23 results on '"Anemia, Refractory pathology"'
Search Results
2. Ghosal hematodiaphyseal dysplasia and response to corticosteroid therapy.
- Author
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Joy P, Yoganathan S, Korula S, Abraham SSC, Barney AM, Walter VM, Gibikote S, and Danda S
- Subjects
- Anemia, Refractory diagnostic imaging, Anemia, Refractory drug therapy, Anemia, Refractory pathology, Child, Female, Humans, Male, Osteochondrodysplasias diagnostic imaging, Osteochondrodysplasias drug therapy, Osteochondrodysplasias pathology, Exome Sequencing, Adrenal Cortex Hormones administration & dosage, Anemia, Refractory genetics, Genetic Predisposition to Disease, Osteochondrodysplasias genetics, Thromboxane-A Synthase genetics
- Published
- 2021
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3. Pseudo-Chédiak-Higashi granules and other unusual cytoplasmic inclusions in refractory anaemia with excess blasts-2.
- Author
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La Gioia A, Bombara M, Fiorini F, and Fiorini M
- Subjects
- Aged, Anemia, Refractory pathology, Humans, Male, Anemia, Refractory etiology, Chediak-Higashi Syndrome pathology, Cytoplasmic Granules metabolism, Inclusion Bodies metabolism
- Published
- 2017
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4. Refractory anemia with ring sideroblasts and RARS with thrombocytosis.
- Author
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Patnaik MM and Tefferi A
- Subjects
- Aspirin therapeutic use, Female, Humans, Iron Overload drug therapy, Iron Overload genetics, Iron Overload pathology, Janus Kinase 2 genetics, Lenalidomide, Male, Mutation, Myelodysplastic Syndromes drug therapy, Myelodysplastic Syndromes genetics, Myelodysplastic Syndromes pathology, Phosphoproteins genetics, Platelet Aggregation Inhibitors therapeutic use, RNA Splicing Factors, Receptors, Thrombopoietin genetics, Ribonucleoprotein, U2 Small Nuclear genetics, Risk Factors, Thalidomide analogs & derivatives, Thalidomide therapeutic use, Anemia, Refractory drug therapy, Anemia, Refractory genetics, Anemia, Refractory pathology, Anemia, Sideroblastic drug therapy, Anemia, Sideroblastic genetics, Anemia, Sideroblastic pathology, Angiogenesis Inhibitors therapeutic use, Thrombocytosis drug therapy, Thrombocytosis genetics, Thrombocytosis pathology
- Abstract
Disease Overview: Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (RARS-T)., Diagnosis: RARS is a lower risk myelodysplastic syndrome (MDS) with dysplasia limited to the erythroid lineage, <5% bone marrow (BM) blasts and ≥15% BM RS. RARS-T is a provisional entity in the MDS/MPN (myeloproliferative neoplasm) overlap syndromes, with diagnostic features of RARS, along with a platelet count ≥450 × 10(9)/L and large atypical megakaryocytes similar to those observed in BCR-ABL1 negative MPN. Mutations and Karyotype: Mutations in the SF3B1 gene are seen in ≥80% of patients with RARS and RARS-T, and strongly correlate with the presence of BM RS; RARS-T patients have additional mutations such as, JAK2V617F (∼60%), MPL (<5%), and CALR (<5%). Cytogenetic abnormalities are uncommon in both RARS and RARS-T., Risk Stratification: Most patients with RARS are stratified into lower risk groups by the International Prognostic Scoring System (IPSS) for MDS and the revised IPSS. Disease outcome in RARS-T is better than that of RARS, but worse than that of essential thrombocytosis. Both RARS and RARS-T have a low risk of leukemic transformation., Treatment: Anemia and iron overload are complications in both diseases and are managed similar to lower risk MDS. Aspirin therapy is reasonable in RARS-T, especially in the presence of JAK2V617F, but the value of platelet-lowering drugs is uncertain. Case reports of RARS-T therapy with lenalidomide warrant additional studies., (© 2015 Wiley Periodicals, Inc.)
- Published
- 2015
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5. Peroxiredoxin 2 expression is increased in neutrophils of patients with refractory cytopenia with multilineage dysplasia.
- Author
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Kazama H, Teramura M, Kurihara S, Yoshinaga K, Kato T, and Motoji T
- Subjects
- Adult, Aged, Aged, 80 and over, Anemia, Refractory genetics, Anemia, Refractory pathology, Cell Lineage, Female, Humans, Male, Middle Aged, Neutrophils pathology, Peroxiredoxins genetics, Prognosis, Proteomics, Anemia, Refractory blood, Neutrophils metabolism, Peroxiredoxins biosynthesis
- Abstract
Myelodysplastic syndromes (MDS) are heterogeneous clonal disorders characterized by cytopenias that arise due to ineffective haematopoiesis and morphological dysplasia and carry an increased risk of incident acute myeloid leukaemia. The pathogenesis of marrow dysfunction in MDS is multifactorial and consistent with a multistep model and may lead to heterogeneity of MDS. We investigated the proteome profile of circulating neutrophils purified from patients with refractory cytopenia with multilineage dysplasia (RCMD) to identify proteins that have a role in the pathogenesis. Using 2-dimensional difference gel electrophoresis and protein identification by matrix-assisted laser desorption ionization time-of-flight mass spectrometry, we found that peroxiredoxin 2 (PRDX2), a member of the peroxiredoxin family that regulates reactive oxygen species, was markedly upregulated in neutrophils of RCMD patients compared to healthy donors. Increased PRDX2 expression in the neutrophils of RCMD patients was confirmed using quantitative reverse transcription polymerase chain reaction, immunoblotting and immunocytochemical analysis. In addition, white blood cell and neutrophil counts in RCMD patients correlated inversely with the PRDX2 expression of. Oxidative stress is a known factor involved in the pathogenesis of MDS, and PRDX2 is associated with tumourigenesis of several solid tumours. Accordingly, our results suggest that PRDX2 may perform an important function in the pathogeneis of RCMD., (© 2014 John Wiley & Sons Ltd.)
- Published
- 2014
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6. Hemosiderin-containing plasma cells.
- Author
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Bain BJ
- Subjects
- Female, Humans, Middle Aged, Anemia, Refractory metabolism, Anemia, Refractory pathology, Bone Marrow Cells metabolism, Bone Marrow Cells pathology, Hemosiderin metabolism, Plasma Cells metabolism, Plasma Cells pathology
- Published
- 2012
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7. Clinical images: Gastric antral vascular ectasia in systemic sclerosis.
- Author
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Thonhofer R, Siegel C, Trummer M, and Gugl A
- Subjects
- Anemia, Refractory complications, Anemia, Refractory pathology, Anemia, Refractory therapy, Blood Transfusion, Female, Gastric Antral Vascular Ectasia complications, Gastric Antral Vascular Ectasia therapy, Gastrointestinal Hemorrhage etiology, Gastrointestinal Hemorrhage therapy, Humans, Laser Coagulation, Lasers, Gas therapeutic use, Middle Aged, Scleroderma, Limited complications, Scleroderma, Limited therapy, Treatment Outcome, Gastric Antral Vascular Ectasia diagnosis, Gastrointestinal Hemorrhage pathology, Scleroderma, Limited diagnosis
- Published
- 2010
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8. Monocytic leukemia cutis diagnosed simultaneously with refractory anemia with monocytosis: a case report.
- Author
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Yavorkovsky LL, Zain J, Wu CD, Trivelli L, and Cook P
- Subjects
- Aged, Anemia, Refractory complications, Anemia, Refractory pathology, Bone Marrow pathology, Fatal Outcome, Female, Humans, Immunophenotyping, Leukemia pathology, Leukemia, Monocytic, Acute complications, Leukemia, Monocytic, Acute etiology, Leukocytosis complications, Leukocytosis pathology, Myelodysplastic Syndromes diagnosis, Myelodysplastic Syndromes pathology, Sepsis etiology, Thrombocytopenia etiology, Anemia, Refractory diagnosis, Leukemia diagnosis, Leukocytosis diagnosis, Monocytes pathology
- Abstract
A case of leukemia cutis (LC) of monocytic lineage in a patient with myelodysplastic syndrome (MDS) is presented. Cutaneous infiltrates were recognized concurrent with diagnosis of refractory anemia (RA) with monocytosis. Skin infiltrates subsequently spontaneously regressed although MDS progressed with increasing monocytosis, anemia, and thrombocytopenia. Death occurred 6 months after diagnosis with evolution of acute monoblastic leukemia complicated by sepsis. This case supports previous observations of poor prognosis associated with leukemia cutis. LC associated with MDS is reviewed including the role of monocytes.
- Published
- 2001
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9. Cyclosporin A therapy in hypoplastic MDS patients and certain refractory anaemias without hypoplastic bone marrow.
- Author
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Jonásova A, Neuwirtová R, Cermák J, Vozobulová V, Mociková K, Sisková M, and Hochová I
- Subjects
- Adult, Aged, Aged, 80 and over, Anemia, Refractory pathology, Blood Cell Count, Bone Marrow pathology, Cyclosporine adverse effects, Female, Humans, Male, Middle Aged, Treatment Outcome, Anemia, Refractory drug therapy, Cyclosporine therapeutic use, Immunosuppressive Agents therapeutic use
- Abstract
We report 17 cytopenic patients with myelodysplastic syndrome (MDS) of refractory anaemia (RA) subtype with hyper-, normo- or hypo-cellular bone marrow (BM), who were treated with cyclosporin A (CyA). Substantial haematological response was observed in 14 patients (82%): their anaemia improved and all transfusion-dependent patients achieved transfusion independence. Complete trilineage recovery was observed in four patients (23%). The CyA therapy has not yet failed in any of the 14 successfully treated patients during follow-up times ranging from 5 to 30 months. CyA was well tolerated in 14 patients; serious side-effects required termination of the therapy in three patients in whom the blood count rapidly deteriorated to former levels upon cessation of therapy. Two patients benefited from a combination therapy of CyA and erythropoietin. Six patients experienced various autoimmune phenomena. CyA could thus offer an alternative treatment for certain MDS patients with RA regardless of hyper-, normo- or hypo-cellularity of bone marrow (BM). The mechanism of the beneficial effect of CyA is discussed and remains the subject of an ongoing study.
- Published
- 1998
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10. Amount of mpl on bone marrow haemopoietic precursor cells from healthy volunteers and patients with refractory anaemia.
- Author
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Takeshita A, Shinjo K, Naito K, Nakamura S, Izumi M, Ling P, Ohnishi K, and Ohno R
- Subjects
- ADP-ribosyl Cyclase, ADP-ribosyl Cyclase 1, Acute Disease, Anemia, Refractory pathology, Antigens, CD34, Antigens, Differentiation, Bone Marrow Cells metabolism, Bone Marrow Cells pathology, Cell Differentiation, Cells, Cultured, Flow Cytometry, Humans, Leukemia, Myeloid metabolism, Leukemia, Myeloid pathology, Membrane Glycoproteins, NAD+ Nucleosidase, Anemia, Refractory metabolism, Antigens, CD, Hematopoietic Stem Cells metabolism, Thrombopoietin metabolism
- Abstract
Using a non-isotopic ligand binding assay using multi-colour flow cytometry, we quantitatively examined the amount of mpl in megakaryocyte-platelet lineage cells. Firstly, we quantified the amount of mpl on cell lines. Mpl gene-transfected BaF3 cells expressed a large amount of mpl, whereas original BaF3, K562, HL-60 and NOMO-1 cells showed no mpl. In bone marrow cells from healthy volunteers, mpl was expressed on CD34+ cells from the very early stage of differentiation when they had no CD38 antigen. The amount of mpl increased with differentiation to CD34+ CD41+ cells, but decreased with further differentiation to CD34- CD41+ cells. In CD34+ CD41+ cells the amount of mpl varied according to cell size: abundant in large cells, moderate in medium-size cells and a little in small cells. In bone marrow cells from patients with refractory anaemia (RA), the amount of mpl was decreased compared with that in bone marrow cells from healthy volunteers. When analysed by the same CD phenotype and same cell size, the amount of mpl was less in RA patients compared with that in healthy volunteers in all phenotypes and sizes tested. The proportion of large CD34+ CD41+ cells was less in RA patients than in normal volunteers.
- Published
- 1997
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11. Refractory cytopenia with t(1;7),+8 abnormality and dysplastic eosinophils showing intranuclear Charcot-Leyden crystals: a fluorescence in situ hybridization study.
- Author
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Ma SK, Wong KF, Chan JK, and Kwong YL
- Subjects
- Adult, Anemia, Refractory genetics, Bone Marrow pathology, Chromosomes, Human, Pair 1, Chromosomes, Human, Pair 7, Chromosomes, Human, Pair 8, Female, Humans, In Situ Hybridization, Fluorescence, Lysophospholipase, Translocation, Genetic, Trisomy, Anemia, Refractory pathology, Chromosome Aberrations, Eosinophilia pathology, Eosinophils pathology, Glycoproteins analysis
- Abstract
A case of refractory cytopenia and marrow eosinophilia showing t(1;7) translocation and concomitant trisomy 8 is reported. The eosinophils were dysplastic, and showed the unique feature of intranuclear Charcot-Leyden crystal formation, giving rise to a 'lip-like' appearance. We speculate that this unusual cytologic feature resulted from abnormal precipitation of Charcot-Leyden crystal protein in the eosinophils. By fluorescence in situ hybridization using a chromosome 8 specific alpha-satellite probe, the abnormal eosinophils were shown to have derived from the abnormal clone. We postulate that the dysplastic clone might have retained a differentiation potential and be responsive to normal haemopoietic stimuli.
- Published
- 1995
- Full Text
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12. Transformation into acute basophilic leukaemia in a patient with myelodysplastic syndrome.
- Author
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Yamagata T, Miwa A, Eguchi M, Kitagawa S, Muroi K, Hatake K, Suda T, Sakamoto S, and Miura Y
- Subjects
- Anemia, Refractory genetics, Basophils pathology, Cell Differentiation, Chromosome Deletion, Chromosomes, Human, Pair 5, Female, Humans, Leukemia, Basophilic, Acute genetics, Middle Aged, Anemia, Refractory pathology, Bone Marrow pathology, Cell Transformation, Neoplastic, Leukemia, Basophilic, Acute pathology
- Abstract
We describe a patient with basophilic leukaemia following a 2-year period with myelodysplastic syndrome (refractory anaemia). The marrow showed 59.4% of blasts with 25.0% of mature and immature basophils. The leukaemic blasts contained granules, positively stained with toluidine blue but negative for peroxidase. The basophilic differentiation was confirmed by ultrastructural analysis demonstrating immature basophil granules. In addition, a morphological transition from immature blasts to more mature basophils was observed. Immunophenotypic analysis of blasts and basophils showed positive for CD5, CD7, CD13, CD33 and CD34. Cytogenetic investigation showed an abnormal karyotype, 46,XY,del(5)(q31q35), in 11% of the cells examined when the initial diagnosis of refractory anaemia was made. However, expansion of the same clone up to 100% was observed concomitantly with transformation to basophilic leukaemia.
- Published
- 1995
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13. Refractory anaemia with preleukaemic polyclonal haemopoiesis and the emergence of monoclonal erythropoiesis on disease progression.
- Author
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Culligan DJ, Bowen DT, May A, White D, Padua RA, and Burnett AK
- Subjects
- Adult, Anemia, Refractory blood, Anemia, Refractory pathology, Bone Marrow pathology, Chromosome Aberrations, Disease Progression, Dosage Compensation, Genetic, Erythroblasts, Erythropoiesis genetics, Female, Humans, Anemia, Refractory genetics, Hematopoiesis genetics, Hematopoietic Stem Cells pathology, Preleukemia genetics
- Abstract
We describe a young woman with a myelodysplastic syndrome (MDS) of the type refractory anaemia (RA) which remained stable for 11 years and then underwent rapid progression manifested by bone marrow failure with the emergence of a complex clonal cytogenetic abnormality. Peripheral blood granulocytes, mononuclear cells and bone marrow erythroblasts were all polyclonal by X-inactivation analysis detected by the probe M27B during the preleukaemic phase. On disease progression, bone marrow erythroblasts developed an extremely skewed monoclonal pattern of X-inactivation. In some cases of MDS, therefore, polyclonal haemopoiesis can be detected for a considerable time during the preleukaemic phase and we report the demonstration of bone marrow erythroblasts changing from a polyclonal to a monoclonal pattern on disease progression.
- Published
- 1995
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14. Myelodysplastic syndrome in two young brothers.
- Author
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Hirose M, Kawahito M, and Kuroda Y
- Subjects
- Anemia, Refractory pathology, Child, Chromosomes, Human, Pair 1, Chromosomes, Human, Pair 7, Humans, Infectious Disease Transmission, Vertical, Leukemia, Radiation-Induced genetics, Male, Translocation, Genetic, Anemia, Refractory genetics, Bone Marrow pathology, Environmental Exposure, Radiation Injuries genetics, Radioactive Fallout adverse effects
- Abstract
We report the youngest cases of myelodysplastic syndrome (MDS) in two brothers aged 7 and 2 years. The maternal grandfather and maternal grandmother had been exposed to radioactive fallout after the atomic bomb attack on Hiroshima in 1945. The elder brother demonstrated pancytopenia with < 1% blast cells in his peripheral blood and < 5% in his bone marrow at diagnosis. The younger brother was thrombocytopenic without increased blasts. The karyotype of bone marrow cells from the elder brother was 46,XY, -7, +der (7), t(1:7) (lqter-lq11::7q11-7pter), but the younger brother's karyotype was normal. Immature myeloid cells in the bone marrow from both brothers were morphologically abnormal. A diagnosis of refractory anaemia (RA) was made in both brothers. Atavism due to radioactive poisoning was suspected in the development of MDS in these two cases.
- Published
- 1995
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15. Abnormal erythrocyte band 4.1 protein in myelodysplastic syndrome with elliptocytosis.
- Author
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Ideguchi H, Yamada Y, Kondo S, Tamura K, Makino S, and Hamasaki N
- Subjects
- Anemia, Refractory pathology, Base Sequence, Bone Marrow pathology, DNA chemistry, Electrophoresis, Polyacrylamide Gel, Glycophorins analysis, Humans, Immunoblotting, Male, Middle Aged, Molecular Sequence Data, Polymerase Chain Reaction, Spectrin analysis, Anemia, Refractory blood, Cytoskeletal Proteins, Erythrocyte Membrane chemistry, Erythrocytes, Abnormal physiology, Membrane Proteins blood, Neuropeptides
- Abstract
A case of myelodysplastic syndrome with haemolytic anaemia and a marked elliptocytosis is reported. Sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) of erythrocyte membrane proteins revealed that the patient's band 4.1 was decreased to about 50-70% of that of control and contained abnormal molecule migrating in a faster mobility than normal band 4.1, which was confirmed by immunoblotting. The actin/spectrin ratio of the patient's ghosts diminished to about 70% of that of control ghosts. Flowcytometric analysis showed that the glycophorin C content of the patient's erythrocytes was reduced but maintained the level of about 70% of that of normal, indicating that the glycophorin C-band 4.1 interaction might not be so seriously damaged as to cause elliptocytic shape change. We postulate that the abnormal band 4.1 produced from the abnormal erythroid clone may be the primary molecular defect and result in a dysregulation of spectrin-actin interaction to cause erythrocyte shape change and membrane instability.
- Published
- 1993
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16. A trial of recombinant human interleukin-1 in patients with severe refractory aplastic anaemia.
- Author
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Walsh CE, Liu JM, Anderson SM, Rossio JL, Nienhuis AW, and Young NS
- Subjects
- Adolescent, Adult, Anemia, Aplastic blood, Anemia, Aplastic pathology, Anemia, Refractory blood, Anemia, Refractory pathology, Bone Marrow pathology, Drug Evaluation, Female, Humans, Lymphocyte Subsets pathology, Male, Anemia, Aplastic drug therapy, Anemia, Refractory drug therapy, Interleukin-1 therapeutic use, Recombinant Proteins therapeutic use
- Abstract
We report here the effects of in vivo administration of recombinant interleukin-1 alpha (rIL-1 alpha) to patients with severe, idiopathic aplastic anaemia. Four patients who were refractory to immunosuppressive therapy and were not bone marrow transplantation candidates received daily doses of 0.03 microgram/kg and 0.10 microgram/kg intravenously as 5 d courses. No significant changes in either peripheral blood counts or bone marrow cellularity were observed at either dose during or following therapy. Two patients showed increased numbers of bone marrow progenitor colonies. Lymphocyte phenotyping demonstrated an elevated percentage of CD8+/DR+ activated suppressor T lymphocytes prior to therapy. After rIL-1 alpha administration, the percentage of CD8+/DR+ cells was reduced or returned to normal in all patients. Significant side-effects included fever, rigours, fatigue, headache and nausea. Transient hypotension was observed at both doses in all patients. These results suggest that while rIL-1 alpha can be safely administered, no significant haematologic improvement was observed in patients with severe aplastic anaemia.
- Published
- 1992
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17. Recurrent oral condylomata acuminata and hairy leukoplakia: an early sign of myelodysplastic syndrome in an HIV-seronegative patient.
- Author
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Ficarra G, Miliani A, Adler-Storthz K, Woods-Francis K, Del Mistro A, de Rossi A, Riccardi R, Ravina A, and De Maio E
- Subjects
- Anemia, Refractory pathology, Humans, Male, Middle Aged, Recurrence, Condylomata Acuminata pathology, HIV Seropositivity, Herpesvirus 4, Human, Leukoplakia, Oral pathology, Lip Neoplasms pathology, Myelodysplastic Syndromes pathology, Tongue Neoplasms pathology, Tumor Virus Infections pathology
- Abstract
Oral hairy leukoplakia (OHL) has been observed in all risk groups seropositive for HIV infection. Recently, this lesion has also been described in HIV-seronegative patients with immunosuppression of iatrogenic origin. We report on a HIV-1 and HIV-2 seronegative, heterosexual man affected by refractory anemia with ringed sideroblasts (myelodysplastic syndrome), who developed recurrent oral condylomata acuminata and OHL as an early clinical manifestation. The diagnosis of OHL was confirmed by identifying Epstein-Barr viral particles by electron microscopy and by in situ DNA hybridization. HIV infection was ruled out using polymerase chain reaction and testing for HIV-1 and HIV-2 antibodies.
- Published
- 1991
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18. Myelodysplastic transformation of polycythemia vera: case report and review of the literature.
- Author
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Shamdas GJ, Spier CM, and List AF
- Subjects
- Aged, Anemia, Refractory etiology, Anemia, Refractory genetics, Anemia, Refractory pathology, Humans, Immunophenotyping, Karyotyping, Male, Myelodysplastic Syndromes genetics, Myelodysplastic Syndromes pathology, Polycythemia Vera genetics, Polycythemia Vera pathology, Myelodysplastic Syndromes etiology, Polycythemia Vera complications
- Abstract
We report a case of refractory anemia with excess blasts (RAEB) developing in a 67-year old man with a history of polycythemia vera; results of cytogenetic and immunophenotyping studies are described. In this report the clinical, cytogenetic and hematologic features of myelodysplasia complicating polycythemia vera are reviewed. Results of immunophenotyping and cytogenetic studies, and the preponderance of cases developing after myelosuppressive therapy suggest that in the majority of cases myelodysplasia is treatment-related.
- Published
- 1991
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19. Are 'dysplastic' and hypogranular megakaryocytes specific markers for myelodysplastic syndrome?
- Author
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Wong KF and Chan JK
- Subjects
- Acute Disease, Adolescent, Adult, Aged, Aged, 80 and over, Anemia, Refractory, with Excess of Blasts pathology, Child, Child, Preschool, Cytoplasmic Granules pathology, Female, Humans, Leukemia, Myeloid pathology, Male, Middle Aged, Anemia, Refractory pathology, Bone Marrow pathology, Leukemia, Myelomonocytic, Chronic pathology, Megakaryocytes pathology
- Abstract
Dysmegakaryocytopoiesis is an integral component of myelodysplastic syndrome (MDS), and has been shown in some studies to be an independent prognostic factor. Megakaryocytic hypogranulation, a feature we have noticed for some time to be fairly common in MDS and acute myeloid leukaemia (AML), has received little attention in the literature as a dysplastic feature of megakaryocytes. This study was performed to determine how frequently this feature was observed in MDS and the specificity of its occurrence. On review of archival materials, hypogranular megakaryocytes were observed in 80.3% of MDS, 30.6% of AML and 1.4% of controls. On the other hand, the other well-recognized dysmegakaryocytopoietic features (hypolobulation, multiple separate nuclei, micromegakaryocyte), though frequent in MDS or AML, were also observed in 20% of controls. We therefore propose including megakaryocytic hypogranulation as a cytological feature of myelodysplasia.
- Published
- 1991
- Full Text
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20. Responsiveness of bone marrow erythropoietic stem cells (CFU-E and BFU-E) to recombinant human erythropoietin (rh-Ep) in vitro in aplastic anemia and myelodysplastic syndrome.
- Author
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Aoki I, Higashi K, Homori M, Chikazawa H, and Ishikawa K
- Subjects
- Anemia, Refractory pathology, Anemia, Refractory, with Excess of Blasts pathology, Anemia, Sideroblastic pathology, Cells, Cultured, Dose-Response Relationship, Drug, Humans, Recombinant Proteins, Reference Values, Anemia, Aplastic pathology, Bone Marrow pathology, Erythroid Precursor Cells drug effects, Erythropoietin pharmacology, Myelodysplastic Syndromes pathology
- Abstract
Responsiveness of bone marrow erythropoietic stem cells (CFU-E and BFU-E) to recombinant human erythropoietin (rh-Ep) was examined in vitro in 23 patients with aplastic anemia and 14 with myelodysplastic syndrome (MDS) to investigate the clinical use of rh-Ep for these diseases. Bone marrow mononuclear cells were cultured by methylcellulose methods for CFU-E and BFU-E assays. In normals, the CFU-E numbers reached a plateau of increase at Ep doses of almost 2-5 units, and no further increase was observed with the addition of larger Ep doses. In aplastic anemia, the responses of CFU-E to Ep were relatively good in nonsevere type and generally poor in severe type. However, the CFU-E numbers increased with increasing doses of Ep in some of the patients with aplastic anemia. Among the patients with MDS, the responses of CFU-E to Ep were relatively good in primary acquired refractory anemia (PARA) and primary acquired sideroblastic anemia. On the other hand, the responses of CFU-E to Ep were poor in refractory anemia with an excess of blasts (RAEB) and RAEB in transformation among the MDS patients. BFU-E responses to Ep were poor in severe aplastic anemia, RAEB, and RAEB-T. However, there are Ep responsive patients in some of aplastic anemia and PARA. High titers of rh-Ep were suggested to be effective clinically in some patients with aplastic anemia and those with PARA.
- Published
- 1990
- Full Text
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21. Evolution of acquired severe aplastic anaemia to myelodysplasia and subsequent leukaemia in adults.
- Author
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de Planque MM, Kluin-Nelemans HC, van Krieken HJ, Kluin PM, Brand A, Beverstock GC, Willemze R, and van Rood JJ
- Subjects
- Adult, Aged, Anemia, Aplastic pathology, Anemia, Refractory pathology, Bone Marrow pathology, Female, Humans, Karyotyping, Leukemia, Myeloid genetics, Leukemia, Myeloid pathology, Male, Middle Aged, Anemia, Aplastic complications, Anemia, Refractory etiology, Leukemia, Myeloid etiology
- Abstract
Myelodysplasia (MDS) and leukaemia following acquired aplastic anaemia has been reported as a rare event occurring in about 5% of patients. Improved results in survival of patients with severe aplastic anaemia (SAA) and subsequent prolonged follow-up created the possibility of evaluating the occurrence of MDS and leukaemia in 38 adult patients with acquired SAA surviving two or more years without bone marrow transplantation. Five patients, age 22, 35, 47, 56, 72 years, two females, three males, all with idiopathic SAA and normal cytogenetic analysis developed a refractory anaemia (RA) 7, 30, 48, 56, 142 months after diagnosis of SAA. In 3/5 RA evolved into an acute myeloid leukaemia (AML) either via a chronic myelomonocytic leukaemia (CMML) (2/3) or via RA with excess of blasts (RAEB) (1/3). Three patients revealed a monosomy 7 during MDS and/or leukaemic phase. One patient died during RA phase without cytogenetic abnormalities. A pattern of evolution could be identified in these patients revealing well-documented SAA - improvement of bone marrow haematopoiesis - dyshaematopoietic features of one or more cell lines with predominance of dyserythropoiesis - RA - RAEB or CMML - AML. These five patients represent more than 10% of all patients surviving at least 2 years. This implies that the risk of developing MDS and leukaemia in SAA patients surviving with autologous marrow, might increase with longer follow-up.
- Published
- 1988
- Full Text
- View/download PDF
22. Megakaryocyte colony formation by bone marrow progenitors in myelodysplastic syndromes.
- Author
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Juvonen E, Partanen S, Knuutila S, and Ruutu T
- Subjects
- Aged, Anemia, Refractory, with Excess of Blasts pathology, Cells, Cultured, Erythrocytes pathology, Female, Granulocytes pathology, Humans, Macrophages pathology, Male, Middle Aged, Anemia, Refractory pathology, Bone Marrow pathology, Hematopoietic Stem Cells pathology, Megakaryocytes pathology
- Abstract
Megakaryocytic colony formation by precursor cells from the bone marrow was investigated in 10 patients with a myelodysplastic syndrome. All but one exhibited abnormal colony formation: four showed no colony formation at all, while a decreased number of colonies was noticed in five. All of the patients showed defective colony formation by erythroid progenitors, but only four showed clearly abnormal granulocyte-macrophage colony formation. The defect in megakaryocytic progenitors seems to be more akin to the defects occurring in erythroid progenitors than to the defects seen in the granulocyte-macrophage lineage.
- Published
- 1986
- Full Text
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23. Natural killer cell-mediated inhibition of bone marrow colony formation (CFU-GM) in refractory anaemia (preleukaemia): evidence for patient-specific cell populations.
- Author
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Kerndrup G and Hokland P
- Subjects
- Anemia, Refractory pathology, Colony-Forming Units Assay, Granulocytes pathology, Humans, Kinetics, Leukocytes, Mononuclear immunology, Macrophages pathology, Anemia, Refractory immunology, Bone Marrow pathology, Killer Cells, Natural immunology
- Abstract
The role of peripheral blood mononuclear cells (PB-MNC) on the growth of bone marrow (BM) CFU-GM was investigated in refractory anaemia (RA) patients. Whereas normal donor PB-MNC were found to inhibit autologous day 7 CFU-GM, PB-MNC from RA patients exhibited little modulatory effect on autologous or allogeneic day 7 CFU-GM. In contrast, patient PB-MNC inhibited autologous CFU-GM at day 10 at a time where no significant inhibition was seen in the PB-MNC/RA CFU-GM combination. The identity of the inhibitory cells was investigated using anti-T8+ and anti-N901+ subsets purified by immune-rosette depletion with a panel of monoclonal antibodies. The activity of these subsets was tested on immature myeloid cells enriched for MY7+ cells, and it was found that cells highly enriched for NK cells were responsible for the inhibition. Further support for NK cells as the inhibitory cells was obtained in experiments where a positive correlation between the level of PB NK cytotoxicity against K562 cells and the degree of CFU-GM inhibition was demonstrated. Thus, these data suggest the presence of a specialized subset of NK cells with a capacity to inhibit autologous CFU-GM. Since RA is a potentially premalignant disease, in which a significant number of cases transform into AML, these findings also suggest a physiological role for NK cells in suppression of newly arisen clonogenic cells at least in early stages of the disease.
- Published
- 1988
- Full Text
- View/download PDF
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