Parovirus B19 infection in an HIV-infected patient with febrile pancytopenia and acute hepatitis.

The case of a 34-year-old male patient with HIV infection referred for severe febrile pancytopenia is reported. Clinical and laboratory evaluations revealed acute hepatitis B infection and concomitant parvovirus B19 infection. The patient died just before treatment with immune globulin was to be administered. Parvovirus B19 has been found to cause a wide variety of hematologic disorders such as neutropenia, thrombocytopenia, pancytopenia, and hemophagocytic syndrome. The role of parvovirus B19 in the pathogenesis of bone marrow or liver involvement is briefly discussed.

Diabetes insipidus revealing acute myelogenous leukaemia with a high platelet count, monosomy 7 and abnormalities of chromosome 3: a new entity?

We describe three cases of acute myeloid leukaemia revealed by diabetes insipidus. The patients were 42, 38 and 39 yr old and they had marked hyperleukocytosis, circulating immature granular cells and a normal or elevated platelet count. The leukaemia was type AML-M2 according to the FAB classification. Cytogenetic studies showed inversion of chromosome 3 (q21;q26) in 2 cases and a translocation (3;3)(q21;q29?) in the remaining case, both associated with monosomy 7. All the cerebral CT scans were normal. Complete remission was never achieved, and all three patients survived less than 14 months. Desmopressin therapy was active but treatment could not be reduced. The association of dysmegakaryopoiesis with a chromosome 3 abnormality and diabetes insipidus is probably not fortuitous and could represent a new entity.

alpha-Interferon therapy for congenital dyserythropoiesis type I.

We report the case of a 28-year-old female followed for congenital dyserythropoiesis type I which required repeated transfusions. Alpha-2a interferon treatment was started because of post-transfusion chronic viral hepatitis type C. Following this treatment, haemoglobin level increased and reached normal value during the 24 weeks of interferon treatment. When interferon therapy was stopped, haemoglobin level returned to previous values, requiring more transfusions. Resumption of interferon therapy resulted again in a complete normalization of haemoglobin level. Erythrokinetic studies demonstrated a striking reduction of the ineffective erythropoiesis, and electron microscopy study a reduction in nuclear structure abnormalities. To our knowledge, this is the first report of the efficacy of interferon in congenital dyserythropoiesis.

First study of immunoglobulin and T cell receptor gene rearrangements in chronic and acute lymphoblastic leukemias from Tunisia.

We report the first characterization at the immunological and molecular level of 12 cases of chronic lymphocytic leukemia (CLL) and acute lymphoblastic leukemia (ALL) from Tunisia. Our results show biallelic IgH gene rearrangement in B-CLL (6/6). A high ratio of T-ALL (4/6) was observed in Tunisian ALL leukemias. One T-ALL expressed CD10 (common ALL) which has already been found in some other cases of T-ALL. We report the occurrence of T cell receptor (TCR) beta and/or gamma gene rearrangements in two precursor B-ALL patients who had normally rearranged Ig genes. In one precursor B-ALL case, multiple rearranged IgH and TCR gamma bands allowed the identification of three clones. Such an oligoclonal ALL is interesting since only rare biclonal TCR beta or gamma gene rearrangements have been described.

[Syndrome of hemophagocytosis associated with infections]. / Syndrome d'hémophagocytose associé aux infections.

Infection-related hemophagocytic syndrome was originally described in viral processes by Risdall in 1979. Recent reports have suggested associations of this syndrome with bacterial, parasitic and fungal infections. It occurs generally in immunosuppressed patients. The clinical and biological manifestations are not specific. The diagnosis is based on morphologic examination of the bone marrow showing a benign proliferation of histiocytes with hemophagocytosis. Treatment is symptomatic, however when an infectious etiology is found a specific treatment must be applied. This pathology has a poor prognosis, with a fifty percent mortality rate. When evolution is favorable, relapses are exceptional. The precise pathophysiological mechanism has not yet been determined. A better understanding of the cytokines' role should permit to consider new therapeutic routes.

CD23 antigen density is related to serum gamma globulin level, bone marrow reticulin pattern, and treatment in B chronic lymphocytic leukemia.

The CD23 antigen density was evaluated by a cytofluorometric technique in 55 patients with chronic lymphocytic leukemia. The quantification method was based on the use of biological standards in indirect immunofluorescence. The CD23 antigen density was correlated with the percentage of CD23 positive cells, but antigen density appeared to be a more informative parameter. CD23 antigen density was lower in stage B than in stages A or C patients, and higher in patients undergoing chemotherapy or previously treated than in untreated patients. There was a significant negative correlation between CD23 antigen density and serum gamma globulin and IgG levels, that existed only in patients in an advanced stage of the disease. CD23 antigen density was higher in patients with abnormal bone marrow reticulin pattern. Serum gamma globulin level was lower in these patients, as well as in patients with prognostically unfavorable histologic bone marrow infiltration pattern. These data emphasize the interest of antigen density as an additional parameter and the complex relationship between CD23 expression, hypogammaglobulinemia, bone marrow histologic findings, and treatment in chronic lymphocytic leukemia.

Hematologic values of fetal blood obtained by means of cordocentesis.

Fetal blood values were evaluated from 541 cordocenteses. Simple regressions were used to find a correlation between blood values and gestational age. We found a linear increase in hemoglobin concentration and hematocrit throughout gestation; a linear decrease of the mean corpuscular volume with the gestation was evidenced as a regular decrease in mean corpuscular hemoglobin. Lastly, the mean corpuscular hemoglobin concentration was constant during the gestation; a linear increase of the platelet count and the nucleated cells was also evidenced. We suggest that each fetal medicine unit should have its own reference ranges. It will permit to accurately diagnose fetal infection, fetal anemia, or any fetal disease where alterations of hematopoiesis have been described.

X-linked alpha-thalassemia/mental retardation syndrome. Linkage analysis in a new family further supports localization in proximal Xq.

Linkage analysis was performed in a three generation family with three males affected by the recently delineated X-linked form of alpha-thalassemia/mental retardation syndrome (ATR-X). Results are in agreement with the linkage study reported by Gibbons et al in 1992 and further confirm that the ATR-X gene is located in proximal Xq. Positive LOD scores were obtained for several markers situated in the pericentromeric region. A maximum LOD score of 2.09 at a recombination fraction of 0 was obtained for DXS453 located at the boundary q12-q13.1. The nearest flanking loci demonstrating recombination with the disease locus were AR at Xq11.2-q12 on the centromeric side and DXS72 at Xq21.1 on the telomeric side. Consequently the authors were able to reduce the previously defined candidate region for the gene location. Their results are compatible with a distal boundary at Xq21.1 instead of q21.31.

X-linked alpha-thalassemia/mental retardation (ATR-X) syndrome. Report of three male patients in a large French family.

Three male patients with X-linked alpha-thalassemia/mental retardation (ATR-X) syndrome in a large French family are reported. Diagnosis was suspected on particular craniofacial dysmorphism associated with severe mental retardation and X-linked transmission. Hematological investigations, and in particular presence of Hb H inclusions in two of the boys, confirmed diagnosis. The clinical, hematological and radiological features are discussed in order to better define what appears to be a characteristic phenotype.

A study of 15 cases of primary mediastinal lymphoma of B-cell type.

Fifteen cases of pure supradiapragmatic lymphoma with initial prominent antero-superior mediastinal involvement displaying a B-cell pattern of reactivity were studied. These cases occurred in six men and nine women with a median age of 33 years at diagnosis (range, 23 to 75 years). Supradiapragmatic peripheral lymphadenopathies were present in three cases, and intrathoracic extension to the lung, pericardium, or pleura was possible. In five cases a thymic origin was obvious. All cases exhibited a B-cell pattern of differentiation, with a great variety of histopathologic aspects associated with a high frequency of fibrosis and/or necrosis. Hodgkin's disease was initially misdiagnosed in four cases. The evolution was purely local, with extrathoracic extension in five cases, at the ultimate phase of the disease. The prognosis appeared to be poor with only five patients still alive at a median survival time of 16 months. A complete chemoresistance and radio-resistance was observed in seven cases; only two complete remissions were achieved with aggressive chemotherapy. Prolonged remission could be achieved after surgical reduction of the mass. Primary B-cell mediastinal lymphoma appears to be a distinct clinical entity with local evolution and resistance to therapy. A new therapeutic regimen, which could include surgery in some cases, should be found for this disease.

Recombinant human erythropoietin: 18 months' experience in hemodialysis patients.

It has been shown that the regular administration of erythropoietin (EPO) permits the correction of anemia in end-stage renal failure patients. We analyzed the effect of chronic administration of EPO in 13 stable, regularly dialyzed end-stage renal failure patients over an 18-month period. The effects of EPO were evaluated according to standard criteria including clinical status, blood pressure control, hematology and biochemistry data, protein nutritional status, and dialysis efficiency. Following a 2-week control period, EPO was administered intravenously (IV) after the dialysis session according to a two-phase protocol. The first period (correction phase) consisted of a stepwise EPO dose increment, starting at 3 x 24 IU/kg/wk and doubling the dose every 14 days according to hemoglobin response in order to achieve a target hemoglobin level of approximately 11.0 g/dL (110 g/L). In the second period (maintenance phase) EPO dose was optimized to maintain the hemoglobin level between 100 and 110 g/L (10.0 and 11.0 g/dL), by adjusting either the unit dose or the frequency of injection. Anemia was corrected in all patients within 11 weeks, with EPO dose increasing from 72 to 360 IU/kg/wk. The stabilization of hemoglobin was achieved with an average EPO dose of 275 IU/kg/wk (50 to 476 IU/kg/wk). Concomitantly, a subjective and clinical improvement was noted in all patients. The dialysis efficacy remained in an acceptable range throughout the study, falling significantly (approximately 10%) through the first 3 months of treatment to stabilize at an effective urea clearance of approximately 120 L/wk. The dietary protein intake calculated from urea kinetic modeling ranged between 1.1 and 1.2 g/kg/d.(ABSTRACT TRUNCATED AT 250 WORDS)

Erythropoietin-induced changes in protein nutrition: quantitative assessment by urea kinetic modeling analysis.

To evaluate objectively the effects of recombinant human erythropoietin (rHuEPO) administration on nutritional status in stable dialyzed patients, we used urea kinetic modeling (UKM) analysis and dietary protein intake evaluation by dietary assessment. Fifteen patients (9 females, 6 males; mean age 46.9 +/- 15.6 years) dialyzed for 9.4 +/- 6.3 years were studied longitudinally for 18 months, consisting of a control period (6 months) and an rHuEPO treatment period (12 months). Treatment modalities based on 3 weekly sessions were hemodialysis in 12 patients (6 cuprophane, 3 cellulose acetate and 3 highly permeable membranes), hemodiafiltration in 2 patients and postdilutional hemofiltration in 1 patient. Bicarbonate buffered dialysate was used in 9 patients and acetate in 6 patients. Urea kinetic modeling using a single-pool model was performed monthly over 1-3 cycles. rHuEPO was administered intravenously at the end of dialysis according to a two-phase protocol: (1) correction of anemia by stepwise increment of rHuEPO dose, and (2) maintenance dose to keep hemoglobin at 10-11 g/dl. rHuEPO administration corrected anemia in all patients, improving their general clinical condition. Dialysis efficacy was significantly reduced (15%) after the 3rd month of rHuEPO therapy. Clearnces were restored by increasing dialysis time and/or improving dialyzer performances, and adequacy of dialysis was maintained in all patients. During the 12 months of rHuEPO therapy, the protein catabolic rate remained stable at 1.2 g/kg/24 h in spite of an increase in appetite. At the same time, dry body weight increased significantly after 9 months, and the ratio dietary protein intake/protein catabolic rate a gross estimation of nitrogen balance, increased.(ABSTRACT TRUNCATED AT 250 WORDS)

[Recombinant human erythropoietin: 18 months of continuous use in substitution hemodialysis]. / Erythropoïétine humaine recombinante: 18 mois d'utilisation continue en hémodialyse de suppléance.

It has been shown that the regular administration of EPO permits the correction of anemia in end stage renal failure patients. We analyzed the effect of chronic administration of EPO in 13 stable, regularly-dialysed end stage renal failure patients over an 18 month period. The effects of EPO were evaluated according to standard criteria including clinical status, blood pressure control, hematology and biochemistry data, protein nutritional status and dialysis efficiency. Following a 2 week control period, EPO was administered intravenously after the dialysis session according to a 2 phase protocol. The first period (correction phase) consisted of a stepwise EPO dose increment, starting at 24 IU/kg x 3 times and doubling the dose every 14 days according to hemoglobin response in order to achieve a target hemoglobin level of 11 g/dl. In the second period (maintenance phase) EPO dose was optimized to maintain the hemoglobin level between 10 and 11 g/dl, by adjusting either the dose or the frequency of injection. Anemia was corrected in all patients within 10 weeks with EPO dose increasing from 72 to 360 IU/kg/week. The stabilisation of hemoglobin was achieved with an average EPO dose of 275 IU/kg/week (50 to 476 IU/kg/week). Concomitantly, a subjective and clinical improvement was noted in all patients. The dialysis efficacy, although remaining in an acceptable range, fell significantly by 10% over the first 3 months of treatment, remaining stable afterwards, yielding an effective urea clearance near to 120 1/week. The dietary protein intake calculated from urea kinetic modelling ranged between 1.1 and 1.2 g/kg/day.(ABSTRACT TRUNCATED AT 250 WORDS)

Interest of simultaneous ultrastructural characterization of morphology, cytochemistry and immune phenotype in a case of putative hybrid acute leukaemia.

A case of acute leukaemia is reported in which blast cells expressed some B-related antigens (namely the CALLA antigen) and no peroxidase activity at the optical level; however, some mature granular cells contained Auer rods. Simultaneous characterization of ultrastructural morphology, cytochemistry and immune phenotype was performed. There was an apparent mutual exclusion in the expression of myeloperoxidase activity and the CALLA antigen, and a heterogeneity in the CALLA expression among the blastic population. These results disagree with the hypothesis of a true biphenotypic leukaemia and demonstrate a complete heterogeneity between the lymphoblastoid cells and the myeloid ones. The interest of such a simple combined method in a case of putative hybrid acute leukaemia is emphasized.

Acute monocytic leukemia with B cell markers expression following B chronic lymphocytic leukemia.

A case of chronic lymphocytic leukemia (CLL) treated with chlorambucil, followed by the development of an acute monoblastic leukemia, is described. Cytofluorometric quantitative immunophenotype was determined during the blastic phase. Whereas small lymphocytes displayed a CD19+; CD24+; CD37+; CD5+ phenotype, the blastic population exhibited, besides CD13, CD14 and CD15 positivity, which is usually noted in such a monoblastic leukemia, definite CD9, CD10, CD22, CD24, CD37, CD5 and CD4 staining. Such results argue against a complete independence between the two clones, although their similarity could not be demonstrated.