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A Julia

Publications and source records attributed to A Julia.

At least 19 recordsLinked to original sources
PubMed2004

Eosinophilic gastroenteritis or eosinophilic chloroma?

Granulocytic sarcoma of the small intestine preceding or as the presenting feature of acute myelogenous leukemia with chromosome 16 abnormalities has been observed in at least 4 patients. We report the case of a patient initially diagnosed with eosinophilic gastroenteritis, responding to corticosteroid treatment for 21 months and eventually transforming into an acute myelogenous leukemia with eosinophilia (M4Eo variant).

Adrenal Cortex Hormones↗
PubMed2001

[Non-simultaneous malignant lymphoma and antiphospholipid syndrome: 4 cases].

OBJECTIVE: We report four cases of non-synchronous antiphospholipid syndrome (APS) and malignant lymphoma, which highlight the complex relationship that seems to exist between these illnesses. METHODS: In a retrospective study conducted in two departments (internal medicine and clinical hematology) of a university hospital, we collected all observations of patients with both APS and malignant lymphoma diagnosed throughout the past decade. RESULTS: An association of APS with malignant lymphoma was recorded in three female and one male patient, median age 42.5 years at the time of diagnosis of the first disease. In each case, the primary APS was diagnosed, with arterial thrombotic events in three cases and venous thrombotic events in one case. One patient had isolated IgG anticardiolipin antibody, whereas the others had a combination of IgG anticardiolipin antibody and lupus anticoagulant with or without IgG anti-beta 2 glycoprotein I antibody. One patient also had an acquired inhibitor to factor VIII:C and a chronic C virus hepatitis. The mean time apparently separating the two illnesses ranged from 18 months to 9 years, but in two cases the diagnosis of APS was delayed due to a progressive, atypical, neurological onset. In two instances, the APS took place at a distance from a cured malignant lymphoma (Hodgkin's disease and nodal large cell B-cell lymphoma), whilst in the others it preceded a B-cell lymphoma (nodal and cutaneous, small cells and primary hepatic, large cells). Treatment resulted in complete haematological response in both cases, with disappearance of anticardiolipin antibody and lupus anticoagulant in the latter following a double autologous peripheral blood stem cell transplantation. In addition, late carcinomas (breast, kidney, thyroid) were seen in two patients. CONCLUSIONS: Our data indicate that the diagnosis of a malignant lymphoma should be considered in patients with a primary APS and peripheral lymph node enlargement or unexplained constitutional symptoms. Conversely, a late onset of arterial or venous thrombotic diathesis after a malignant lymphoma may indicate not only late relapse of malignant lymphoma but also a subsequent APS.

Adult↗
PubMed2001

Platelet function during pregnancy: an evaluation using the PFA-100 analyser.

In clinical practice, the only tests of platelet function are bleeding time and platelet number. Bleeding time lacks sensitivity and specificity but the PFA-100, an in vitro analyser of platelet function may be of value. This study aimed to evaluate any correlation between platelet number and function using the PFA-100 in pregnant women. During a 21-month period, platelet function was evaluated in whole blood as part of the pre-anaesthetic coagulation testing screen with the PFA-100 using collagen and epinephrine (PFA-EPI) or ADP (PFA-ADP) as platelet agonists. Thrombocytopenia was defined as a platelet number less than 150 G litre(-1). The patients were divided into four groups: Group I (n=110) normal pregnancy; Group II (n=38) thrombocytopenia of pregnancy; Group III (n=13) women with pre-eclampsia without thrombocytopenia; Group IV (n=19) women with pre-eclampsia and thrombocytopenia. Results are expressed as mean (SD). Platelet count was not statistically different between Groups II and IV (111.1 (23.1) vs 99.5 (28.0) G litre(-1)). PFA-EPI was statistically increased in Group II (124.0 (26.3) s), Group III (128.3 (17.9) s), and Group IV (143.6 (47.7) s) compared with normal pregnant patients (114.6 (27.3) s, P<0.05, Mann-Whitney U-test). PFA-ADP was statistically increased only in Group II compared with normal pregnant patients (90.5 (18.9) vs 80.2 (11.2) s, P<0.05). PFA values were increased above normal laboratory values in (four of 38) Group II patients and (six of 19) Group IV patients but in no patients in Group III. PFA-ADP results were correlated with platelet count only in Group IV (r=-0.74, P=0.0003). The increased PFA values and the correlation between PFA-ADP and platelet number in hypertensive thrombocytopenic women confirms that platelet function may be decreased in such patients. In patients with pregnancy-induced thrombocytopenia, platelet function may be preserved when the platelet count is as low as 60 G litre(-1).

Adolescent↗
PubMed1997

[Acquired hemophilia caused by autoantibodies against factor VIII coagulation activity. Clinical, biological study and therapeutic indications. Experience based on a study of 9 cases].

STUDY DESIGNS: To describe retrospectively the experience of the Internal Medicine and Clinical Hematology Departments of a University Hospital on adult acquired hemophilia (AH) caused by autoantibody against factor VIII coagulant (f.VIII:C) activity. Diagnosis, clinical datas, associated diseases, treatment and final outcome are described and compared to the published literature. MATERIAL AND METHODS: All cases admitted in both departments since 1989 were enrolled in the study. Clotting analyses comprised clotting times (activated partial thromboplastin time, prothrombin and thrombine times), measurements of f.VIII:C level, antifactor VIII detection and measurement by the Bethesda method assay, Search for an etiologic factor could not be standardized. All patients were followed until cure, sustained improvement, or death. RESULTS: From 1989 to 1996, AH was diagnosed in nine adult patients. Mean age was 76 +/- 24.6 years (range : 65-89) and sex ratio male to female was 2. Eight bleeding episodes occurred in seven patients, resulting consistently in severe hemorrhagic anemia and leading to hemodynamic failure in two, while two others remained asymptomatic for prolonged periods. The initial levels of f.VIII:C ranged from less than 1% to 20%, and the titers of inhibitors ranged from 0.5 to 100 Bethesda units. An underlying disease, to which the appearance of their inhibitor could be related, either concomitantly or up to 1 year later, was found in four cases including (one case each): rheumatoid arthritis, lupus erythematosus with antiphospholipid syndrome, followed by non-Hodgkin malignant lymphoma, relapsing carcinoma and, biliary tract surgery. Six acute bleeding episodes necessitated symptomatic measures, based on activated prothrombin complex concentrates in four instances, with a good response in all cases. Preparation to minor surgical operations was achieved in two asymptomatic subjects by either highly purified factor VIII concentrations infusion or intravenous 1-desamino-8-D-arginine vasopressin, with a good control of local hemostasis in each case. Three received intravenous immunoglobulins, which resulted in success in one, failure in one and, questionable response in the latter. Immunosuppression, mainly with corticosteroids, cyclophosphamid, or both, was given to seven, resulting in disappearance of inhibitor in five (delay to cure ranged from 2 weeks to 10 months), improvement in one, and failure in one (in this latter case, cure was eventually achieved with the anti-Hodgkin disease MOPP chemotherapy). After a 27-month mean follow-up, six patients experienced a sustained complete response and one a sustained partial response to immuno-suppression, two untreated patients remained asymptomatic, two died later from malignancy (carcinoma and myelodysplastic syndrome). CONCLUSION: AH usually presents as a severe or even a life-threatening disease, necessitating prompt and thorough symptomatic measures directed at the cessation of bleedings and prevention of their relapse. In our experience, no death was attributable to AH or its treatment. Immunosuppression is useful in selected cases, but must be carefully discussed, since it can be highly toxic, especially in the elderly. Given the possibility of a delayed onset of some etiologic factors, a prolonged surveillance of each case of idiopathic AH is mandatory.

Aged↗
PubMed1995

Administration of lipid-emulsion versus conventional amphotericin B in patients with neutropenia.

OBJECTIVE: To evaluate the usefulness of a 20% lipid emulsion as a delivery system for amphotericin B (1 mg/mL) administered over 1 hour to patients with neutropenia with hematologic malignancies compared with amphotericin B (0.1 mg/mL) administered in dextrose 5% solution over the same time. DESIGN: A prospective, comparative, randomized, labeled study. SETTING: Hematology unit, pharmacy service, university general hospital. PARTICIPANTS: Twenty patients with neutropenia with hematologic malignancies and proven or suspected fungal infections, 10 in the fat emulsion group (group 1) and 10 in the dextrose 5% group (group 2). MAIN OUTCOME MEASURES: Clinical tolerance (i.e., fever, shaking chills, nausea, blood pressure, pulse rate) and biologic tolerance (i.e., urea, creatinine, sodium, potassium). RESULTS: Clinical tolerance was comparable in both groups although amphotericin B in fat emulsion was better tolerated. Medication for symptoms related to the administration of amphotericin B was given in 6 cases in group 1 and in 8 cases in group 2. There was a statistically significant difference in the urea concentrations between the 2 groups (p = 0.023); there was an observed increase between the initial and the final serum urea (56.8 mg/d in group 1, 79.8 mg/dL in group 2). Statistically significant differences in creatinine serum concentrations (84.9 mumol/L in group 1, 123.8 mumol/L in group 2) (p = 0.047) were found. No differences were found in the antifungal efficacy of the treatment. However, as amphotericin B was started in the majority of cases (75%) as empiric treatment for fever unresponsive to antibiotic therapy, it is difficult to compare the efficacy of both preparations. CONCLUSIONS: The clinical tolerance of lipid-emulsion infusions is similar to that of conventionally administered amphotericin B therapy. Renal toxicity appears to be decreased when the drug is administered in a fat emulsion. This type of preparation permits the reduction of the volume and the time of administration for amphotericin B therapy.

Adolescent↗
PubMed1991

Pregnancy outcome in hematologic malignancies.

By means of a mail questionnaire, information on a series of 56 pregnancies i in 48 women diagnosed with leukemia or lymphoma was collected from ten hospitals. Seven patients conceived while receiving treatment for their neoplasms; in 22 patients, the hematologic disease was diagnosed during pregnancy, and the remaining 27 patients became pregnant after completion of the antineoplastic treatment. When a comparison was made of the evolution of these pregnancies to that of pregnancies in a healthy population, no increase in the incidence of complications was observed: 64% of the pregnancies went to term, 9% resulted in spontaneous abortion, and 5% resulted in premature births. The observed incidence of one major malformation in 56 pregnancies did not differ from the frequency of malformations in the offspring of healthy individuals. There were no fetal losses in six pregnancies in which conception occurred during the first year after chemotherapy. In spite of the inherent limitations derived from the design of this type of study and the type of subject analyzed, the data here support the hypothesis that the cytostatic treatment of hematologic malignancies, if deemed necessary, should not be postponed because of pregnancy. Moreover, the authors agree with advice recommending that no antifolic or alkylating agents be used for prolonged periods and that radiotherapy be avoided, especially to those fields involving the pelvic area.

Abortion, Induced↗
PubMed1989

Ehlers-Danlos syndrome, clotting disorders and muscular dystrophy.

Ehlers-Danlos syndrome includes 11 distinct entities. The diversity of this collagen dysplasia and its combination with other abnormalities make it difficult to understand physiopathologically. A case of Ehlers-Danlos syndrome is reported, which is novel owing to its combination with clotting abnormalities and especially with muscular dystrophy. To our knowledge this has not previously been reported. The patient was a young man aged 16 years who presented with Ehlers-Danlos syndrome satisfying Perelman's diagnostic criteria. His father and two brothers had comparable clinical symptoms, but his mother and sister were healthy. The four male subjects had an increased cephalin-kaolin time, reduced levels of factor VIII and Willebrand's factor (but without haemophilia A or Willebrand's disease), and, especially, an abnormal platelet ATP secretion. The proband alone had muscular disease with bilateral quadriceps fatigability and amyotrophy. The muscle enzyme levels were greatly increased, the electromyographic trace was myogenic, and the biopsy showed severe muscular dystrophy. This new observation poses the problem of the relation between clotting abnormalities and collagen abnormalities in the Ehlers-Danlos syndrome. It is difficult to classify this case within any of the 11 known types because of its muscular manifestations. It may perhaps be a fortuitous combination or an extension of the nosological framework of this syndrome.

Adenosine Triphosphate↗
PubMed1986

Survival after intestinal mucormycosis in acute myelogenous leukemia.

A young woman with acute myelocytic leukemia developed acute lower gastrointestinal bleeding immediately after a first remission induction of her leukemia. After the site of bleeding was located in the descending colon, a necrotic bleeding ulcer was resected. Histologic examination of the ulcer established the diagnosis of gastrointestinal mucormycosis. Treatment with amphotericin B was administered because of the high risk of dissemination. The patient has been followed for 9 months with no evidence of relapse of infection. Survival after gastrointestinal mucormycosis in acute leukemia has not previously been reported in the English language literature. Success in managing mucormycosis depends on the adherence to the recommended principles of early aggressive diagnostic measures, excisional surgery, amphotericin B therapy, and control of the underlying predisposing condition.

Adult↗
PubMed1985

Liver involvement in multiple myeloma.

One hundred twenty-eight records of patients with multiple myeloma were reviewed to assess the incidence and manifestations of liver involvement. Histologic study of the liver was available in 21 patients. Diffuse infiltration of the liver by plasma cells was observed in 10 patients, myeloid metaplasia in four, amyloidosis in two, toxic hepatitis in two, and extrahepatic cholestasis secondary to infiltration of the peripancreatic tissue by plasma cells in one. The clinical signs of plasma cell infiltration of the liver consisted of hepatomegaly in seven patients, mild elevation of liver enzymes in five, and portal hypertension in two. Jaundice was only observed in patients with hepatitis or extrahepatic cholestasis. Liver infiltration by plasma cells did not appear to have a major prognostic significance.

Biopsy↗
PubMed1985

Myelodysplastic syndromes: a study of 101 cases according to the FAB classification.

The myelodysplastic syndromes (MDS) are a group of closely related disorders characterized by chronic cytopenias with cellular marrow, poor prognosis and refractoriness to treatment. We studied 101 consecutive cases of MDS diagnosed over a 7-year period. Peripheral blood (PB) and bone marrow (BM) samples were reviewed and classified according to the proposals of the French-American-British (FAB) cooperative group for MDS. The combined analysis of the initial laboratory features and qualitative haematological abnormalities readily allowed the distinction between the different subgroups. Thirty-two of 79 cases (40.5%) evolved towards other diseases, frequently acute leukaemia (24/79, 30%), or transformed into other MDS (7/79, 9%). In five cases, initially classified as refractory anaemia (RA) or refractory anaemia with ring sideroblasts (RAS), a transitory change to another type of MDS--two chronic myelomonocytic leukaemias (CMML), two refractory anaemias with excess of blasts (RAEB) and one refractory anaemia with excess of blasts 'in transformation' (RAEB-t)--was observed before the evolution towards acute leukaemia. This provides a new link between all these syndromes and increases the number of transitions to other MDS. Overall prognosis was very poor, with differences between subgroups. RA had the best prognosis whereas RAEB-t had the worst one. This study shows that the FAB classification is readily usable and defines well-characterized subgroups of MDS, although there are frequent transitional forms, and as the natural history of the MDS unfolds they may convert into another. The actual poor prognosis and the frequent evolution towards acute leukaemia makes necessary to investigate new methods of treatment for these disorders.

Adult↗