapy (HAART). Twenty-six of 29 patients received treatment as planned, while chemotherapy had to be discontinued in three patients (2 persistent thrombocytopenias, 1 cerebral hemorrhage). Grade 3 or 4 toxicity was observed as follows: neutropenia 79%, anemia 45%, thrombocytopenia 34%, bacterial infection 34%, opportunistic infection 7%, mucositis 17%. A dose reduction was necessary in 22%. Complete remission was achieved in 86% of the patients, partial remission in 4%. Ten percent had progressive disease. After a median follow-up of 9 months the median overall survival is not reached. The actuarial survival at 2 years is 80% and the actuarial progression-free survival is 79%. Four of 29 patients (14%) have died, three from NHL and one from cryptosporidiosis. These findings suggest that the combination of rituximab with iCDE in patients with HIV-associated NHL is safe and feasible and that the addition of the anti-CD20 antibody does not increase the risk for infections. The high complete remission rate also indicates a potential therapeutic benefit and warrants further randomized trials.

[Acute interstitial pneumopathy with fever caused by hydroxyurea]. / Pneumopathie interstitielle aiguë fébrile à l'hydroxyurée.

BACKGROUND: Hydroxyurea has few side effects excepting the known bone marrow toxicity. Fever with or without pneumonia has occasionally been reported. CASE REPORT: A patient given hydroxyurea for polycythemia suddenly developed severe interstitial pneumonia with fever and hypoxemia. All bacteriological tests were negative and an empirical antibiotic regimen was ineffective. Fever recurred after reintroducing hydroxyurea and definitive cure was achieved after its withdrawal. The clinical course was rapidly favorable without the need for corticosteroids. DISCUSSION: Fever, and in some cases interstitial pneumonia, in patients given hydroxyurea generally suggests an infection. However, 15 cases of pneumonia have been reported as caused by hydroxyurea in patients treated for a myeloproliferative syndrome. Delay to onset is 3 to 8 weeks after initiating treatment. The course is favorable after withdrawal, with or without corticosteroids. Fever may be the only sign of a drug reaction, resolving with withdrawal and recurring at re-challenge. The underlying mechanism remains unknown. Definitive cure can be achieved by discontinuing hydroxyurea, avoiding the need for further investigations.

Detection of lymphotropic herpesvirus DNA by polymerase chain reaction in cerebrospinal fluid of AIDS patients with neurological disease.

Cerebrospinal fluid (CSF) samples from 49 acquired immunodefficiency disease syndrome (AIDS) patients with a central nervous system (CNS) disease were examined by polymerase chain reaction (PCR) to evaluate the association between the positivity for cytomegalovirus (CMV) and Epstein-Barr virus (EBV), and clinical diagnosis of a CNS disease. Frequency and clinical relevance of detection of DNA of human herpesviruses 6 (HHV-6), 7 (HHV-7) and 8 (HHV-8) were also determined. DNA of one or more of the following viruses was found in 26 of 49 patients (53%): CMV in 16 (33%), EBV in 13 (27%), human herpesvirus 6 (HHV-6) in 2 (4%), human herpesvirus 7 (HHV-7) in 1 (2%), and human herpesvirus 8 (HHV-8) in 1 (2%). The CMV detection was significantly associated with encephalitis and peripheral neuropathy (7/16 vs. 2/33, p = 0.003), while EBV with primary CNS lymphoma (P-CNSL) (8/13 vs. 0/36, p < 0.0001). HHV-6 DNA was found in CSF of two patients with neuroradiological features suggestive of cerebral lesions. HHV-8 or HHV-7 DNA was detected in the CSF of patients with unexplained neurological symptoms. This study confirms that the PCR analysis of CSF is a valid tool for the diagnosis of neurological diseases associated with CMV and EBV. On the other hand, HHV-6, HHV-7 and HHV-8, instead, were rarely detected in CSF of AIDS patients and have certainly no correlation with the CNS disease found.

[Intracranial hypertension in severe diabetic ketoacidosis with coma. Two cases]. / Hypertension intracrânienne au cours de l'acidocétose diabétique grave avec coma. Deux observations.

We observed two cases of severe diabetic ketoacidosis with coma and shock. In one case, coma was present at admission and in the second occurred within 15 hours. In both cases, intracranial hypertension was confirmed with an extradural captor. These findings are in agreement with observations of brain oedema in diabetic ketoacidosis with coma. Clinical data suggest that brain oedema may occur after a latency period but that clinical expression is much more rare, perhaps favoured by treatment (excessive rehydratation, alkalinization, too sharp drop in blood glucose level). In our cases, despite major fluid infusion, shock persisted requiring norepinephrine. This shock could have been the expression of the severe ketoacidosis or have resulted from an underlying infection. In case of sudden onset coma, a regularly encountered manifestation of brain oedema, respiratory assistance and mannitol infusion must be instituted rapidly. With this type of management, it should be possible to improve the severe prognosis of brain oedema in diabetic ketoacidosis.

[Candidal bezoar of the urinary tract during Candida albicans septicemia]. / Bézoard candidosique des voies urinaires au cours d'une septicémie à candida albicans.

We report a case of an obstructing fungus ball of the urinary tract. Typical predisposing factors were present, i.e. bladder catheter, vesico-ureteral reflux, urinary infection, parenteral nutrition, prolonged antibiotherapy, hyperglycemia. Candida albicans septicemia was noted. Diagnosis was made by percutaneous nephrostomy under echographic control allowing urinary sampling (C. albicans) and anterograde pyelography showing an ureteral stenosis. Urinary drainage and local and general administration of amphotericin lead to a complete resolution of the fungus ball and the systemic candidiasis.

Polyneuropathy: potential cause of difficult weaning.

Severe critically ill polyneuropathy (CIP) is a neurologic syndrome with potential effects on ventilatory weaning. Fifteen CIP patients with the following clinical criteria were diagnosed: limb weakness, amyotrophy, and reduced deep tendon reflexes. Electromyogram (EMG) confirmed polyneuropathy by the following signs: denervation, normal nerve conduction velocities, normal distal latencies, and decreased compound action potential. It was observed that motor signs were more important than sensitivity. EMG performed during ICU hospitalization showed axonal abnormalities. Ventilation and mean duration of weaning were unusually long. Four patients had EMG a few years after discharge; the recovery was delayed and incomplete. The present study suggests that CIP is a multifactorial disease.

[Electrophoresis of cholinesterases of the cerebrospinal fluid in the Guillain-Barré syndrome]. / Electrophorèse des cholinestérases du liquide céphalorachidien dans le syndrome de Guillain-Barré.

Polyacrylamide gel electrophoresis of cholinesterase from cerebrospinal fluid was performed in 22 patients with Guillain-Barré syndrome. Fifteen of these patients had an abnormal cerebrospinal fluid with emergence of a second electrophoretic migration band corresponding to non-specific cholinesterase. Among 182 patients with a variety of diseases who served as controls, only one presented with this abnormality. From these data the sensitivity and specificity of cerebrospinal fluid cholinesterase electrophoresis were calculated at 68 and 99 percent respectively. The second migration band seems to appear early in the course of the disease and disappears when the patient is cured. Moreover, the occurrence of this band is correlated with the severity of the condition, as shown by a greater number of patients under artificial ventilation and by a longer stay in intensive care unit. Cerebrospinal fluid electrophoresis could be used as a prognosis factor.

[Diagnosis of amniotic embolism by bronchoalveolar lavage]. / Diagnostic de l'embolie amniotique par lavage broncho-alvéolaire.

A broncho-alveolar lavage performed in a patient who developed pulmonary oedema a few hours after a caesarian section, produced foetal squamae which led to the diagnosis of amniotic fluid embolism.