Biomedical subjects

A Chott

Publications and source records attributed to A Chott.

At least 109 records · Page 6Linked to original sources

PubMed1996

The dominant T cell clone is present in multiple regressing skin lesions and associated T cell lymphomas of patients with lymphomatoid papulosis.

This study was undertaken to determine the clonality of lymphomatoid papulosis (LyP), its clonal relationship to lymphomas, which occur at high frequency in LyP patients, and to define the cell lineage of Reed-Sternberg-like cells in type A lesions of LyP. Punch biopsies of skin of 11 adult patients with LyP were analyzed for morphologic subtype of LyP, surface antigens, and clonal T-cell receptor (TCR) gene rearrangements. Clonal rearrangements were identified by semiquantitative polymerase chain reaction amplification and sequencing of TCR-beta chain genes in nine patients and TCR-gamma chain genes in two patients. A single dominant clone was detected in multiple separate LyP lesions, often of different histologies, in nine patients. The same clone was detected in LyP lesions and the anaplastic large cell lymphoma (ALCL) of 2 patients and the mycosis fungoides (MF) of 2 other patients. No dominant clone could be detected in one patient with LyP uncomplicated by lymphoma or in a second patient with LyP and MF. A T-cell lineage was evident for RS-like cells in cell culture and in type A lesions. These results show that multiple regressing skin lesions and associated T cell lymphomas (MF and ALCL) are clonally related in most LyP patients, which suggest that the disease in these patients was initiated by a non-random genetic event.

Adult↗

PubMed1995

Structure and function of the CD1 family of MHC-like cell surface proteins.

The CD1 family of proteins are structurally related to MHC class I proteins, but are only distantly related to the class I proteins or other MHC-linked class I-like proteins. Sequence comparisons indicate that the CD1 proteins have evolved into two subfamilies, those which are similar to human CD1a, b, and c and those which are similar to human CD1d. The CD1A-, B-, and C-like genes were deleted from rodents and the CD1D gene was duplicated. CD1a, b, and c are expressed by thymocytes, dendritic cells, activated monocytes, and B cells (CD1c), a tissue distribution which strongly suggests a role in antigen presentation. In contrast, CD1d and its murine homologues are expressed by many cells outside of the lymphoid and myeloid lineages. The CD1 proteins are in most cases expressed as beta 2mg-associated membrane glycoproteins, but may associate with additional proteins. CD1d is expressed on the surface of intestinal epithelial cells in a nonglycosylvated form without beta 2mg. Whether the CD1 proteins function as antigen-presenting molecules is unresolved, but it is unlikely that they present conventional peptide antigens. Strong evidence indicates that murine CD1 proteins are recognized by a population of NK1.1+, CD4+ or CD4-CD8- (double negative, DN) T cells which express an invariant TCR alpha chain. CD1d is most likely recognized by the homologous T cell population in humans. DN alpha beta T cells which recognize CD1a, b, or c have been isolated, including clones which recognize a lipid antigen from mycobacteria presented by CD1b. A third potential population of CD1 reactive cells are CD8+ T cells in the intestinal epithelium. Taken together, these observations indicate that CD1 proteins interact with several specialized populations of T cells. The precise biological functions mediated through these interactions remain to be determined.

Amino Acid Sequence↗

PubMed1995

Increase of bone marrow cellularity during erythropoietin treatment in myeloma.

Treatment of myeloma-associated chronic anemia with recombinant human erythropoietin (rHuEPO) has been shown to be successful in the majority of patients. We have morphometrically investigated bone marrow sections from the iliac crest of 20 anemic myeloma patients prior to rHuEPO therapy. The 15 responding patients were re-examined after three months and, if possible, after 6 and 12 months of treatment. Significant differences were found between responders and nonresponders prior to therapy. Nonresponders presented with a pronounced shift to the right in their erythroid bone marrow cell compartment and partly with higher serum levels of endogenous erythropoietin. During rHuEPO therapy, responders showed increases in all subsets of erythropoiesis and in the total amount of hemopoietic tissue. Response was accompanied by a marked drop of serum ferritin levels, a rise in serum levels of transferrin receptors and the emptying of bone marrow iron stores; the World Health Organization performance status improved. Responders tended to present with less advanced disease stages and better performance status and showed significantly longer survival times. Loss of responsiveness to rHuEPO was observed in one patient during the terminal stage of the disease. In conclusion, morphometric examination of bone marrow biopsies during the course of rHuEPO therapy showed that the response achieved in hemoglobin values was clearly mirrored in equivalent increments of the erythroid bone marrow cell compartment.

Aged↗

PubMed1994

Granulocytic sarcoma of the prostate as the first manifestation of a late relapse of acute myelogenous leukemia.

We describe a 68-year-old patient who developed granulocytic sarcoma of the prostate 9 years after complete remission following successful treatment of acute myelogenous leukemia (FAB, M2). PCR analysis of bone marrow samples in first remission and at the time of relapse detected an AML1/ETO rearrangement typical for AMLs with t (8;21). The CD56 antigen was not expressed on the leukemic cells. Systemic chemotherapy led to a short-lasting regression of the tumor, but the patient subsequently developed overt bone marrow relapse and died during chemotherapy. While granulocytic sarcoma as a primary manifestation of AML is well known, as the first manifestation of relapse it appears to be very uncommon.

Aged↗

PubMed1994

Karyotype and prognosis in non-Hodgkin lymphoma.

In malignant non-Hodgkin lymphomas (NHL), cytogenetic analysis may provide prognostic information including prediction of histologic evolution and responsiveness to therapy. In this study, we correlate clinical data and chromosomal aberrations in 70 adult patients with newly diagnosed NHL followed for a median of 20 months. Clonal aberrations were detected in 68/70 patients (97%). Besides t(2;5)(p23;q35), observed exclusively in three patients with anaplastic large cell lymphoma, Ki-1 positive, none of the characteristic aberrations observed was specific for a given histological subtype. Aberrations of chromosome 7 (n = 21) occurred in all histological subtypes together with aberrations of chromosome 3 and of the short arm of chromosome 17. They were clinically associated with a high serum lactate dehydrogenase level (LDH) and a trend to short survival. Anomalies of the long arm of chromosome 13 (n = 10) were found in patients with high grade B-cell lymphomas and bulky disease. In t(14;18)(q32;q21) bearing lymphomas (n = 27), distinct patterns of additional aberrations were observed in low grade and high grade lymphomas: trisomy 3 and trisomy 18 occurred concomitantly in high grade lymphomas (n = 6, p < 0.001) as well as aberrations of 1q, 5q, 6q and +der (18)(q21). In conclusion, cytogenetic analysis provides information about the complexity of genetic changes in NHL. These changes act not only as indicators of disease activity, but influence clinical outcome as demonstrated by their stringent correlation to the International Index and might reveal more general rules of tumor growth and spreading.

Adolescent↗

PubMed1993

[Manifestation of malignant lymphomatous polyposis of the nasopharynx].

A 66 year old male patient with malignant lymphomatous polyposis of the gastrointestinal tract showed a manifestation in the nasopharynx. After 6 cycles of chemotherapy according to the CHOP-Scheme complete remission could be observed. 9 months after therapy no signs of recurrence were evident.

Aged↗

PubMed1992

[Malignant lymphomatous polyposis of the gastrointestinal tract].

Two years after endoscopic excision of several polyps of the colon (histological examination revealing infiltration by a malignant non-Hodgkin lymphoma) a 67-year-old man was again found to have blood and mucus in his stool. Because local recurrence was suspected a coloscopy was performed. This revealed multiple polyps throughout the entire colon, predominantly in the sigmoid and rectum. Biopsy showed focal infiltration of the submucosa and mucosa with centrocytic non-Hodgkin lymphoma, compatible with the diagnosis of malignant lymphomatous polyposis of the gastrointestinal tract. Gastroduodenoscopy then demonstrated duodenal polyps and massive coarsening of the gastric folds due to infiltration. For the first time tumour masses were also found in the epipharynx. After 6 chemotherapy cycles according to the CHOP scheme (day 1: cyclophosphamide, 1500 mg, adriblastin, 100 mg and vincristine, 2 mg; days 1-5: prednisolone, 100 mg) all lymphoma infiltrates regressed completely. The patient has been symptom-free for 9 months.

Aged↗

PubMed1992

MACOP-B treatment in patients with Ki-1-positive large-cell anaplastic lymphoma.

Nine adult patients with Ki-1-positive large-cell anaplastic lymphoma were treated with MACOP-B. Two suffered from relapsed disease and had previously received chemotherapy; a third patient had received a single dose of 100 mg/m2 cisplatin before initiation of MACOP-B. The stage of lymphoma was determined according to the Ann Arbor Conference criteria and was II in one, III in two and IV in six patients. All patients had constitutional symptoms. Five patients had achieved complete remission 4 weeks after termination of the protocol and there were two partial remissions. One patient died of massive pulmonary embolism during the 4th week of treatment; another patient, who had received MACOP-B as salvage therapy, died of progressive lymphoma 1 month after completion of the regimen. Maximal observed toxicities according to WHO were mucositis grade 3 (n = 3) and there were three cases with thromboembolic complications, including a fatal pulmonary embolism in a young patient. However, MACOP-B appears an effective, fairly well-tolerated and feasible therapy for patients with Ki-1-positive large-cell anaplastic lymphoma.

Adult↗

PubMed1992

Simultaneous occurrence of t(14;18) and t(8;22) common acute lymphoblastic leukemia.

A young male patient progressed rapidly from localized abdominal lymph node enlargement to overt acute lymphoblastic leukemia. Despite aggressive treatment, he died of progressive CNS leukemia 5 months after initial presentation. At diagnosis, karyotypic analysis of an abdominal lymph node revealed the coexistence of t(14;18) (q32;q21), specific for follicular lymphoma, and t(8;22) (q24;q11), a variant Burkitt translocation. Such cases might be considered as a model for a general mechanism of tumor progression with cascade-like involvement of oncogenes.

Adult↗

PubMed1992

Recurrent granulocytic aplasia as clinical presentation of a persistent parvovirus B19 infection.

We report a case of persistent infection with human parvovirus B19 (PVB19), manifesting clinically as recurrent agranulocytosis and in bone marrow biopsy as recurrent pure granulocytic aplasia. Persistence of parvovirus infection was documented by the presence of PVB19 DNA and anti-PVB19-IgM antibodies in the serum for a period of 19 months. Granulocytic aplasia occurred only when anti-PVB19-IgG antibodies were not detectable in the serum and granulopoiesis showed immediate recovery with high dose intravenous immunoglobulin treatment. This case report suggests that the erythroid precursor cell may not be the only target cell of PVB19 infection. We suggest testing for active parvovirus infection in cases of aplasia of any lineage of the haematopoietic system.

Aged↗

PubMed1992

Peripheral T-cell lymphomas of the intestine.

Twenty-seven cases of primary peripheral T-cell lymphomas of the intestine (PTLI) were investigated. Seven patients had histories of malabsorption. The most frequent symptoms at presentation were weight loss, abdominal pain, and acute abdomen. The jejunum was the most common site of lymphoma and multifocal disease was found in 72% of the cases. Twenty-two patients (92%) presented with localized disease confined to the intestine and abdominal lymph nodes, only two patients had generalized disease. According to the pattern of lymphoma infiltration and the morphology of the uninvolved small intestinal mucosa, 21 cases were separated histologically into three categories; 1) enteropathy-associated T-cell lymphoma (EATCL, n = 9) showing predominant intramucosal lymphoma spread and villous atrophy of uninvolved mucosa with high density of intraepithelial lymphocytes (IEL), 2) EATCL-like lymphoma without enteropathy (EATCL-LLWE, n = 5) but with an infiltration pattern similar to EATCL, and 3) T-cell lymphoma without features of EATCL (Non-EATCL, n = 7). Distinctive features of EATCL were the high incidence of malabsorption states, multifocal intestinal disease in all cases, and the high frequency of intestinal recurrences. On frozen sections four of eight PTLI showed the phenotype CD3+ CD4- CD8- HML-1+, which is also expressed on a small subset of normal IEL. The morphologic and immunomorphologic findings suggest that the majority of PTLI is derived from mucosal T lymphocytes. This derivation may be responsible for certain biologic features, such as the preferential spread to and relapse of PTLI at small intestinal sites.

Adult↗

PubMed1991

Translocation (16;21)(p11;q22) in acute monoblastic leukemia with erythrophagocytosis.

A patient with acute monoblastic leukemia with erythrophagocytosis and a t(16;21) (p11;q22), poor response to chemotherapy, early relapse, and a short survival of ten months is presented. Hematologically, this patient could be considered as a case of FAB M5b/t(8;16) but without the characteristic chromosomal translocation, i.e., there is no visible alteration on chromosome 8 and the breakpoint on chromosome 16 appears to be very proximal. These findings are briefly discussed in the light of other variants.

Adult↗

PubMed1991

Hepatic and splenic abscesses--a common complication of intensive chemotherapy of acute myeloid leukemia (AML). A prospective study.

In order to determine the frequency of hepatosplenic abscesses in AML patients during chemotherapy and to evaluate the clinical and laboratory characteristics of this complication we performed a prospective study over a 28-month period. Fifty-five consecutive patients with de novo AML or relapse who received intensive chemotherapy underwent regular ultrasound examinations. In 16 patients (29.1%) hepatic and/or splenic abscesses were detected sonographically. Histopathological evidence for abscess formation was obtained in five of these 16 patients. In three patients granulation tissue and in one patient necrotizing granulomas were found. Causative micro-organisms were proven in only three patients: Candida hyphae were demonstrated in one patient, gram-positive cocci in another. Bacteria and fungi were seen in the tissue specimen of the third patient. Patients with hepatosplenic abscesses had significantly prolonged fever after neutrophil recovery but did not differ from patients without abscesses in any other laboratory or clinical features. Due to the absence of specific alerting clinical and laboratory signs and symptoms of hepatosplenic abscesses, routine ultrasound examination is required for detection of this complication. The presence of hepatic and/or splenic abscesses does not necessarily worsen the prognosis, but it may influence the decision on further chemotherapy and antimicrobial treatment.

Abscess↗

PubMed1991

Interferon in essential thrombocythaemia.

In the present investigation, 20 patients with ET were treated with recombinant interferon alfa-2c (IFN) for up to 4 years. Initially, IFN was administered subcutaneously at a dosage of 6-45 MU/week. The dosage was adjusted according to individual tolerance and response. The median dose during induction was 20 MU/week, 10 MU/week during the remaining first year, 6 MU/week during the second year and 2 MU/week thereafter. 13 patients (65%) achieved complete remission (platelet count less than 440/nl), four patients (20%) had partial remission (greater than 440/nl but a reduction by more than 50% of the initial count). The median platelet count remained steady throughout the 4-year period of treatment, in spite of extreme dose reductions. After withdrawal of IFN, however, platelet counts again increased. The white blood cells showed a marked decrease similar to that of platelet counts, whereas the haemoglobin level remained fairly stable. In the bone marrow, a significant decrease in megakaryocyte density and size could be observed. Concurrently with the improvement of haematological parameters, clinical symptoms improved, but reappeared after withdrawal of IFN. During induction, fever, bone and/or muscle pain, fatigue, lethargy and psychological symptoms were the most prominent side-effects in the majority of patients. In three patients these symptoms led to discontinuation of the treatment. With repeated dose reductions, excellent long-term tolerance was achieved, and during late maintenance treatment the only observed side-effect was an induction of thyroid autoimmunity in three patients. IFN is an effective, well-tolerated alternative in the long-term treatment of symptomatic ET. However, since withdrawal of IFN leads to recurrence of thrombocytosis, continued treatment is to be recommended.

Adult↗

PubMed1991

[Low dosage long-term treatment with recombinant alpha interferon induces in hairy cell leukemia continuous complete remission].

Long-term treatment with recombinant interferon alpha (IFN) leading to complete remission in a patient with hairy cell leukaemia is presented. Four months after the beginning of treatment with IFN, the parameters of the peripheral blood had normalized. In spite of a continuous dose reduction (to a dose of 1 Mio. U/week) remission could be maintained during the six years of treatment. Two years after the start of treatment with IFN, a metastasizing carcinoma of the prostate was diagnosed, leading to death after four years. With regard to the hairy cell leukaemia, the patient was free of symptoms and the obduction confirmed the complete remission: the histological examination of bone marrow, spleen, liver and lymph nodes showed no more signs of hairy cell leukaemia. This case report shows that long-term treatment with IFN in some cases is effective to induce and to maintain complete remission in hairy cell leukaemia.

Aged↗

PubMed1991

[Angiolymphoid hyperplasia with eosinophilia. Histologic study and successful therapy with the argon laser].

We report a case of angiolymphoid hyperplasia with eosinophilia (AHE) with regional lymphadenopathy. The multiple papules were composed of both a lymphoid infiltrate of predominant T-cell type with eosinophils and anomalous vascular proliferation lined by "histiocytoid" endothelial cells, which were in close association with a ruined artery at the subcutaneous level in the largest nodule. Immunoelectron microscopy revealed factor-VIII-related antigen to be concentrated within the Weibel-Palade bodies of the endothelial cells. Their cytoplasmic filaments consisted of vimentin. In the pericytes large amounts of actin were demonstrable. Since AHE has a benign progressive course and only local recurrence is noted, the lesions were treated with argon laser. Subsequently, lymphadenopathy resolved spontaneously and the blood parameters normalized. There is no evidence of recurrence 1 year after treatment.

Angiolymphoid Hyperplasia with Eosinophilia↗

PubMed1990

Peripheral T-cell lymphomas: a clinicopathologic study of 75 cases.

Seventy-five peripheral T-cell lymphomas (PTLs) were classified according to the recently proposed "Updated Kiel Classification of Non-Hodgkin's Lymphomas" (mycosis fungoides and Sezary's syndrome excluded). Thirty-seven PTLs belonged to the low-grade category (T-cell chronic lymphocytic leukemia [T-CLL], 3; lymphoepithelioid, 4; angioimmunoblastic, 22; T-zone, 6; pleomorphic small cell, 2) and 38 belonged to the high-grade category (pleomorphic medium and large cell, 24; immunoblastic, 1; large-cell anaplastic Ki-1-positive, 13). Loss of pan-T antigens occurred exclusively in high-grade PTLs; on paraffin sections UCHL 1 was slightly more sensitive than MT 1. Sixty patients presented with lymphadenopathy and 15 patients (20%) presented with extranodal disease most frequently affecting the skin and upper aerodigestive tract. B-cell lymphoma symptoms were found in 43 cases (57%) and bone marrow involvement (T-CLL excluded) was found in 12 cases (17%). Staging (T-CLL excluded) revealed stage I in 13%, stage II in 15%, and stages III and IV in 72% of the cases. Among the intensively treated patients, 37% achieved complete remission and 15 are still in complete remission after 4 to 79 months (median: 24 months). The overall median survival (MS) rate was 23 months. Peripheral T-cell lymphoma of pleomorphic medium and large-cell type was the most aggressive lymphoma (MS: 8 months). B-cell lymphoma symptoms, bone marrow involvement, and Ki-67 positivity 60% or greater significantly shortened survival times, whereas age (under 60 versus over 60 years), stage (I and II versus III and IV), and grade had no significant influence. Ki-67 reactivity was found to be a prognostic factor which allows prediction of probable poor outcome, especially in cases with limited stage of disease.

Adolescent↗

PubMed1990

Ki-1-positive large cell lymphoma. A clinicopathologic study of 41 cases.

We report the clinicopathologic findings of 41 patients with Ki-1 (CD30)-positive large cell lymphoma. The median age was 50 years; 13 patients were under 40 years of age. Ten patients presented with extranodal disease. Fifty-five percent of the patients presented with stage I or II disease, and bone marrow involvement was histologically documented in 30% and occurred exclusively in patients over 40 years of age. Two cytomorphologically distinct groups of Ki-1--positive large cell lymphomas could be separated. Group A lymphomas consisted of pleomorphic large cells, sometimes with wreathlike and embryo-like nuclei, whereas group B lymphomas displayed a rather monomorphic appearance. Clinically the two groups of lymphomas differed with respect to stage of disease, frequency of bone marrow involvement, and median survival. On paraffin sections, the Ki-1--related antibody Ber-H2 provided excellent staining results in all cases. Immunologic phenotyping disclosed a T cell type in the majority of cases, revealed marked loss of differentiation antigens, and frequent expression of HLA-DR and IL-2 receptor. The overall median survival was 13 months. Age below 40 years, limited stage of disease (I and II), and, although not statistically significant, lymphoma morphology were associated with longer survival. We conclude, that Ki-1--positive large cell lymphomas represent a morphologically and immunologically heterogeneous category of hematolymphoid neoplasms derived from dedifferentiated and activated lymphoid cells with marked age-dependent prognosis.

Adult↗