| 1. |
- Andersson, Per-Ola, 1964, et al.
(författare)
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Leukemic transformation of essential thrombocythemia without previous cytoreductive treatment.
- 2000
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Ingår i: Annals of hematology. - 0939-5555. ; 79:1, s. 40-2
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Tidskriftsartikel (refereegranskat)abstract
- Blastic transformation of essential thrombocythemia (ET) preceded by chemotherapy is occasionally described in the literature. In ET as well as in other myeloproliferative disorders the leukemogenic effect of alkylating agents and (32)P is well established, and recent reports also indicate a certain leukemogenic effect of hydroxyurea in these disorders. However, leukemic transformation in untreated ET seems to be a rare event. This is probably due to the fact that, at some time during their clinical course, most ET patients receive chemotherapy and are thereby exposed to leukemogenic challenge. We report on a woman with ET who had not received cytoreductive treatment prior to the development of acute myeloid leukemia, indicating that this transformation was a natural progression of her disorder.
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| 2. |
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| 3. |
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| 4. |
- Hasselblom, Sverker, et al.
(författare)
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Testicular lymphoma--a retrospective, population-based, clinical and immunohistochemical study.
- 2004
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Ingår i: Acta oncologica (Stockholm, Sweden). - : Informa UK Limited. - 0284-186X .- 1651-226X. ; 43:8, s. 758-65
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Tidskriftsartikel (refereegranskat)abstract
- From a population-based registry, 35 patients with histologically verified testicular lymphomas were identified: diffuse large B-cell lymphomas (DLBCL) in 33 and peripheral T-cell lymphomas in two cases. Twenty-two patients had localized disease (Pe stage I and II). Twenty-eight patients received systemic chemotherapy, 17 of whom also received intrathecal prophylaxis, and 12 out of these 17 also received radiotherapy to the contralateral testis. In the Pe stage I/II group, 7 out of 21 patients in complete remission (CR) relapsed. In 5 of them the CNS was involved (isolated CNS relapse in three). Remarkably late relapses occurred (up to 127 months). Intrathecal prophylaxis seemed to reduce the frequency of relapses involving the CNS, but the relatively short follow-up (median 45 months, range 34-88, for censored patients) prevents firm conclusions regarding efficacy. The outcome for the stage IV patients was poor, with only 1 out of 11 patients in continuous CR. Immunohistochemical analysis of the DLBCL tumours revealed that 31% had the germinal centre B-cell-like phenotype. CD44 was expressed in all the tumours of stage IV patients but in less than half of the Pe stage I/II patients. A high intratumoural microvessel density was correlated with a high degree of Ki-67 positive tumour cells and an inferior overall survival.
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| 5. |
- Hou, M, et al.
(författare)
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Plasma thrombopoietin levels in thrombocytopenic states: implication for a regulatory role of bone marrow megakaryocytes.
- 1998
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Ingår i: British journal of haematology. - 0007-1048. ; 101:3, s. 420-4
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Tidskriftsartikel (refereegranskat)abstract
- To evaluate the diagnostic value of thrombopoietin (TPO, c-mpl ligand) measurements, and clarify the regulatory mechanisms of TPO in normal and in thrombocytopenic conditions, the plasma TPO concentration was determined in normal individuals (n = 20), umbilical cord blood (n = 40), chronic idiopathic thrombocytopenic purpura (ITP; n = 16), in severe aplastic anaemia (SAA; n = 3), chemotherapy-induced bone marrow hypoplasia (n = 10), myelodysplastic syndrome (MDS; n = 11), and sequentially during peripheral blood progenitor cell transplantation (n = 7). A commercially available ELISA and EDTA-plasma samples were used for the analysis. The plasma TPO concentration in the normals and umbilical cord blood were 52 +/- 12 pg/ml and 66 +/- 12 pg/ml, respectively. The corresponding values in patients with SAA and chemotherapy-induced bone marrow hypoplasia were 1514 +/- 336 pg/ml and 1950 +/- 1684 pg/ml, respectively, and the TPO concentration, measured sequentially after myeloablative chemotherapy and peripheral blood progenitor cell transplantation, was inversely related to the platelet count. In contrast, the plasma TPO recorded in patients with ITP (64 +/- 20 pg/ml) and MDS (68 +/- 23 pg/ml) were only slightly higher than normal levels. In conclusion, TPO levels were significantly elevated in patients in which bone marrow megakaryocytes and platelets in circulation were markedly reduced, whereas TPO levels were normal in ITP patients, and only slightly increased in the MDS patients. These latter patients displayed a preserved number of megakaryocytes in bone marrow biopsies. Our data support the suggestion that megakaryocyte mass affects the plasma TPO concentration. In thrombocytopenic patients a substantially increased plasma TPO implies deficient megakaryocyte numbers. However, TPO measurements do not distinguish between ITP and thrombocytopenia due to dysmegakaryopoiesis, as seen in MDS patients.
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| 6. |
- Jacobsson, Stefan, 1951, et al.
(författare)
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Flow cytometric analysis of megakaryocyte ploidy in chronic myeloproliferative disorders and reactive thrombocytosis.
- 1996
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Ingår i: European journal of haematology. - 0902-4441. ; 56:5, s. 287-92
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Tidskriftsartikel (refereegranskat)abstract
- Megakaryocyte (MK) ploidy patterns were analysed by flow cytometry in 29 newly diagnosed and previously untreated patients with chronic myeloproliferative disorders (MPD) and concomitant thrombocytosis, in 9 patients with reactive thrombocytosis (RT) and in 12 healthy individuals. Unfractionated bone marrow from routine aspirates was used. MKs were identified with a fluorescein labelled monoclonal antibody specific for glycoprotein IIIa (GPIIIa) and DNA was stained with propidium iodide. For the 12 healthy volunteers the mean modal ploidy number was 16 N; the 9 patients with RT displayed an identical MK ploidy pattern. The frequency of MKs with a ploidy > or = 32 N was 45% among the patients with essential thrombocythaemia (ET) compared to 32% among the healthy volunteers (p < 0.001). MKs with ploidy number > or = 64 N, comprising approximately 13% of the total number of MKs, was a characteristic finding in the patients with ET. Similar findings were present in 8 patients with polycythaemia vera (PV). In patients with PV 34% and 6% of the MKs displayed ploidies > or = 32 N and > or = 64 N, respectively. In contrast, a distinct shift towards lower ploidy number, with 63% of MKs < or = 8 N, was found among the 4 patients with chronic myeloid leukaemia (CML). The present results indicate that by using flow cytometric analysis of MK ploidy distribution in patients with thrombocytosis, those with a reactive cause are likely to be discriminated from patients with myeloproliferative thrombocytosis, i.e. PV and ET on one hand and CML on the other hand. The distinction between ET and PV, however, has to be made on other grounds.
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| 7. |
- Olsson, Bob, 1969, et al.
(författare)
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Increased number of B-cells in the red pulp of the spleen in ITP
- 2012
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Ingår i: Annals of Hematology. - : Springer Science and Business Media LLC. - 1432-0584 .- 0939-5555. ; 91:2, s. 271-277
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Tidskriftsartikel (refereegranskat)abstract
- Platelets are targeted by autoantibodies and destroyed in the reticuloendothelial system in the spleen, liver and bone marrow in patients with immune thrombocytopenia (ITP). Other mechanisms such as destruction by cytotoxic T-cells and defective production of platelets in the bone marrow also exist. Splenectomy normalizes the platelet count in 70% of ITP patients, however, precious little is known about the spleen in this disease. Our aim was therefore to investigate the splenic morphology and especially the number and localization of splenic leukocytes in patients with ITP and controls and to evaluate factors predicting outcome of splenectomy. Spleen sections from 29 ITP patients and 11 individuals splenectomized due to trauma were analyzed by immunohistochemistry. All except one of the ITP patients had a normalized platelet count 12months after splenectomy and the platelet count was inversely correlated with age. ITP patients had an increased number of B-cells in the red pulp. The number of white pulp B-cells and number of T-cells in both compartments was unchanged. In conclusion, B-cells are increased in the red pulp of the spleen and together with cytotoxic T-cells, helper T-cells and macrophages line the sinusoids enabling the immunological attack on platelets in ITP.
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| 8. |
- Olsson, Bob, 1969, et al.
(författare)
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Recruitment of T cells into bone marrow of ITP patients possibly due to elevated expression of VLA-4 and CX3CR1.
- 2008
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Ingår i: Blood. - : American Society of Hematology. - 1528-0020 .- 0006-4971. ; 112:4, s. 1078-84
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Tidskriftsartikel (refereegranskat)abstract
- In idiopathic thrombocytopenic purpura (ITP), platelets are destroyed in the spleen, liver, and bone marrow (BM) by autoantibodies and cytotoxic T cells. In a DNA microarray screen of peripheral blood T cells, we found that VLA-4, CX3CR1, and CXCR4, involved in T-cell homing, had increased expression in ITP patients compared with controls. However, we only found increased protein expression of VLA-4 on T cells from peripheral blood by flow cytometry. To address a possible recruitment of T cells into the organs involved in platelet destruction, we analyzed T cells in BM. In BM, T-cell surface expression of VLA-4 and CX3CR1 was increased in ITP patients compared with controls. Furthermore, the number of CD3(+) T cells in BM, but not in blood, was increased in ITP patients compared with controls. This finding was confirmed by immunohistochemistry of BM biopsies. The number of regulatory T cells (CD4(+)/CD25(bright)) was decreased in the BM of ITP patients, whereas Fas expression was increased. In conclusion, ITP is associated with accumulation and activation of T cells in the BM. Recruitment of T cells into the target organ (eg, BM) is plausible and may be facilitated through increased VLA-4 and CX3CR1 expression. These molecules might serve as new treatment targets in ITP.
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| 9. |
- Ridell, Börje, et al.
(författare)
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Dysplastic megakaryopoiesis with thrombocytopenia and chromosomal aberration.
- 1992
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Ingår i: American journal of clinical pathology. - 0002-9173. ; 98:2, s. 227-30
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Tidskriftsartikel (refereegranskat)abstract
- A case of isolated thrombocytopenia with decreased platelet production and abnormal megakaryopoiesis is described. In the bone marrow, a chromosomally aberrant clone, 45,XX,-11,-18,+der (11;18)(11q13;18p11), was found. These findings indicate a myelodysplastic nature of the abnormal thrombocytopoiesis. The described case demonstrates the value of a bone marrow examination including histopathology with immunologic techniques to evaluate the megakaryopoiesis in thrombocytopenia and the interest of cytogenetic studies not only in instances with overt hematologic malignancies or complete myelodysplastic syndromes, but also when morphologic abnormalities occur in a single cell line.
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| 10. |
- Ridell, Börje, et al.
(författare)
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Incidence of chronic myeloproliferative disorders in the city of Göteborg, Sweden 1983-1992.
- 2000
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Ingår i: European journal of haematology. - 0902-4441. ; 65:4, s. 267-71
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Tidskriftsartikel (refereegranskat)abstract
- An estimation of the incidence of polycythaemia vera (PV), essential thrombocythaemia (ET) and chronic idiopathic myelofibrosis (CIM) in the city of Göteborg, Sweden during the period 1983-1992 was made from a retrospective case analysis of patients registered as chronic myeloproliferative disorders (CMPD) at the Departments of Medicine and the Department of Pathology of the two major hospitals in the city. A total of 125 cases of PV, 56 males and 69 females were identified. The number of cases as well as the age-specific incidence increased with age. The over all annual gender-specific incidence was 2.69 cases per 10(5) male inhabitants and 3.12 cases per 10(5) female inhabitants. The incidence of PV in relation to the European Standard Population was 2.02 cases per 10(5) inhabitants and year. There were 72 cases, 20 males and 52 females, with ET. The age-specific incidence was in all ages higher for females than for males and increased with age. The annual gender-specific incidence was 0.96 per 10(5) male inhabitants and 2.35 per 10(5) female inhabitants. The incidence of ET in relation to the European Standard Population was 1.28 per 10(5) persons and year. There were 20 cases of CIM, 11 males and 9 females. The annual gender-specific incidence of CIM was 0.53/10(5) male inhabitants and 0.41/10(5) female inhabitants. The incidence of CIM in relation to the European Standard Population was 0.31 per 10(5) persons and year. Seven persons, 2 males and 5 females, had a CMPD that could not be included in any of the above-mentioned groups, but were registered as CMPD, unclassified.
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