Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of 14 patients who underwent allogeneic or syngeneic bone marrow transplantation, 6 had a transient appearance of small blastoid cells in the bone marrow after transplantation. Most of these patients (11) had leukemia, although 3 had severe aplastic anemia. The cells were 8-18 micron in diameter and had scant cytoplasm and dense nuclei with smooth, homogeneous chromatin. They often had distinct nuclear clefts. These cells constituted 4.0-21.3% of the total number of bone marrow cells. They were not reactive with peroxidase, alpha-naphtyl butylate esterase, naphthol AS-D chloroacetate esterase, or periodic acid-Schiff stains. Immunocytochemical analysis revealed that the small blastoid cells expressed terminal deoxynucleotidyl transferase, Ia-like, CD19, and CD10 antigens and cytoplasmic mu heavy chains, indicating a precursor B-cell phenotype. CD20 antigen was not expressed on these cells. The data suggest that cytoplasmic mu may be expressed earlier than CD20 antigen in the differentiation of B-cell lineage. The morphologic, cytochemical, and immunophenotypic characteristics did not distinguish these nonneoplastic cells distinctly from leukemic lymphoblastic cells. The increase of small blastoid cells was a transient and self-limited phenomenon, in contrast to that of neoplastic blasts. These cells should be recognized as a common component of the bone marrow of marrow transplant recipients. The significance and role of these cells in immune recovery and hematopoiesis remain uncertain.
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PMID:The transient appearance of small blastoid cells in the marrow after bone marrow transplantation. 161 19

This is a review of preleukaemic states in children. In a prospective series of 109 children with AML the overt disease was preceded by MDS in 22 cases. Ten of these patients had Down's syndrome. Advanced FAB groups were represented in the series. An important subgroup is the bone marrow monosomy 7 syndrome. Cytogenetic anomalies are common in MDS, and multiple and complicated abnormalities develop in nearly all patients with progressing disease. Some children die before transformation to overt ANLL. Transformation usually occurs, few children survive. With cytostatic treatment the risk of irreversible aplasia is great. The choice of schedule should therefore be carefully considered. Bone marrow transplantation has proved beneficial in a number of cases, but these are still quite few. The dysfunction of the bone marrow preceding ALL is due to transient aplastic anaemia--spontaneous remission--overt ALL, often FAB type L1, immunophenotype CALLA. The ALL reacts to the same treatment as de novo ALL of the same type and the prognosis is the same.
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PMID:Bone marrow dysfunctions preceding acute leukemia in children: a clinical study. 173 77

We have compared CD10 antigen expression in normal fetal bone marrow with that of B-linage acute lymphoblastic leukemia (ALL). Both quantitative indirect immunofluoresence (QIFI) and direct immunofluorescence (IF) tests with Quantum beads were used to convert median fluorescence intensity (MFI) values into numbers of antigen molecules expressed per cell (AgE). Lymphoid precursors in the fetal marrow and liver expressed 3-12.5 x 10(3) CD10 molecules/cell with an upper limit of 5 x 10(4)/cell (MaxAgE). The median CD10 AgE in the different cases of acute B-lineage ALL were variable and ranged from undetectable to very high values (> 1.8 x 10(5). In 24 of the 72 cases (33%) tested with QIFI the median CD10 AgE was above the highest values seen in normal samples (> 5 x 10(4)/cell). An additional 23.6% of cases had higher median values than the normal median CD10 AgE. Next, CD10 antigen was quantitated in 78 cases during the routine multiparameter analysis of B-lineage leukemia using CD10/class II/CD34 3-color IF test or CD10/TdT 2-color IF test. The aberrant overexpression was confirmed in 43.6% of ALL cases. The CD10bright display suggested ALL diagnosis even when few cells were available for study, e.g., in early relapse and in ALL masquerading as aplastic anemia. The levels of CD10 expression were maintained in relapse. In addition, different CD10 levels were associated with the various chromosomal alterations: high CD10 levels (> 3 x 10(4)/cell) with hyperdiploidy, low CD10 levels (1.8-4 x 10(3)/cell) with the t(1;19) and undetectable levels (< 1.2 x 10(3)/cell) with the t(4;11) translocations.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Leukemia-associated changes identified by quantitative flow cytometry: I. CD10 expression. 789 27

Cyclosporin A (CsA) is used to prevent rejection in transplantation and to treat autoimmune and hematologic diseases such as aplastic anemia. However, the tumor growth-promoting effect of CsA remains controversial. We report the case of a 24-year-old man who developed acute lymphoblastic leukemia of precursor-T-cell origin after 75 months of treatment with CsA for aplastic anemia. The surface antigen phenotype of his leukemic cells was CD2+, CD3+, CD5+, CD7+, CD4-, CD8-, CD10-, CD20-, CD34-, CD41-, and CD56-. Southern blot analysis revealed a monoclonal rearrangement of T-cell receptor-Jgamma nongermline fragments in HindIII digestion.
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PMID:T-Cell type acute lymphoblastic leukemia following cyclosporin A therapy for aplastic anemia. 1137 36

A 91-year-old woman was hospitalized with acute respiratory distress syndrome due to pneumonia in June 1997. Since she had pancytopenia and a bone marrow aspirate indicated hypocellularity with no increase in myeloblasts, dysplasia or abnormal chromosomes, aplastic anemia (AA) was diagnosed. Pulse therapy with methylprednisolone and antibiotics proved successful, and blood cell numbers stabilized. In June 2001, she was readmitted to our hospital with persistent low grade fever and leukopenia. A bone marrow aspirate from the sternum and iliac bone biopsy revealed compact proliferation of small lymphocytes, and the surface marker CD5- CD10- CD11c+ CD19+ CD20+ CD23- was detected through immune staining and flowcytometry. CD30+, CD34+and CD56+cells were scarce. Tests for surface immunoglobulins, IgG, IgA, IgM and IgD, were negative. No nodal or extranodal lesions were evident. Since Southern blot analysis of bone marrow cells indicated rearrangement of the immunoglobulin heavy chain and abnormal chromosomes were evident, small lymphocytic lymphoma (SLL) was diagnosed. Four intravenous infusions of rituximab (375mg/m2) were administered without critical adverse effects. Tests conducted four weeks later revealed saturation of CD20+ antigens of lymphoma cells and chromosomal abnormalities and rearrangement of the immunoglobulin heavy chain were still apparent. Though complete remission of the pancytopenia was not achieved, serum concentrations of lactate dehydrogenase and soluble interleukin-2 receptor decreased, and the numbers of platelets and erythrocytes increased. There was also an improvement in systemic condition. This was a rare case of SLL having the surface marker of CD5- CD10- CD11c+ CD19+ CD20+ CD23-, which had evolved from AA and infiltrated bone marrow.
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PMID:[CD5- CD11c+ CD23- small lymphocytic lymphoma evolving from aplastic anemia]. 1538 87

This study was purposed to explore the diagnostic role of flow cytometry in immunorelated pancytopenia (IRP). After 50 IRP patients were hospitalized, the concentration of serum ferritin, folic acid and vitamin B(12), immunologic test, platelet antibody, test of hepatitis A, B and C, haemolysis test and bone marrow smear examination were carried out, meanwhile the chromosome karyotype analysis and some routine examinations were performed. The 50 patients were divided into group A and group B. Group A consisted of 22 patients who were undefinedly diagnosed and intended to diagnosed as IRP, group B consisted of 28 definedly diagnosed patients with hematologic malignancies, including 7 cases of aplastic anemia, 2 of paroxysmal nocturnal hemoglobinuria, 10 of myelodysplastic syndrome, 9 of megaloblastic anemia. In addition, 30 normal people were used as normal control group (group C). For groups A and B, the binding autoantibodies of bone marrow stem/progenitor cells, erythroblasts and myelocytes were detected by flow cytometry, meantime the ratio of total B-(CD10(+)) and CD5(+) B-lymphocytes in peripheral blood was assayed. For control group, the ratios of CD19(+) and CD5(+) B lymphocytes in peripheral blood were determined alone. The results indicated that the detection of bone marrow autoantibodies in 20 patients of group A showed positive with 90.90%. The IgG type was found mostly in antibody binding types, next the IgM type, the IgA type was fewer. The detection of bone marrow autoantibodies of 2 patients in group B showed positive with 7.14%. The positive rate in group A was obviously higher than that in group B (p < 0.01). The ratios of CD19(+) and CD5(+) B lymphocyte in peripheral blood were significant higher in group A than that in group B and control group (p < 0.01), but there was no significant difference between groups B and control. It is concluded that the application of flow cytometry in detecting the autoantibodies of bone marrow cells and CD19(+) B-and CD5(+) B-lymphocyte in peripheral blood can provide reliable diagnostic evidence and detection measure for diagnosis and differential diagnosis of IRP, as well as may contribute to draw up more effective therapeutic strategy.
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PMID:[Clinical significance of flow cytometry in diagnosis of immunorelated pancytopenia]. 1937 90

It is often difficult to distinguish myelodysplastic syndrome (MDS) from aplastic anemia (AA) because of the considerable clinical, cytologic histologic similarities between these two disorders; however, distinguishing between AA and MDS is of great importance because there is a higher risk of progression to acute leukemia in patients with MDS compared with AA. Up to now, CD34(+) cells in MDS and AA patients have been studied extensively; however, little information is available on myeloid granulocytes. The aim of this study was to determine whether immunophenotype of myeloid granulocytes in AA patients was different from that of MDS. Flow cytometry was used to assess the immunophenotype of myeloid granulocytes in 22 patients with MDS, 12 with AA, and 10 normal subjects. Our data showed that the percentages of CD13(+) granulocytes, CD33(+) granulocytes, CD34(+) granulocytes, and HLA-DR(+) granulocytes were significantly higher in patients with MDS than in AA patients and normal subjects (P < 0.05). The percentages of CD15(+) granulocytes and CD10(+) granulocytes were significantly lower in patients with MDS than in AA patients and normal subjects (P < 0.05). There were no significant differences in the expression of these markers between patients with AA and normal subjects (P > 0.05). As refractory anemia progressing to refractory anemia with excess blasts, the percentages of CD13(+) granulocytes, CD33(+) granulocytes, CD34(+) granulocytes and HLA-DR(+) granulocytes were significantly increased, whereas, the percentage of CD15(+) granulocytes was significantly decreased (P < 0.05). These data suggest that immunophenotype of myeloid granulocytes may be a useful parameter for the differential diagnosis of MDS and AA.
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PMID:Immunophenotype of myeloid granulocytes: a pilot study for distinguishing myelodysplastic syndrome and aplastic anemia by flow cytometry. 1996 21