PubMed Health⌕ Search

Biomedical subjects

J Gardais

Publications and source records attributed to J Gardais.

At least 19 recordsLinked to original sources
PubMed2001

Is there any significance for intracellular crystals in plasma cells from patients with monoclonal gammopathies?

Several plasma cells morphological changes have been described in monoclonal gammopathies, including intracytoplasmic crystals. We report one case of indolent kappa-chain multiple myeloma with renal insufficiency, featuring plasma cells with Auer-rod-like intracytoplasmic inclusions. The relationship between such aberrations and those found in multiple-myeloma-associated adult Fanconi syndrome is discussed. The significance of intracellular storage and crystallisation of immunoglobulin within plasma cells remains partially unknown.

Aged↗
PubMed2000

Dyshaemopoiesis in adults: a practical classification for diagnosis and management.

Dyshaemopoiesis is a heterogeneous disease that may be classified into non-clonal and clonal dyshaemopoiesis. Non-clonal dyshaemopoiesis comprises reversible disorders with DNA synthesis impairment in dividing cells of the bone marrow by avitaminosis through various mechanisms or direct DNA damage from multiple causes. Complete haematologic recovery is obtained after vitamin supplementation or suppression of a myelotoxic agent. On the contrary, clonal dyshaemopoiesis is a group of chronic and usually irreversible diseases that may culminate in acute leukaemia (AL). These so called myelodysplastic syndromes (MDS) and their variants may be classified as primary, secondary and other diseases with doubtful clonality. A detailed classification of dyshaemopoiesis in adults may offer partial help in the diagnosis and management of dyshaemopoiesis. Pathobiological studies in progress allow better understanding of MDS and consequently the establishment of new modalities of treatment.

Adult↗
PubMed1997

Successive transformation of chronic myelomonocytic leukaemia into acute myeloblastic then lymphoblastic leukaemia, both with minor-bcr rearrangement.

We report a case of chronic myelomonocytic leukaemia (CMML), which transformed first into acute myeloblastic leukaemia (AML) and then into acute lymphoblastic leukaemia (ALL). In the AML and ALL phases, chromosome analysis showed a classic Philadelphia chromosome (Ph) t(9:22)(q34:q11). Molecular studies showed breakpoint cluster region rearrangement between exons e1 and a2 compatible with a p190(bcr/abl) breakpoint as observed in Ph-positive lymphoblastic acute leukaemia. The minor (m-bcr) rearrangement was also detected during complete remission. This observation supports a multistep pathogenesis of leukaemias, and that the p190(bcr/abl) breakpoint may influence the course of the disease.

Aged↗
PubMed1996

C-1-inhibitor binding monoclonal immunoglobins in three patients with acquired angioneurotic edema.

The syndrome of acquired angioneurotic edema (AAE) is characterized by the adult onset of angioedema, the lack of evidence for inheritance of the disorder, and the frequent association of the C1-inhibitor (C1-INH) deficiency with lymphoproliferative or other malignant diseases. Recently, a new type of AAE (type II AAE) has been described. The two major biologic differences of this new syndrome compared with all other previously reported AAE cases (type I AAE) are the presence in patients' sera of both anti-C1-INH autoantibodies, often monoclonal, and a circulating low molecular weight (95 kd) C1-INH protein. From the clinical point of view, the absence of underlying lymphoproliferative disease is the hallmark of type II AAE compared with type I AAE. However, the distinction between type I and type II AAE may not be so clear-cut. We report three patients with monoclonal gammopathies and AAE for whom the initial diagnosis was type I AAE. The demonstration by ELISA of the C1-INH binding ability of their monoclonal immunoglobulins in addition to the presence of 95 kd C1-INH protein enables us to change the diagnosis to type II AAE. From the therapeutic point of view, it is crucial to detect the anti-C1-INH antibody and to analyze the C1-INH size to distinguish type I and type II AAE, especially if patients have a monoclonal gammopathy, to give the appropriate treatment (attenuated androgens vs immunosuppressive regimen, respectively) to prevent a fatal outcome.

Aged↗
PubMed1995

[Immunohistochemical characterization of acute leukemia. Study of 31 bone marrow biopsies].

An immunohistochemical study by avidin-biotin-peroxidase was performed on paraffin-embedded and decalcified bone marrow biopsies in 31 acute leukemias (19 myeloid and 12 lymphoblastic). The Ulex Europaeus lectin and 14 antibodies (anti-CD45, -CD34, -myeloperoxidase, -lysozyme, -CD15, -CD68, -carcinoembryonic antigen, -factor VIII-related antigen, BNH9, anti-CD45RO, -CD3, -CD20, DBB42 and DBA44) were tested. All acute myeloid leukemias from M0 to M5 type were stained by either the anti-myeloperoxidase or anti-lysozyme antibodies. CD68, CD15 and the carcinoembryonic antigen were respectively expressed in 80%, 40% and 20% of myeloid leukemias from M1 to M5 type. The Ulex Europaeus lectin and the anti-factor VIII-related antigen antibody stained only the M7 leukemia and the anti-CD3 antibody stained only the T acute lymphoblastic leukemia. DBB42 was expressed by 63% of B-lineage lymphoblastic leukemias and CD20 by 36%. No leukemia was stained by DBA44. Immunohistochemistry on bone marrow biopsy can assess the lineage of most acute leukemias with the use of a panel of antibodies such as the anti-myeloperoxidase, -lysozyme, -CD68, -CD20, DBB42, -CD3, BNH9, anti-factor VIII-related antigen antibodies and the Ulex Europaeus lectin.

Acute Disease↗
PubMed1994

Translocation t(5;12)(q31-q33;p12-p13): a non-random translocation associated with a myeloid disorder with eosinophilia.

A t(5;12)(q33;p13) translocation has been detected in two patients with myeloid disorder and eosinophilia. Six other patients with haematological disease with eosinophilia with similar translocation have been published previously. The existence of a new entity, a myeloproliferative disorder with eosinophilia and t(5;12) (q31-q33;p12-p13), is suggested by the results of the present study.

Aged↗
PubMed1993

Lymph node disease with lymphocytic abnormal chromatin clumping in a myelodysplastic/myeloproliferative syndrome.

A case of abnormal chromatin clumping (ACC) which arose during the course of a myelodysplastic/myeloproliferative syndrome is described in a 61 year old woman who died of haemorrhage 43 months after diagnosis. Mature granulocytes exhibited the same nuclear abnormality described in other patients reported. Unusually, she presented with advanced splenomegaly and lymphadenopathy. This case was the third example of ACC in lymphocytes, the first with clinically confirmed lymphadenopathy. Diagnosis of this subset can be based on: older age; short duration of symptoms; no specific karyotypic damage; non-rearranged bcr; proliferative growth pattern in vitro; numerous circulating myelocytes; profound thrombocytopenia.

Chromatin↗
PubMed1992

Heterogeneity of acquired idiopathic sideroblastic anaemia (AISA).

Clinical, haematological and outcome data were studied in 84 patients with acquired idiopathic sideroblastic anaemia (AISA) from a registry of 613 consecutive myelodysplastic syndromes (MDS) recorded by five institutions in western France. Two groups could be identified and compared: 'pure' erythroblastic AISA (AISA-E: 59 pts), and AISA with myelodysplastic features, i.e. dysgranulo and/or dysmegakaryopoiesis (AISA-M: 25 pts). Results were also compared to those of a series of 71 cases of refractory anaemia without sideroblastosis (RA) carried out from the same registry. Dyserythropoiesis was present in 90% of all AISA subtypes, dysgranulopoiesis in 88% of the AISA-M cases; dysmegakaryopoiesis was observed in 44% of AISA-M. Ten patients with both forms of AISA showed high platelet counts. These cases appeared particular in that four of them were associated with a splenomegaly and/or a hyperleucocytosis. They had to be distinguished from myeloproliferative syndromes. Outcome comparison of AISA-E with AISA-M showed a significant discrepancy of survival duration (60 vs 38 months respectively). Progression towards refractory anaemia with excess of blasts or acute leukaemia, was significantly higher for AISA-M than for AISA-E. The risk of transformation increased to 24% for the AISA-M group similarly to those of RA patients (17%). We conclude that AISA must be divided into two categories, 'pure' AISA and AISA-M, because survival duration and risk of transformation are different.

Aged↗
PubMed1990

Prognostic factors of myelodysplastic syndromes--a simplified 3-D scoring system.

From 1 January 1982 to 31 December 1986 in five haematological centers of the west of France (Rennes, Rouen, Nantes, Tours and Angers), we have collected 503 cases of myelodysplastic syndrome (MDS). These cases were classified by FAB recommendation as followed: 85 refractory anemia with ring sideroblasts (RARS); 273 refractory anemia in which 86 were without blasts (RA), 153 were with excess of blasts (RAEB) and 34 were with excess of blasts and in transformation (RAEB-t); 111 chronic myelomonocytic leukaemia (CMML); and 34 cases with borderline features. The point date for statistical study was 31 December 1988, and the scoring method of Bournemouth was applied to compare with our findings (62% resulted in death, 18% in leukemic transformation). It was demonstrated that haemoglobin, platelets, and bone marrow-blasts are the best factors to predict survival or leukaemic transformation (LT). But peripheral neutrophils don't affect the survival time excepted when lower than 500 microliters (13 months vs 19.6 months). A scoring system based on haemoglobin (Hb), platelets (Pl), and bone marrow blasts (BMB) may be represented in a three-dimensional space and is a good tool to know the own value of each parameter. This 3-D system shows that BMB and Pl are the most important factors and are correlated with survival, per cent of death, and LT (p less than 0.0001). The LT is observed in 18% of the whole population. RAEB and RAEB-t progress in AML2 (14.6%) or AML4 (1.4%), and CMML progress in AML2 (8.1%) or AML4 (11.7%). We observed that monocytes are not good parameters to predict the type of leukemic transformation. Furthermore, survival of RA treated with Ara-C(ld) or not treated was similar.

Aged↗
PubMed1987

Blast crisis supervening on chronic lymphocytic leukaemia. A monoclonal progression of the disease as defined by cell surface markers.

Acute leukaemia is a rare event during the course of chronic lymphocytic leukaemia (CLL), and only a small fraction of such cases have been shown to be true acute lymphoblastic crises. 1 case is described where both small lymphocytes and proliferating lymphoblasts have the same monoclonal pattern as defined by direct immunofluorescence of membrane-bound immunoglobulins. Previous cases are reviewed and do not appear to be mere coincidence: acute blast crisis may represent a part of the natural history of CLL.

Aged↗
PubMed1984

[Leukemoid eosinophilic reaction in angioimmunoblastic adenopathy].

A case of eosinophilic leukemoid reaction is described. This picture is seldom encountered during the course of angio-immunoblastic adenopathy. After reviewing various diagnoses and pointing out similar cases of the literature, the authors discuss the possible pathophysiologic mechanisms of eosinophilia in this disorder.

Aged↗
PubMed1983

[Double gammopathies. Incidence and characteristics (83 cases). Clinical correlations (62 cases)].

Within 13 years (1969-1981) 1 339 monoclonal gammopathies were recognized and fully investigated. The following were remarked: the high frequency of "double gammopathies": 6,2 p. 100 (83 cases); the high frequency of the association of (G + M): 52,4 p. 100 of double gammopathies of different classes (63 cases); both of these probably in relation of the abnormal frequency of the IgM-gammopathies (35,4 p. 100 in the Anjou province). Clinical correlation of double gammopathies do not appear to be different from those noticed in monoclonal gammopathies. In the 62 cases of obvious diagnosis, chronic lymphoproliferative diseases are the more frequent: 32,3 p. 100, followed by 17,7 p. 100 of multiple myelomas, 14,5 p. 100 of carcinoma, 4,9 p. 100 of other malignant diseases, 22,6 p. 100 of benign disorders and 8 p. 100 of idiopathic cases. In all the cases of multiple myeloma and Waldenström's macroglobulinemia, a reciprocal correlation is remarked between predominant monoclonal immunoglobulin and the diagnosis. In all the other diseases (except for 3 cases) one of the two monoclonal components is an IgM.

Adenoma↗
PubMed1982

[Metoclopramide in hematology].

Metoclopramide is very useful in hematology. This French drug has been continuously used for twenty years as a powerful inhibitor of nausea and vomiting induced by numerous antineoplastic associations. When administered intravenously in patients under sequential chemotherapy for leukemia or lymphoma, metoclopramide prevents digestive discomfort. After each infusion, the ingestion of solution or tablets prolongs the antiemetic action for several hours without any side-effects. Conversely, corticosteroid therapy is well tolerated in ulcerous and gastritic patients and tolerance is preserved in others when they are submitted for the first time to high doses of metoclopramide, because of the acceleration of gastric evacuation.

Hematologic Diseases↗
PubMed1982

[Malignant systemic mastocytosis (author's transl)].

The case of a 37 year old man with systemic malignant cutaneous mastocytosis is reported. The outcome was fatal after eight months. Death was due to a diffuse hemorrhagic syndrome which originated in an abnormal circulating heparinoid. Nosologically, this is a border-line case between mast cell leukemia and leukemic systemic mastocytosis.

Adult↗