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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A one-year-old boy diagnosed as refractory anemia with excess of blasts in transformation is reported. Hematological examination revealed anemia, thrombocytopenia and the presence of blasts in both peripheral blood (3.5%) and bone marrow (20.1%) specimens. Chromosomal analysis showed abnormal karyotype; 48, XY, +21, +marker, r (7). Analyses with cytochemical stainings, electronmicroscopy and monoclonal antibodies to cell surface markers could not define the lineage of blasts. Induction chemotherapy was started with VP-16 (230 mg/m2 x 5 days) as a single agent and complete remission was achieved. Thereafter, he had been treated for 11 months with the intensive chemotherapy which consisted of VP-16, cytosine arabinoside, daunorubicin, vincristine, vinblastine, 6-mercaptopurine, prednisolone, mitoxantrone and CNS prophylaxis. He has been in complete remission for 18 months. The usefulness of VP-16 to MDS in pediatric patients is documented.
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PMID:[Successful induction chemotherapy for childhood RAEB-T with VP-16: a case report]. 281 Jul 90

Dysplastic features of peripheral blood granulocytes were studied to investigate the diagnostic value estimating cytoplasmatic hypogranulation and nuclear abnormalities of the pelgeroid type in myelodysplastic syndromes (MDS). Hypogranulation was measure both as the percentage of agranular neutrophils and as a score value, taking into account also cells with slight or moderate hypogranulation. We studied 62 cases of MDS (18 refractory anemia, 11 sideroblastic anemia, 26 refractory anemia with excess of blasts, three chronic myelomonocytic leukemias, four refractory anemia with excess of blasts in transformation). For comparison we studied 13 cases of myeloproliferative disorders, 18 patients with different forms of anemia and 20 normal controls. Reference values were defined as the 95% probability limit of the mean values of normal controls. In MDS 52/62 patients (84%) had increased numbers of pelgeroid cells, 40/62 (65%) had abnormal granulation scores while only 14/62 (23%) had increased percentages of agranular neutrophils. The mean granulation score (+/- S.D.) in MDS (225.0 +/- 57.4), was significantly lower (p less than 0.001) than in myeloproliferative disorders (282.7 +/- 9.0), anemias (288.8 +/- 8.0) and normal controls (281.7 +/- 12.9). Pelgeroid neutrophils were significantly (p less than 0.001) more common in MDS (11.6% +/- 7.8) compared to myeloproliferative disorders (1.1% +/- 1.0), anemias (3.1% +/- 2.0) and normal controls (1.9% +/- 1.5). There was no significant correlation between the degree of hypogranulation and the percentages of pelgeroid cells in individual patients. Hypogranulation tended to be more pronounced in the more immature forms of MDS while pelgeroid cells were equally common in the different subgroups. When the two parameters were combined peripheral blood dysplasia was recognized in 92% of the MDS cases. The results suggest that quantitative estimation of hypogranulation and of nuclear abnormalities in peripheral blood polymorphs are simple and valuable diagnostic tools in MDS.
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PMID:Diagnostic significance of dysplastic features of peripheral blood polymorphs in myelodysplastic syndromes. 292 74

A 50-year-old woman with typical acquired primary pure red cell aplasia (PRCA) was successfully treated with prednisone. A later relapse was preceded by a period of ineffective erythropoiesis characterized by a reticulocyte response inappropriately low for the degree of anemia, serum iron of 189 micrograms/dl, total iron-binding capacity (TIBC) of 213 micrograms/dl, and erythroid hyperplasia. In addition there was marked dyserythropoiesis and erythroblast-phagocytosis. One month later, bone marrow examination showed classic PRCA. This rarely reported evolutionary stage of PRCA has several implications: 1) it suggests antibody induced erythroblast cytotoxicity as one mechanism of PRCA; 2) at a particular time in the development of PRCA there is potential for misdiagnosis as primary refractory anemia (PRA); and 3) some cases of PRA with similar morphologic and laboratory findings may be pathogenetically related to PRCA and may benefit from evaluation for immune-mediated suppression of erythropoiesis.
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PMID:Dyserythropoiesis and erythroblast-phagocytosis preceding pure red cell aplasia. 312 7

A 21-year-old Fanconi anemia patient developed refractory anemia. Laboratory studies revealed a transitory increased platelet count and a typical del(5q). Bone marrow karyotyping showed a -6, +der(6)t(1;6)(q12;p25) rearrangement and, two years later, a mosaic -6, +der(6),t(1:6)(q12;p25)/-2, +der 2), t(1;2)(q12;q37) constitution. The chromosome mechanism operating in this patient is discussed.
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PMID:Karyotype evolution in the bone marrow of a patient with Fanconi anemia: breakpoints in clonal anomalies of this disease. 324 78

Anatomical observations have indicated a decrease of marrow cellularity with age, but these changes are not associated with anemia in the healthy geriatric patient. Aged patients with refractory anemia should be studied by utilizing red cell volume (MCV) and red cell heterogeneity (RDW). A classification with these indices initially can separate the anemias for a more fruitful investigation. By old age the anemias of hereditary red cell membrane or hemoglobin disorders should be known to the patient. In the absence of tumor, elderly patients have an increasing frequency of refractory anemias that can be called preleukemia or myelodysplastic syndrome. Morphological observations have emphasized the importance of abnormal megakaryocytes and platelets in all phases of preleukemia, and these cytologic changes should be used to guide the physician in the early diagnosis of the syndrome complex. This group of refractory anemias have a limited survival, but nonspecific marrow stimulation can be effective and should be tried. With a more complete classification of the chromosomal abnormalities in the myelodysplastic syndrome, a more accurate prognosis can be anticipated. The anemias of marrow aplasia and ineffective iron utilization (anemia of chronic disease) are found frequently in the elderly, and the physician may offer more effective therapy by an early diagnosis.
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PMID:Refractory anemia in the elderly. 354 48

Bone marrow failure encompasses a broad spectrum of disorders including aplastic, dysmyelopoietic and myelophtisic anemias. In the present study, these anemias were characterized according to the degree of erythroid proliferation and efficiency of erythropoiesis. Total erythropoietic activity was evaluated in 43 patients by measuring the erythron transferrin uptake (ETU). It averaged 20% of basal (range 3-43%) in 13 patients with severe aplastic anemia, 75% of basal (range 60-103%) in 3 patients with extensive bone marrow infiltration by neoplastic cells, 131% of basal (range 50-217%) in 16 patients with refractory anemia, and 452% of basal (range 63-720) in 11 patients with idiopathic refractory siderobastic anemia. Respective efficiencies of erythropoiesis were 74% in aplastic anemia, 70% with bone marrow infiltration, 46% in refractory anemia, and 14% in sideroblastic anemia. Based on the ETU, patients could be categorized into absolute marrow failure, relative marrow failure, and adequate erythropoietic response to anemia. This simple determination of proliferating activity of the erythroid marrow can provide useful information on the pathophysiology of marrow failure and a basis for the selection of therapeutic approaches.
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PMID:Pathophysiological classification of acquired bone marrow failure based on quantitative assessment of erythroid function. 365 64

A total of 109 patients with myelodysplastic syndromes (MDS) was analyzed to determine the clinical and pathologic features of the five recently defined French-American-British Cooperative Group (FAB) subtypes, and to assess the utility of this classification system in predicting survival, evolution to acute nonlymphocytic leukemia (ANLL), and cause of death. All patients with MDS presented with anemia; additional cytopenias were present in patients with refractory anemia with excess blasts (RAEB), chronic myelomonocytic leukemia (CMML) and refractory anemia with excess blasts in transformation to ANLL (RAEB/Tr). Thirty-two patients received some form of antileukemic therapy for MDS. ANLL developed in 16 of the 77 remaining untreated patients, including 18% (2/11), 0% (0/21), 22% (5/23), 33% (2/6), and 44% (7/16) of patients with refractory anemia (RA), refractory anemia with ring sideroblasts (RARS), RAEB, CMML, and RAEB/Tr, respectively (P = 0.02). The FAB subtype was highly predictive of survival with median survivals ranging from 71 months for RARS to 5 months for RAEB/Tr (P = less than 0.0001). Patients with RAEB, CMML, and RAEB/Tr frequently died of direct consequences of MDS, while patients with RA and especially RARS generally survived or died from unrelated disorders (P = less than 0.0001). MDS encompass a spectrum of disorders. RA and RARS, are relatively indolent and often do not lead to the patient's demise. RAEB, CMML, and RAEB/Tr are aggressive disorders which are often responsible for the patient's death whether or not actual progression to overt leukemia occurs. FAB subtype predicts survival, evolution to ANLL, and cause of death, although the five morphologic subtypes appear to separate into only two disease groups, especially with regard to survival and cause of death.
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PMID:Myelodysplastic syndromes. A clinical and pathologic analysis of 109 cases. 389 Oct 71

Erythropoiesis was studied in vitro in 16 selected patients with primary refractory anemia without excess of blasts who have been followed for an average of 4.8 years. The number of erythroid colonies and bursts grown in vitro from the patients' marrows did not correlate with any parameter of their disease or their prognosis. The response of marrow erythroid precursor cells to erythropoietin was found to be normal. In no case was a serum or IgG inhibitor of erythropoiesis detected either by quantitation of heme synthesized by marrow cells or by the erythroblast cytotoxicity assay. A clinically significant response of the anemia to corticosteroids was noted in three out of 14 patients. Ten patients died during the followup period, eight of them as a consequence of their hematologic disorder. Bone marrow aplasia with pancytopenia developed in six cases, increased number of marrow blasts in two cases, myelofibrosis with myeloid metaplasia in one case and a spontaneous remission in another case. Refractory anemia without excess of blasts is a heterogeneous disorder with variable natural history including evolution into marrow aplasia.
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PMID:Primary refractory anemia: clinical and laboratory study of erythropoiesis in 16 patients. 400 31

The suppressive effect of two types of human interferon (fibroblast and leukocyte types) on bone marrow and peripheral blood erythroid colony formation by cells from patients with various disorders of erythropoiesis was studied. Bone marrow or peripheral blood mononuclear cells were isolated and cultured in plasma clots with Epo, and benzidine-positive erythroid colonies counted after 7 to 14 days' incubation. Specimens included cells from patients with thalassemia, sickle cell anemia, secondary polycythemia, nutritional anemia, hemolytic anemia, refractory anemia, and normal controls. Results show that with the exception of nutritional anemia cells, erythroid colony formation by all specimens was significantly inhibited (84% to 100%) by 100 to 200 U of either interferon type per milliliter. Erythroid colony formation by nutritional anemia bone marrow cells ws inhibited only 30% to 40% by 200 U/ml fibroblast or leukocyte interferon, and 100 U/ml were ineffective. Sickle cell peripheral blood mononuclear cells and refractory anemia bone marrow demonstrated marked inhibition of colony formation (86% to 97%) with 50 U/ml fibroblast or leukocyte interferon. Inhibition of colony formation by sickle cell peripheral blood mononuclear cells was completely abolished by addition of anti-interferon. Colony formation by refractory anemia bone marrow was inhibited 46% to 51% by as little as 10 U/ml fibroblast or leukocyte interferon. This concentration of interferon was ineffective with cells from thalassemia, secondary polycythemia, nutritional anemia, hemolytic anemia, and controls. Mouse bone marrow colony formation was not suppressed by 200 U/ml leukocyte interferon. These results demonstrate that fibroblast or leukocyte interferons inhibit in vitro erythroid colony formation by human bone marrow or peripheral blood mononuclear cells, the effect is abolished by anti-interferon, and inhibition may be species-specific. These studies reveal that cells obtained from certan patients are particularly sensitive to the cytoxic effects of interferon, and it may be useful to monitor the erythropoietic state of the patient during interferon chemotherapy.
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PMID:Suppressive effect of human interferons on erythroid colony growth in disorders of erythropoiesis. 615 73

The haematological features of 118 cases of primary myelodysplastic syndromes (PMDS) were reviewed to see how these could be related and classified according to the recent FAB proposals. A majority of the cases were elderly who presented with a macrocytic or normocytic anaemia and reticulocytopenia. Cases of acquired idiopathic sideroblastic anaemia (AISA) usually had normal leucocyte and platelet counts, erythroid hyperplasia, marked dyserythropoiesis and more than 20% ringed sideroblasts. Cases of refractory anaemia with excess of blasts (RAEB) had frequent neutropenia and thrombopenia usually with prominent dysgranulopoiesis and dysthrombopoiesis. Refractory anaemia or refractory cytopenia appeared morphologically to be a heterogeneous group. Leukaemic transformation did not occur in any of these 16 cases of AISA whereas six of the 34 cases of RAEB transformed into acute leukaemia. It appears that the cases of PMDS present with well defined haematological features which permit recognition of different groups; these latter groups appear to be morphologically and prognostically distinct.
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PMID:Haematological features of primary myelodysplastic syndromes (PMDS) at initial presentation: a study of 118 cases. 661 10


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