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Biomedical subjects

A Ferrant

Publications and source records attributed to A Ferrant.

216 records · Page 12Linked to original sources

A new hematologic syndrome with a distinct karyotype: the 5 q--chromosome.

Five patients, four women and one man, age 32-8- yr, all whites, had refractory anemia with the same abnormal bone marrow karyotype, i.e., a partial deletion of the long arm of the No. 5 chromosome. The hematologic syndrome was practically the same in these five cases. Examination of the blood revealed a moderate to severe, generally macrocytic anemia with slight leukopenia but normal or elevated platelet count. The bone marrow showed a depressed erythroid series and some abnormalities of the granulocytic series with an occasional excess of myeloblasts. Most of the megakaryocytes had a nonlobulated nucleus. These features, as well as cytogenetic, electron microscopic, isotopic, platelet function, and immunologic studies, are described in detail. The relationship of this newly established syndrome to other hematologic diseases is discussed. The syndrome constitutes another example of the association between a specific abnormal chromosome and a distinct hematologic disorder.

Adult↗

The elution of 99-Tcm from red cells and its effect on red-cell volume measurement.

Technetium-labelled erythrocytes provide a satisfactory agent for the measurement of red-cell volume with the advantages that there is a low radiation dose and repeated measurements can be readily carried out. A method of labelling red cells using (99)Tc(m)O(4) (-) with a small amount of stannous chloride has been evaluated in 20 patients, and compared with measurements of red-cell volume with (51)Cr-labelled erythrocytes carried out simultaneously in the same subjects. No significant differences between the results by either method occurred if the period of observation was limited to 20 minutes after the injection of labelled cells. Thereafter, elution of Tc from the cells made the determination of red-cell volume with (99)Tc(m) less reliable. Accordingly, the use of (99)Tc(m) as the label for red-cell volume measurements will result in errors if mixing of the erythrocyte pool is delayed, unless an elution correction factor is applied.

Anemia, Aplastic↗

Intensive chemotherapy for acute non-lymphoblastic leukemia after primary myelodysplastic syndrome.

Twenty-five patients with a primary myelodysplastic syndrome (MDS) transformed into acute non-lymphoblastic leukaemia (ANL) were treated with intensive chemotherapy. A complete remission (CR) was obtained in six patients (24 per cent). In five of these six patients two courses of chemotherapy were needed to achieve CR. In eight patients chemotherapy cleared the bone marrow of blasts, but the aplasia was fatal. A partial effect on bone marrow blasts was seen in four patients and no effect in another six. Eleven patients (44 per cent) died from the consequences of chemotherapy-induced cytopenia. A short interval between MDS and transformation into ANL was associated with a better chance of achieving complete remission. Age, karyotype, type of MDS, peripheral blood or bone marrow findings had no influence on the result of chemotherapy. The median survival from start of treatment was 5 months (range 0.5-24 months). In the patients who achieved a CR, the median duration of the remission was 7 months (range 3-12 months). The poor response rate, the short duration of the remissions and the high treatment-related mortality suggest that current intensive anti-leukemic chemotherapy in ANL after primary MDS is of limited benefit.

Adolescent↗

Chronic lymphocytic leukemia: changes in bone marrow composition and distribution assessed with quantitative MRI.

The purposes of this study were (a) to determine the prevalence of bone marrow abnormalities in patients with chronic lymphocytic leukemia (CLL) using quantitative MR assessment of axial marrow composition and peripheral marrow distribution; (b) to assess the agreement between both quantitative MR methods and compare their sensitivities to detect marrow alterations; and (c) to correlate MR findings with clinical and laboratory parameters. Twenty-nine consecutive patients with biopsy-proven CLL were investigated on a .5-T MR imager to determine bulk T1 relaxation times of the vertebral bone marrow and proportion of proximal femur surface area occupied by nonfatty marrow on coronal T1-weighted MR images of one hip. Of the 29 patients, 12 (41%) had abnormal increase in lumbar marrow T1 values (>600 msec) and 16 (55%) had increased proportion of surface area occupied by nonfatty marrow in the proximal femur (>+1 SD compared to normal values determined in sex- and age-matched healthy subjects). The results of both quantitative MR methods were normal in 12 patients and abnormal in 11 patients (agreement, 79%). Patients with alterations in peripheral marrow distribution had significantly higher T1 relaxation times (P = .001) than those with normal peripheral marrow. Patients with abnormal marrow composition or distribution at MRI had significantly higher blood and marrow lymphocytosis than patients without these features. In conclusion, the agreement between both quantitative MR methods suggests a parallelism between changes in axial marrow composition and in peripheral marrow distribution in patients with CLL. The limits of quantitative MRI in CLL must be kept in mind, because quantitative MR methods failed to detect leukemic marrow infiltration in 41% of patients.

Adult↗

Pericarditis induced by high-dose cytosine arabinoside chemotherapy.

Pericarditis is a rare side effect of chemotherapy. We report here the case of a patient treated with high-dose cytosine arabinoside (HD ara-c) who developed severe pericarditis. Interruption of HD ara-c and initiation of corticosteroid treatment were effective in resolving the pericardial effusion. This case illustrates this rare but potentially life-threatening cardiac complication of HD ara-c therapy as well as the beneficial effect of corticosteroid treatment.

Adult↗

Penicillin or vancomycin plus ceftazidime in febrile neutropenic patients after stem cell transplantation.

The response of gram-positive cocci to third generation cephalosporin therapy in febrile neutropenic patients is not optimal. We evaluated the safety and efficacy of ceftazidime plus penicillin (C + P) and compared it to ceftazidime plus vancomycin (C + V) in febrile neutropenic patients. The study includes 64 patients admitted to the Department of Haematology. Thirty-six patients were treated with C + V and 28 patients with C + P. Control of infection was observed in 78% of the patients in the C + V group and in 57% of the patients in C + P group (p = 0.5). Infection was the cause of the death of 1 patient in each group. We conclude that the combination of C + P has the same activity as the combination of C + V in febrile neutropenic patients. The morbidity and mortality were identical in both groups but cost effectiveness was in favour of the C + P group.

Adolescent↗

Effect of various doses of intravenous polyclonal IgG on in vivo levels of 12 pneumococcal antibodies in patients with chronic lymphocytic leukaemia and multiple myeloma.

Polyclonal intravenous IgG (IVIG) was administered as an infusion 6 times every 3 weeks (week 0, 3, 6, 9, 12, 15) in doses of 0.1, 0.4 and 0.8 g/kg BW to determine the dose causing an increase in 12 pneumococcal antibody types above the protective level of 200 ng/ml of antibody N. The dose of 0.4 g/kg BW was found to be optimal in patients with chronic lymphocytic leukaemia (CLL). From the first infusion onwards at least 80% of CLL patients had increases in all 12 antibodies. Five weeks after the last infusion the antibody levels were still elevated in 80% of patients with CLL. The dose of 0.8 g/kg raised all 12 antibodies in 53-73% of CLL patients when assessments were made after each infusion. In multiple myeloma (MM) patients, 73-82% and 73-91% of patients had increased antibody levels, respectively, before and after the 4th-6th infusions at the 0.8 g/kg dose level. However, in only 45-50% of patients did the antibodies remain increased 2 weeks after the treatment at this dose. The dose of 0.4 g/kg caused antibody increases in only 30-50% of patients when measured before the 4th-6th infusion. Serum IgG increased significantly only in the CLL patients, whereas in the MM patients it was high from the beginning owing to the disease. Therefore, the pneumococcal antibody levels were a better marker for the purpose of dose finding. The dosage recommendation in CLL is 0.4 g/kg every 3 weeks until week 12, when steady state is reached. The maintenance dose is 0.4 g/kg every 5 weeks. In MM patients, who have a faster elimination rate of antibodies, the recommended loading dose is 0.8 g/kg, followed by 0.4 g/kg every week as a continuous treatment. Treatment with IVIG in CLL and MM was generally well tolerated. Only 25% of patients experienced minor side-effects, the most frequent being febrile reactions, shivering and headache.

Adult↗

Clinical and pathological features of 14 non-Hodgkin's lymphomas associated with coeliac disease.

BACKGROUND: It is well established that enteropathy associated T-cell lymphoma is associated with malabsorption which is due to gluten sensitivity (coeliac disease). Our study was performed to define the clinical features, histological subtypes, response to treatment, and outcome of the association of coeliac disease and T-cell lymphoma. PATIENTS AND METHODS: A retrospective study was performed in the UCL Group of Hematology to collect data on patients with a diagnosis of non-Hodgkin's lymphoma and coeliac disease. Fifteen cases were observed between 1985 and 1999. Case records for all but one patient were available and the pathological specimens of 14 patients were reviewed by two pathologists. RESULTS: Six previously diagnosed coeliac patients developed lymphoma; interval between coeliac symptoms and onset of the lymphoma ranged from 2 to 48 years (median 16 years). Five patients had coeliac disease and non-Hodgkin's lymphoma diagnosed concomitantly or less than 6 months before the symptoms leading to the diagnosis of lymphoma. Three patients had the diagnosis of coeliac disease after lymphoma diagnosis (1, 8 and 10 years later respectively). Ten non-Hodgkin's lymphomas were of T-cell origin and 4 were B-cell lymphomas. Eight out of 14 presented on a surgical emergency. Thirteen were treated using chemotherapy. The median survival from the diagnosis of enteropathy associated T-cell lymphoma was 12 months (range 1-126). CONCLUSIONS: Lymphomas associated with coeliac disease are heterogeneous and their diagnosis is difficult. The enteropathy-associated T-cell lymphoma is the most frequent, aggressive and fatal complication of coeliac disease but it is not rare to observe association with B-cell lymphoma. Chemotherapy is highly toxic in those patients. Despite a poor prognosis, long-term survival can be expected in a fraction of these patients.

Adult↗

Donor cell leukemia developing after hematopoietic stem cell transplantation for multiple myeloma.

The development of secondary leukemia in donor cells after allogeneic stem cell transplantation is a rare event. We describe the occurrence of acute myeloid leukemia in donor cells 4 years after a stem cell transplantation for multiple myeloma. The multiple myeloma was relapsing at the time of the onset of acute myeloid leukemia. Secondary leukemia in donor cells after transplantation for multiple myeloma has not yet been reported.

Adult↗

Scintigraphy with Indium-labelled leukocytes in acute pancreatitis.

The authors assessed the clinical and prognostic value of abdominal (ASCI) and pulmonary (PSCI) scintiscans with 111Indium-labelled leukocytes in the early phase of acute pancreatitis. A grading scale was constructed for both ASCI (scored 0 to 3) and PSCI (scored 0 to 2) according to the intensity of isotope fixation versus adjacent structures. Results were compared in accordance to the presence or absence of a Ranson's score greater than or equal to 3, the presence or absence of respiratory failure and of late pancreatic complications. ASCI showed an important lack of specificity making its use uninteresting. PSCI revealed a very significant correlation with evaluation by Ranson's score and appears to have a high sensitivity and specificity to evaluate the patients who will develop ARDS or risk of ARDS. This demonstrates the pathophysiological role of leukocytes in the early phase of severe acute pancreatitis. The test might also be a reliable test for the assessment of therapeutic efficiency in acute pancreatitis.

Abdomen↗

[Exudative enteropathy caused by an acute graft-vs-host reaction. Apropos of a case report].

The authors report the case of a 41-year-old woman who developed an acute graft-versus-host disease following bone marrow transplantation for post-polycythemic myeloid metaplasia; liver, cutaneous and intestinal lesions were present, associated, with a severe protein-losing enteropathy. Symptoms, diagnostic procedures and therapy of intestinal graft-versus-host disease are reviewed. The intestinal clearance of alpha-1 antitrypsin was particularly useful to assess the response to medial treatment, which induced a complete remission.

Adult↗

The plicamycin and hydroxyurea combination chemotherapy for chronic granulocytic leukemia in myeloid transformation.

Between 1987-1988 we treated, in a cooperative study, 13 patients in blast crisis of CGL according to Koller and Miller's regimen. We observed no return to the chronic phase of the disease, while some patients suffered major drug-induced side effects. Six patients in accelerated phase were treated with the same combination therapy. All of them responded with a return to the chronic phase for a median of 4.5 months. In our hands the combination of plicamycin and hydroxyurea was ineffective in patients with CGL in blast crisis. This regimen could deserve further evaluation in patients with CGL in acceleration.

Adult↗

[Skeletal and medullary involvement in Hodgkin's disease].

In 70 patients with clinical stage II, III or IV Hodgkin's disease, 31 were found to have skeletal involvement. The value of the bone scanning in the demonstration of osseous and marrow lesions is emphasized : scanning enabled localised marrow involvement in 24 patients to be detected and could be used as a guide for the biopsy needle in 11 cases. The histopathological features of marrow involvement by Hodgkin's disease are described. In view of the better knowledge of the frequency of splenic, skeletal or hepatic involvement, the preferential involvement of a particular organ in disseminated diseases is discussed. The high frequency of splenic involvement could be due to the inadequacy of the methods detecting hepatic or marrow involvement, although the nature of the disease could also be a factor.

Bone Neoplasms↗

Philadelphia chromosome-positive acute myeloid leukemia: cytoimmunologic and cytogenetic features.

BACKGROUND: Little is known about the morphological and clinical features of the minority of acute myeloid leukemias (AML) that carry the t(9;22)(q34;q11) translocation. MATERIALS AND METHODS: Cytologic, cytogenetic and clinical features were studied at diagnosis and during disease evolution in 11 patients presenting with de novo AML. Diagnoses according to the FAB criteria were AML-M2 (3 cases), AML-MO and AML-M4 (2 cases each), AML-M4eos, AML-M5, AML-M6, AML-M7 (1 case each). RESULTS: Immunophenotyping disclosed positivity for CD33 or CD13 and for the CD34 stem cell antigen in all cases tested. Lymphoid-associated markers (LM) were detected in 7/9 patients. In 5 cases the expression of at least 2 LM was seen. In addition, evidence of clonal rearrangement of the immunoglobulin (Ig) and/or T-cell receptor (TCR) genes was documented in 3/4 evaluable cases. The Ph chromosome was found as the sole change in 5/11 cases; the karyotype reverted to normal in 2/4 patients who achieved complete remission. Rearrangement in the M-bcr region was detected by Southern blotting in 2/7 cases. CONCLUSIONS: This infrequent cytogenetic subset of de novo AML appears to be characterized by heterogeneous cytologic features, with frequent expression of lymphoid markers, and by unfavorable prognosis. The combination of clinical, cytologic and cytogenetic studies is important in distinguishing de novo AML with the t(9;22) from chronic myelogenous leukemia blast crisis.

Acute Disease↗