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Biomedical subjects

A Le Tourneau

Publications and source records attributed to A Le Tourneau.

At least 19 recordsLinked to original sources

Mantle cell lymphoma, in leukaemic phase with prominent splenomegaly. A report of eight cases with similar clinical presentation and aggressive outcome.

Mantle cell lymphoma (MCL) is a well-defined peripheral B-cell lymphoma usually diagnosed upon peripheral lymph node biopsy. We report eight cases of peripheral B-cell leukaemia that demonstrate presumptive evidence of mantle cell characteristics. The patients had a median age of 68.5 years, and five were male. All presented with an enlarged spleen without any peripheral lymphadenopathies, and they were leukaemic at presentation (median lymphocytosis, 38x10(9)/l). Morphological diagnosis of MCL was very difficult in five cases but easier in three because we were able to analyse either pre- or post-mortem lymph nodes and spleen. The immunophenotype of blood lymphocytosis using flow cytometry, the presence of a t(11;14)(q13;q32) and a cyclin D1 expression by leukaemic cells all fit with the diagnosis of MCL. All patients progressed and died with a median overall survival of 8 months. Multifocal areas of transformation in blastoid or large cell variants were observed in the three autopsied patients. In summary, one should consider the diagnosis of MCL at presentation in leukaemic phase even in the absence of peripheral adenopathies.

Aged↗

Quality control and sensitivity of polymerase chain reaction techniques for the assessment of immunoglobulin heavy chain gene rearrangements from fixed- and paraffin-embedded samples.

Frozen tissue is considered the gold standard if DNA is to be extracted for polymerase chain reaction (PCR) analysis. In molecular studies from paraffin-embedded material, only positive results are usually taken into account. Our goal was to evaluate both the sensitivity and the specificity of PCR techniques for immunoglobulin heavy chain (IgH) gene rearrangement according to various lengths and types of fixative before paraffin embedding. One set of studies compared IgH rearrangement in a case of mantle cell lymphoma tissue that had been fixed in 14 different ways before paraffin embedding and frozen tissue. Formalin fixation was found not to be deleterious for DNA, amplification being possible up to 15 days after fixation with good sensitivity. In contrast, the performance of PCR decreased for samples fixed in Bouin's liquid for longer than 6 hours or after 48 hours of incubation in a vacuum infiltration processor (in which Bouin's liquid-fixed and formalin-fixed samples are mixed). In addition, we undertook a retrospective study of 20 routinely processed B-cell lymphomas, with frozen formalin-fixed and Bouin's liquid-fixed tissues for each case. Of the 14 positive cases on frozen material, 13 were also clonal from paraffin-embedded tissues. Whatever the IgH locus analyzed, each time the adapted control was positive, results from paraffin-embedded material were identical to results obtained from frozen tissue. In this study, we showed that the use of paraffin-embedded tissue is efficient for the study of IgH gene rearrangement. Whenever adapted controls are used, it is even possible to assess negative results.

DNA Primers↗

Bone marrow manifestations of infections and systemic diseases observed in bone marrow trephine biopsy review.

Bone marrow modifications resulting from infections and systemic diseases can be studied by analysis of morphology and aetiology. Two types of lesions or modifications can be observed, those occurring in the connective tissue comprising inflammatory processes, acute and chronic, as well as immune reactions, and those involving the normal haematopoietic cell lines, with possible hyperplastic or aplastic changes in one or more cell lines. The main lesions are described (oedema, haemorrhage, necrosis, suppuration, granulomas, lymphoid nodules and hyperplasia, immunoblastic or plasmacytic hyperplasia), as well as the main aetiologies. In association, the three main haematopoietic cell lines show hyperplasia, hypoplasia, aplasia of one or all of the cell lines, sometimes with dysmyelopoiesis. The stroma and vessel reactions comprise myelofibrosis, gelatinous transformation or amyloid deposits. The methods for identifying aetiological agents are emphasized. It should also be stressed that malignant neoplasias of different types involving the bone marrow can be responsible for such inflammatory or immune reactions.

Bacterial Infections↗

Intravascular large B-cell lymphoma with bone marrow involvement at presentation and haemophagocytic syndrome: two Western cases in favour of a specific variant.

AIMS: To report two cases of an unusual form of intravascular lymphoma, characterized by bone marrow involvement at presentation with haemophagocytic syndrome. METHODS AND RESULTS: We describe the clinicopathological features of two patients with intravascular lymphoma primarily involving bone marrow. Both patients complained only of fever with pancytopenia and reactive haemophagocytic syndrome. Diagnosis was made on bone marrow examination, which showed large tumour cells of B-cell lineage confined within the lumen of sinuses. CONCLUSION: These two cases and five previous reports could represent a variant of intravascular lymphoma, characterized by early involvement of bone marrow without dissemination to other organs. This form of intravascular lymphoma, called IVL-HS, seems to be an 'Asian' variant with a high prevalence in Asian people and a very low prevalence in Western countries. At a practical level, bone marrow biopsy may be useful in the diagnosis of intravascular lymphoma when the clinical presentation is restricted to fever of unknown origin with a reactive haemophagocytic syndrome.

Aged↗

Splenic marginal zone lymphoma with or without plasmacytic differentiation.

We report a series of 31 cases of splenic marginal zone lymphomas with an enlarged spleen and a multimicronodular macroscopic pattern. Two groups, A and B, were distinguished based on the presence (A) or absence (B) of a lymphoplasmacytic component with monoclonal immunoglobulin expression in the cytoplasm. There were no differences between the groups as far as age, sex, spleen weight, and progression. The only difference was the presence in group A of a monoclonal serum component and autoimmune disorders, particularly autoimmune hemolytic anemia. In most cases in which a liver and/or bone marrow biopsy was performed, lymphomatous infiltration was detected. Seven cases had a seric monoclonal IgM of 5 g/L or more and liver or bone marrow infiltration, corresponding to the definition of Waldenstrom's macroglobulinemia. Lymphoma cells had a monocytoid, centrocytoid and, in group A, lymphoplasmacytic morphology. The lymphomatous cells were positive for CD20, CD45 RA, and bcl-2. They expressed IgD in 9 cases, partially in 6, and were negative for IgD in 9 of the 24 cases studied. Progression seems to be slow, with a long survival. Three patients presented with transformation into a large B-cell lymphoma, which was responsible for death in two patients.

Adult↗

Spleen localization of light chain deposition disease associated with sea blue histiocytosis, revealed by spontaneous rupture.

Splenic involvement by a light chain deposition disease (LCDD) associated with sea-blue histiocytosis occurred in a 55-year-old man presenting with LCDD of the kidney without myeloma. Lambda light chain deposits were demonstrated by immunohistochemistry in vessel walls and along the ring fibres of the red pulp sinuses. Accumulation of sea blue histiocytes in the cords was also present. Stiffness of the walls of the red pulp sinuses resulting from light chain deposits may have induced accumulation and destruction of circulating blood cells. Lipid catabolism with production of ceroids may have resulted in lipidic histiocytosis with a sea blue histiocyte pattern.

Humans↗

Incidence of haematological malignancies in French Polynesia between 1990 and 1995.

To determine the incidence of haematological malignancies in French Polynesia from 1990 to 1995, we collected cases from the local cancer registry, sanitary evacuation files and all the histopathology and clinical biology laboratories. All leukaemias, non Hodgkin's lymphomas, and multiple myelomas incidence was slightly lower among French Polynesians than among Maoris from New-Zealand and Hawaiians of Hawaii. Standardised Incidence Ratio (SIR) for Hodgkin's disease among females was 0.08 when comparing to Hawaiians and 0.33 when comparing to Maoris. Other salient features were a high proportion of high grade and Burkitt's lymphoma, the absence of Hodgkin's disease after 40 years of age, a low incidence of chronic lymphoid leukaemia, and a high non lymphoblastic/lymphoblastic acute leukaemia ratio in childhood. This study stresses the peculiar incidence of some haematological malignancies in this south pacific area.

Adolescent↗

[Orbital lymphoma and AIDS. Apropos of a case].

We report the case of a 33-year-old HIV-seropositive male who had a large cell Epstein-Barr virus-associated non-Hodgkin's orbital lymphoma. A thorough medical examination enabled us to find on thoracic CT-scan a mediastinal lymph node involvement as large as 18 cm in diameter. The regression of the tumor was dramatic under chemotherapy with complete disappearance of the tumor and no recurrence was found after more than 21 months follow-up. We discuss the differential diagnosis with orbital infection.

Adult↗

Sea-blue histiocyte syndrome in bone marrow secondary to total parenteral nutrition.

Clinical and hematological abnormalities can occur in patients receiving intravenous fat emulsions as part of a long-term parenteral nutrition; they consist of hepatosplenomegaly and peripheral blood cytopenia(s). These abnormalities lead to bone marrow examination which revealed numerous macrophages laden with blue staining pigment granules and separate lipid vacuoles, presenting the typical histochemical characteristics of sea-blue histiocytes. Thus, long-term parenteral nutrition including fat-emulsion sources may represent a further condition in addition to the wide variety of disorders which can be associated with sea-blue histiocytosis. Moreover, in view of its clinical and morphological presentation, this storage pathological state could be compared with the so-called sea-blue histiocyte syndrome described by Silverstein and colleagues.

Bone Marrow↗

Sphenoid sinus localization of multiple myeloma revealing evolution from benign gammopathy.

BACKGROUND: Plasma-cell neoplasms of the head and neck include extramedullary plasmacytoma and solitary plasmocytoma of bone or may represent a local manifestation of multiple myeloma. Involvement of sphenoid sinus has been rarely reported in multiple myeloma. METHODS: We present the case of a 77-year-old man with a 3-year-history of benign monoclonal IgG-lambda gammopathy who developed left sixth-nerve palsy and malaise. RESULTS: Computed tomography scan and magnetic resonance imaging scan disclosed a large soft-tissue mass of the sphenoid sinus with bone destruction. Sphenoid sinus biopsy revealed an IgG monoclonal plasma cell neoplasm. Diagnosis of multiple myeloma stage IA was then established. CONCLUSIONS: Diagnosis of plasma-cell neoplasm should be considered in sphenoid sinus tumors and depends upon histologic examination. This case enlightens the relationships between monoclonal benign gammopathy and plasma-cell neoplasms of the head and neck which constitute a continuum of B-cell lymphoproliferative disorders.

Aged↗

Disseminated superficial porokeratosis after autologous bone marrow transplantation.

A case of disseminated superficial porokeratosis (DSP) is reported in a black man 5 years after autologous bone marrow transplantation (BMT) for acute promyelocytic leukemia. Porokeratosis is a rare hyperkeratotic disorder arising from clonal keratinocytes with a high potential to develop squamous cell carcinoma. Inherited forms are classical but recent observations of acquired porokeratosis have been reported in immunocompromized patients (AIDS, immune disorders, immune suppressive drugs or organ transplantation). Two cases of DSP have been reported after allogeneic BMT in patients treated for chronic GVHD. Our case is the first one after autologous BMT, in a black man, on no immunosuppressive drug at the time of diagnosis of DSP. Hematopoietic and immune reconstitution was apparently complete. The cancer-prone character of porokeratosis could be favored by total body irradiation used in conditioning regimen. Thus, porokeratosis has to be associated with other late effects after BMT such as HCV seropositivity, cataract and infertility that were observed in this patient.

Adult↗

[Rare non-Hodgkin's lymphoma in the course of infection with human immunodeficiency virus. Two cases with bone marrow invasion].

Malignant non Hodgkin's lymphomas in patients with acquired immunodeficiency syndrome are usually of high grade and have a B-cell phenotype. We describe two cases of rare lymphomas during acquired immunodeficiency syndrome: one case of pleomorphic T cell lymphoma with medium and large cell predominance, of high grade of malignancy, according to the updated Kiel classification, involving mainly the bone-marrow, and one case of low grade monocytoid B-cell lymphoma with bone-marrow extension, which is a rare condition in this type of lymphoma. These lymphomas seem to be a casual event during acquired immunodeficiency syndrome, but the immunodeficiency can be responsible of a more aggressive behavior.

Adult↗

Epstein-Barr virus-associated lymphoproliferative disease during methotrexate therapy for psoriasis.

BACKGROUND: Epstein-Barr virus (EBV)-associated lymphoproliferative disorders have recently been observed during treatment of rheumatoid arthritis and dermatomyositis with low-dose methotrexate. OBSERVATION: A patient with psoriasis developed a B-cell lymphoproliferative disorder during long-term treatment with low-dose methotrexate. The lymphoid cells expressed EBV latent membrane protein 1, and the EBV viral genome was present as demonstrated by in situ hybridization. Evaluation for EBV clonality showed that the lymph node contained clonal EBV DNA. Polymerase chain reaction studies confirmed that the B-cell lymphoproliferative disorder was mainly monoclonal, suggesting that the disorder arose from a single EBV-infected B-cell clone. CONCLUSIONS: Lymphoproliferative disorders associated with Epstein-Barr virus in which the clinicopathological presentation is similar to those occurring in patients after transplantation may be observed in patients with psoriasis treated with methotrexate. While it is impossible to rule out a fortuitous occurrence of an EBV-associated lymphoproliferative disorder and psoriasis treated with methotrexate in the same patient, EBV appears to be critical in the pathogenesis of the lymphoproliferative disorder in this patient.

Antigens, Viral↗

Bone marrow involvement by disseminated toxoplasmosis in acquired immunodeficiency syndrome: the value of bone marrow trephine biopsy and immunohistochemistry for the diagnosis.

A bone marrow biopsy was performed on four patients with acquired immune deficiency syndrome (AIDS) for a long-running course fever of unknown origin associated with a recent pancytopenia. In the four cases, striking histological similarities, such as interstitial edema, foci of necrosis and only few scattered or clustered histiocytes, were found. Near or in the foci of necrosis, free forms, and pseudocysts of Toxoplasma gondii were observed not only in the cytoplasm of macrophages and of some granulocytes, but also within megakaryocytes. No sign of other parasitic, bacterial, or fungus infection has been found. The diagnosis was confirmed by immunohistochemistry in the four cases and ultrastructural examination in one case. This case study stresses the importance of bone marrow histological changes for the diagnosis of severe toxoplasmosis in AIDS patients and particularly the localization of T gondii within the cytoplasm of megakaryocytes.

AIDS-Related Opportunistic Infections↗

A case report of Down syndrome and centroblastic lymphoma.

We describe a case of left cervical stage I centroblastic lymphoma in a 29-year old male patient with Down's syndrome due to a (14; 21) Robertsonian translocation. The disease presented as extensive lymph node necrosis leaving rare areas of tumor cells, accounting for the diagnostic difficulties. According to our review of the literature, lymphoma is one of the most common neoplasms in DS patients and may represent the second most common malignancy in this condition, far behind leukemia.

Adult↗

Detection of bcl-2 rearrangement in HIV-related follicular lymphoid hyperplasia.

Rearrangement of the bcl-2 gene at the MBR (major breakpoint region) locus with the immunoglobulin heavy-chain joining region has been reported in a high proportion of follicular lymphomas. This rearrangement has also been reported in very few normal B cells of the blood, tonsils, follicular lymphoid hyperplasia (FLH) of the lymph nodes. HIV infection is often associated at the onset of the disease with FLH, but the presence of rearranged bcl-2 B cells in these lymph nodes has not been described. In using a standard PCR assay with Southern blot or a semi-nested PCR on 48 cases of FLH, we demonstrated that there were a few bcl-2 rearranged B cells in HIV FLH, at almost the same level as that in non-HIV-related FLH. The usual absence of bcl-2 rearrangement in the HIV-associated B-cell lymphomas suggests that the bcl-2 oncogene in the rearranged B cells of FLH is not cooperating with other oncogenes during HIV lymphomagenesis.

Adolescent↗

Sea-blue histiocyte syndrome in bone marrow secondary to total parenteral nutrition including fat-emulsion sources: a clinicopathologic study of seven cases.

Bone marrow examination revealed a lipid-laden histiocytosis in seven patients undergoing long-term total parenteral nutrition necessitated by extensive short-bowel surgical resection. Clinical abnormalities occurred during this treatment which required bone marrow examination. These included hepatosplenomegaly and peripheral blood cytopenia; the median time to the detection of these abnormalities was 64 months. The most striking change within the bone marrow was the presence of many pigment-laden histiocytes which had the typical morphology of sea-blue histiocytes seen in the so-called idiopathic sea-blue histiocyte syndrome. The occurrence of sea-blue histiocytosis in the bone marrow in association with long-term parenteral nutrition for short-bowel syndrome has not, to our knowledge, been reported previously and should now be considered in the differential diagnosis of bone marrow sea-blue histiocytosis.

Adult↗