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Biomedical subjects

J Cartron

Publications and source records attributed to J Cartron.

At least 19 recordsLinked to original sources
PubMed1999

[Neonatal alloimmune neutropenias. Importance of treatment with granulocyte colony-stimulating factor (G-CSF). Report of 2 new cases].

UNLABELLED: Alloimmune neonatal neutropenia is a rare event. Usually asymptomatic, it may however in some cases result in a severe sepsis. Treatment with recombinant granulocyte colony-stimulating factor (G-CSF) has been recently proposed. CASE REPORT: We report two new cases in infected newborns of a successful treatment of alloimmune neonatal neutropenia with G-CSF, resulting in complete neutrophil recovery in less than 72 hours. Moreover, the treatment was well tolerated. CONCLUSION: The analysis of these two cases and of those previously reported indicates that G-CSF represents the first-choice treatment in this affection when infectious signs are present in the neonate.

Age Factors↗
PubMed1999

Human parvovirus B19 infection in organ transplant recipients.

We report a 61-yr-old kidney transplant recipient with human Parvovirus B19 (HPV B19) infection presenting as a severe pancytopenia 1 month after transplantation. Bone marrow aspiration revealed severe erythroid hypoplasia with giant and dystrophic proerythroblasts. Bone marrow cells were positive for HPV B19 DNA detected by polymerase chain reaction (PCR). Pancytopenia resolved shortly after administration of intravenous immunoglobulins. Nineteen cases of HPV B19 infection in organ transplant recipients have been so far reported in the literature. Immunocompromised patients should be considered at risk from developing symptomatic HPV B19 infections. In such patients, specific anti-HPV B19 IgM and IgG antibodies may be absent or transient and therefore their negativity cannot rule out the diagnosis of HPV B19 infestation. Bone marrow smear morphological findings may suggest the diagnosis but testing for viral DNA by PCR is mandatory. Patients may spontaneously recover. However, since specific anti-viral therapy is not currently available, intravenous immunoglobulin administration appears to be the more efficacious treatment.

DNA, Viral↗
PubMed1997

Parvovirus B19 associated neutropenia. Treatment with Rh G-CSF.

In a 39-year-old patient under follow-up for metastatic adenocarcinoma of the colon, a post-operative fever and a profound neutropenia occurred during hospitalization. A diagnosis of primary infection with parvovirus B-19 was made in view of the association of reticulocytopenia, together with anti-B19 IgM antibodies and positive serum results for viral DNA. The granulocytic lineage appeared to be normal on bone-marrow smears, and anti PMN autoantibodies were not found. Rh G-CSF therapy resulted in a sharp increase in the PMN count. Although the pathophysiology of B19-associated neutropenia remains unclear, Rh G-CSF seems to be effective in this disorder.

Adenocarcinoma↗
PubMed1997

[Elective phagocytosis of polynuclear neutrophils caused by medullary macrophages and autoimmune neutropenia in children].

UNLABELLED: Autoimmune neutropenia (AIN) is a frequent cause of chronic neutropenia especially in youngest children. Its diagnosis is established by immunological proof of the autoimmune mechanism. The aim of this study is to better describe this autoimmune process and to show the contribution of bone marrow smears to this diagnosis. PATIENTS AND METHODS: Ten children, six girls and four boys, were examined between 1990 and 1995. Eight of them had typical AIN, confirmed by the presence of antibodies against neutrophils. Two other patients were included on the basis of bone marrow pictures. Five non-neutropenic children with normal bone marrow smears were chosen as controls. Bone marrow analysis was always performed by the same cytologist according to a reproducible technique. RESULTS: Six out of ten patients had important features of elective phagocytosis of neutrophils by marrow macrophages (unlike controls) without signs of dysgranulopoiesis or hemophagocytosis. Antibodies against neutrophils were detected in six patients with phagocytosis and in four patients without these cytological features. In two other children presenting the same bone marrow picture and clinical profile, an autoimmune process was probable, even in the absence of antibodies against neutrophils. Some patients had several infections and were given immunoglobulins and/or granulocyte colony-stimulating factor (G-CSF) therapy. The efficacy of Immunoglobulin was not constant, whereas G-CSF was effective at low doses and shortened the duration of infections. CONCLUSION: Prolonged neutropenia in childhood must lead to look for phagocytosis by marrow macrophages in bone marrow smears, as a possible sign of autoimmunity. Growth factors may temporarily be used associated with antibiotics therapy in severe and prolonged infections.

Autoimmune Diseases↗
PubMed1996

Molecular basis of the altered antigenic expression of RhD in weak D(Du) and RhC/e in RN phenotypes.

The RH blood group locus is composed of two sequence-related genes, RHD and RHCE, encoding the D, Cc, and Ee antigens in common Rh-positive phenotypes. In this report, we have analyzed the molecular basis of Rh antigens expression in weak D (Du) and RN donors, in whom there is a severe reduction of the D and C/e antigens, respectively. Genomic and transcript analysis of three unrelated low-grade weak D (Du) variants indicated that the very low expression of the D antigen is not the result of rearrangement or mutation in the coding sequence of the RHO gone. Accordingly, weak D (Du) erythrocytes should carry a normal RhD polypeptide, which is in agreement with the observation that these variants never produce anti-D antibodies. Comparative polymerase chain reaction analysis showed a lower steady-state level of RhD transcripts in weak D (Du) reticulocytes, as compared with normal RhD-positive controls, thus providing direct evidence that the difference between the D antigen of D-positive and weak D (Du) red blood cells is quantitative only. Conversely, analysis of the molecular genetic basis of the RN phenotype Indicated that the severely decreased expression of the RhC and Rhe antigens in three variants is associated with a qualitative alteration identified as a segmental DNA exchange between the RHCE and RHD genes. These genomic rearrangements, which resulted in hybrid RhCe-D-Ce proteins expressing the low frequency Rh32 but not the high incidence Rh46 antigens, involved either axon 4 alone or both exons 3 and 4. These findings show that an identical phenotypical alteration of Rh antigens (reduced expression) may result either from a quantitative or a qualitative alteration of the RH genes expression.

Alleles↗
PubMed1996

Non Hodgkin's lymphoma presenting as neutropenia related to an IgM monoclonal anti-i antibody.

A sixty-year old female was referred to the Internal Medicine Department for the treatment of a diffuse high-grade non Hodgkin's lymphoma. She presented episodes of fever in context of neutropenia (neutrophils 0.35 x 10(9)/1 from 1.6 x 10(9)/1 white blood cells). Hemoglobin level was 8.2 g/dl and platelets 132 x 10(12)/1. A monoclonal IgM-Kappa protein (48 g/l) was detected in her serum. A direct antiglobulin test on the red cells proved positive with anti-C3d but not with anti-IgG antiglobulin, due to the presence of an IgM cold antibody with a serological anti-i specificity. The IgM antibody was found on the patient's neutrophils as well as in her serum. The antibody recognized all neutrophils tested in conventional serological tests whether the neutrophil phenotypes in systems NA, NB, and 5. It was demonstrated that it recognized the i antigen expressed on the neutrophils. These results suggest that a cold agglutinin anti-i might be responsible for neutropenia in some patients.

Antibodies, Anti-Idiotypic↗
PubMed1996

Fatal intravascular autoimmune hemolytic anemia after fludarabine treatment for chronic lymphocytic leukemia.

We report the case of a patient with chronic lymphocytic leukemia (CLL) who developed fatal intravascular autoimmune hemolytic anemia (AIHA) after fludarabine treatment. He had previously received several treatments including two courses of fludarabine. The direct antiglobulin test (DAT) was negative at diagnosis but was found to be positive with anti-IgG after the first fludarabine treatment. When the patient was treated again with fludarabine nine months later, the DAT became positive with anti-IgG and anti-C3d antiglobulins after the second course of treatment. Abrupt, fatal intravascular hemolysis occurred after the third course. The occurrence of severe AIHA in CLL patients treated with fludarabine has been reported by several authors. Physicians should be aware of the risk of severe AIHA in CLL patients with a history of AIHA or positivation of the DAT during previous fludarabine administration, or in case of secondary fixation of complement to the red cell membrane occurring during fludarabine treatment.

Aged↗
PubMed1996

[Sarcoidosis with hematologic involvement and hypogammaglobulinemia].

BACKGROUND: Hematological changes are uncommon in childhood sarcoidosis. CASE REPORT: Case 1. This patient developed severe hemolytic anemia associated with splenomegaly at the age of 11 months. A second episode of hemolysis was seen at the age of 6 years; it was associated with thrombocytopenia and hypogammaglobulinemia. A partial splenectomy permitted histological diagnosis of sarcoidosis that was confirmed by elevated angiotensin-converting enzyme level, and subsequently developement of pulmonary involvement. Case 2. This boy suffered from autoimmune hemolytic anemia and thrombocytopenia associated with splenomegaly at the age of 3 years. At the age of 7 years, he had splenomegaly, enlarged cervical and lomboaortic lymph nodes and hypogammaglobulinemia. Diagnosis of sarcoidosis was made from lymph nodes and spleen biopsies after splenectomy; the patient had increased angiotensin-converting enzyme concentration, and subsequently developed specific renal involvement. CONCLUSION: Association of hemolytic anemia, thrombocytopenia, hypogammaglobulinemia and splenomegaly should suggest the diagnosis of sarcoidosis and could be considered as part of the disease spectrum.

Agammaglobulinemia↗
PubMed1996

[Immuno-hemolytic transfusion reactions. III. Report of 61 cases].

Blood transfusion is mainly bound to immunological and infectious risks. The immunological risk originates from an incompatibility between the blood of the donor and that of the recipient; this risk remains insufficiently assessed. A multicentre study has been carried out by the French Blood Transfusion Society and the National Institute for Blood Transfusion. Sixty-one accidents due to an erythrocyte incompatibility were found: 26 cases with ABO incompatibility, and 35 cases with alloantibodies of other blood group systems. For the former category of accidents, the most frequent cause was due to a failure in the realization of the bedside ABO check. For the latter, the main problem was the achievement and the interpretation of antibody screening. The long term follow-up shows no chronic after-effects of immunological accidents. For each accident, errors have been identified and analysed. It was proven that they all originate from health care establishments.

ABO Blood-Group System↗
PubMed1995

Heterogeneity of neutrophil antibodies in patients with primary Sjögren's syndrome.

Our aims were to determine the prevalence of neutrophil antibodies in patients with primary Sjögren's syndrome (pSS), identify their target antigen(s), and evaluate their functional significance. Neutrophil antibodies were detected using an indirect immunofluorescence (IIIF) test and an enzyme-linked immunosorbent assay (ELISA), using recombinant human Fc-gamma receptor (Fc gamma RIIIb) as a capture agent. Luminol-dependent chemiluminescence was then measured by an established technique. Antibodies to neutrophils were detected in 30 of 66 patients (45%) and categorized on the basis of positivity for the two assays: IIF+/ELISA+ (group A: five patients), IIF+/ELISA- (group B: five patients), and IFF-/ELISA+ (group C: 20 patients). All positive sera contained antibodies directed to the neutrophil specific Fc gamma RIIIb, and none of them bound to NAnull neutrophils. The titer of neutrophil-reactive antibodies (groups A and B) showed no correlation with the neutrophil count, but these autoantibodies did reduce the cell ability to generate a respiratory burst. Thus, neutrophil antibodies are common in patients with pSS. Their main target appears to be Fc gamma RIII, and this may partly account for the dysfunction in Fc gamma R-mediated clearance by the reticuloendothelial system reported in these patients.

Adult↗
PubMed1995

Anti-Fc gamma receptor III autoantibody is associated with soluble receptor in rheumatoid arthritis serum and synovial fluid.

We sought to detect anti-Fc gamma receptor (Fc gamma R) autoantibodies and soluble Fc gamma R in the serum and synovial fluid (SF) from patients with rheumatoid arthritis (RA) and to correlate these serological abnormalities to the polymorphonuclear cell (PMN) activation state. Paired samples of blood and SF were obtained from 33 RA patients as well as blood from 25 normal adults from SF from 20 non-RA patients. Anti-Fc gamma R autoantibodies were assessed by an enzyme-linked immunosorbent assay (ELISA) using recombinant human Fc gamma R as the substrate. Soluble Fc gamma RIII was determined by an ELISA based on the combination of two monoclonal antibodies (MAb). The mean fluorescence intensity (MFI) of complement receptor 1 (CD35) and 3 (CD11b) and Fc gamma RIII (CD16) was evaluated by flow cytometry on the membrane of PMN. IgM anti-Fc gamma RIII activity was present in seven RA sera and five SF, and IgG in eight RA sera and six SF. The average concentration of soluble Fc gamma RIII was 1.80 +/- 3.50 micrograms/ml in RA patients and 0.33 +/- 0.06 micrograms/ml in normal adults (P < 0.05). This was elevated in the SF of 15 RA, while normal in that of all the non-RA patients. There was an inverse correlation between the CD16 MFI on the PMN and the serum/SF soluble Fc gamma RIII level, whereas the density of CD35 and CD11b was markedly augmented. Anti-Fc gamma RIII activity exists in RA patients, associated with soluble Fc gamma RIII. PMN activation could be due to these autoantibodies and thereby obviate the clearance of immune complexes.

Adult↗
PubMed1994

[Hemolytic anemia after kidney transplantation].

Several cases of haemolysis after bone marrow or organ transplantation have been reported. An allospecific anti-erythrocyte antibody has been described in each case. We report a similar case after renal transplantation. Berger's disease led to end-stage renal failure in an 18-year-old girl. During haemodialysis carried out for 6 years, the patient had received 9 transfusions of phenotyped, filtered, packed red blood cells. The recipient was grouped as O Rh positive, CcDee, Kell negative, HLA-A24 A11 B35 B-DR1 DR2. The donor was her father, grouped as O Rh negative, ccddee, Kell negative, HLA-A24 A- B35 B- DR2 DR-. Screening for erythrocyte antibodies was negative in the recipient as were screening for lymphocytotoxic antibodies and compatibility testing. However anti-D antibody was present in the donor who had received a transfusion of Rh positive blood several years before renal donation. The recipient was given 2 units of O Rh positive, phenotyped and filtered red blood cell concentrates during transplantation. Immunosuppressive therapy associated azathioprine and prednisone. The onset of graft function was immediate. Haemoglobin fell to 52 g/l on day 14 post transplantation (103 g/l on day 6). Anti-D antibody was identified in the serum. The direct antiglobulin test was positive with anti-IgG, antiglobulin and the eluate contained an antibody exhibiting also anti-D specificity. The anti-D of the patient and the donor had the same Gm allotyping. The outcome was favourable though the antibody was persistent for several months. This case of haemolysis is consistent with the fact that immunocompetent B lymphocytes transferred with the grafted organ are still able to produce clinically significant antibodies.

Adolescent↗
PubMed1994

[Erythropoietin for autologous transfusion. Use in a case of severe anemia with allo-immunization].

In view of the transfusional risks of viral transmission (notably HIV), autologous transfusion is increasingly used; it is often the only possible type of transfusion. A 42-year-old woman with lupus erythematosus, chronic renal failure and triple cardiac valve disease demanding surgery was admitted for multifactorial severe anaemia. Treatment with erythropoietin (8000 units/day) iron replenishment, corticosteroids and polyvalent immunoglobulins was initiated. The patient was operated upon in April 1990. A preoperative cell-saver autotransfusion was performed during surgery. The postoperative period was uneventful. Homologous transfusion was not necessary. In this case where homologous transfusion was ruled out, erythropoiesis stimulated by erythropoietin enabled autotransfusion and cardiac surgery to be performed.

Adult↗
PubMed1994

[Acquired and constitutional neutropenia in children].

The evaluation of a neutropenia first must document its etiology. Besides the particular etiological aspects in the newborn, neutropenia in a child may be 1) acquired, 2) constitutional, part of a complex genetic disease, 3) constitutional, isolated. Primary acquired neutropenia, also called benign chronic neutropenia, is the most frequent cause of chronic neutropenia in children; it is usually well tolerated and has a frequent favorable outcome in 12-14 months. Many complex genetic diseases include a neutropenia, among which several immunologic disorders that must be ruled out before considering the diagnosis of isolated constitutional neutropenia. Infantile agranulocytosis is the main primary constitutional neutropenia. It may be sporadic or hereditary (autosomal recessive or dominant inheritance) and is present at birth. It is profound, usually < 0.5 G/l (< 500/mm3) and exposes to severe pyogenic and fungal infections. In the neonatal period neutropenia must primarily suggest a bacterial infection, although other etiologies have to be known, particularly neonatal neutropenia caused by passive transfer of maternal antibodies and neutropenia related to gravidic maternal hypertension. The treatment of severe chronic neutropenia is directed towards the prevention of infections. It includes prophylactic antibiotherapy, the most commonly used one being the trimetroprim-sulfamethoxazole association, and granulocyte colony stimulating factor (G-CSF). G-CSF has considerably improved the condition of patients; it is usually well tolerated, but secondary effects have been reported (hypersplenism, glomerulonephritis, osteoporosis, vasculitis), and a potential leukemogenic risk has been evoked.

Child↗