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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A study of 196 healthy geriatric females and 26 males confirmed the high prevalence of anemia in this population. Forty-two females (21.4%) had a hematocrit of less than 36% and nine (34%) of the males had a value of less than 40%. A careful evaluation, including a therapeutic trial of oral iron in some subjects, made a diagnosis of iron deficiency anemia in only three and the anemia of chronic disease could be diagnosed in two. No subject had folate or B12 deficiency and hemolytic anemia was not present. Thus, in most of these subjects, the lower hematocrit was not due to commonly recognized causes. Further evaluation revealed a high prevalence of leukopenia in this population; 30% having counts less than 5 X 10(3)/microliter and 10% having values less than 4 X 10(-3)/microliter. Of most importance, leukocyte counts were significantly lower in anemic subjects in whom a high incidence of neutropenia was also demonstrated. This suggests that the mechanisms of the unexplained anemia and neutropenia is an overall reduction in hematopoietic cell numbers. This conclusion is consistent with the observation that many aged subjects have a decreased ability to mount an adequate leukocyte response to infection. These findings suggest that the presence of anemia marks an overall reduction in hematopoietic reserve and provides a clue to those aged subjects of greatest risk of an inadequate response to stress.
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PMID:The anemia of senescence. 727 May 45

A 6-month-old full-term infant had severe anemia and neutropenia. The patient was being fed cow's milk and a diet of corn flour. Thorough investigation revealed low serum iron concentration, severe hypocupremia, low ceruloplasmin, retardation of bone age, and metaphysial irregularities and spurring. Bone marrow aspirate revealed cytoplasmic vacuolization in precursors of the erythroid and myeloid series and ringed sideroblasts. Therapy with oral iron, folic acid, and vitamin C was futile. Administration of copper sulfate resulted in brisk increase in neutrophils and reticulocytes. The child maintained normal levels of hemoglobin, neutrophils, serum copper and ceruloplasmin, and serum iron one year after copper therapy was discontinued. The probable role of unrecognized copper deficiency in causing anemia in infants more than 6 months of age is discussed, and the importance of serum copper examination in refractory iron deficiency anemia and neutropenia is stressed. To the best of our knowledge, no such case has previously been described in the literature.
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PMID:Copper deficiency with cow's milk diet. 727 67

This report updates the combined experience of four centres involved in the long-term treatment of transfusional iron overload in 84 patients with the oral iron chelator deferiprone (L1) over 167 patient-years. The source of L1 was variable, including two university research laboratories and three pharmaceutical firms. Compliance was rated as excellent in 48%, intermediate in 36%, and poor in 16% of patients. On a mean L1 dose of 73-81 mg/kg/d, urinary iron excretion was stable, at around 0.5 mg/kg/d, with no indication of a diminishing response with time. Serum ferritin showed a very steady decrease with time from an initial mean +/- 1 SD of 4207 +/- 3118 to 1779 +/- 1154 micrograms/l after 48 months (P < 0.001). 17 patients abandoned L1 therapy. Major complications of L1 requiring permanent discontinuation of treatment included agranulocytosis (three), severe nausea (four), arthritis (two) and persistent liver dysfunction (one). The remaining patients abandoned treatment because of low compliance (three) and conditions unrelated to L1 toxicity (four). Lesser complications permitting continued L1 treatment included transient mild neutropenia (four), zinc deficiency (12), transient increase in liver enzymes (37), moderate nausea (three) and arthropathy (17). There was no treatment-related mortality. Although the complications associated with L1 treatment are significant and require close monitoring, they do not preclude effective long-term therapy in the vast majority of patients. Further well-controlled prospective studies of L1 are required in order to enable proper judgement of its suitability for general long-term clinical use.
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PMID:Results of long-term deferiprone (L1) therapy: a report by the International Study Group on Oral Iron Chelators. 757 38

Trace mineral deficiencies tend to have profound effects on the integrity of formed blood elements. Anemia and neutropenia are commonly seen in copper (Cu) deficiency. We therefore developed a serum-free medium to examine the trace mineral requirements, in particular iron and Cu, for proliferation and retinoic acid (RA)-induced differentiation of HL-60 cells. This defined medium (DFM) was composed of Iscove's Modified Dulbecco's Medium (IMDM) supplemented with insulin and human apo-transferrin (each at 5 micrograms/ml) and 1.4 microM FeSO4. The iron concentration range for optimal cellular proliferation was narrow (2-3 microM). HL-60 cells could be maintained in DFM for 15 passages with a doubling time of 38-40 hr. The Cu content of IMDM was very low. Thus, by the fourth passage in DFM, the activity of cuproenzymes (cytochrome c oxidase, CCO; and copper-zinc superoxide dismutase, CuZnSOD) began to decline. Supplementation of DFM with CuSO4 (50 nM) restored enzyme activities. Treatment of cells with a Cu chelator (tetrathiomolybdate, 1 microM) rapidly reduced the activities of both CCO and CuZnSOD. Over the Cu concentration range examined (5-350 nM), Cu supplementation had little effect on HL-60 proliferation. Cell retained the ability to differentiate along the granulocytic pathway when treated with RA, but seemed to be less sensitive to the inducing agent except at the highest concentration tested (1 microM). This decreased sensitivity to RA did not seem to be related to the Cu status of the cells but rather to the absence of a component of serum. Indeed, cells grown in DFM regained their sensitivity to RA when allowed to differentiate in IMDM with 5% serum. These data indicate that the processes of growth and terminal differentiation in HL-60 cells are not greatly influenced by Cu. Thus, it seems likely that the insult resulting in neutropenia which is associated with Cu deficiency may occur earlier than the promyelocytic stage. However, the possibility that the mechanisms contributing to neutropenia may be unrelated to primary defects in the biochemistry of neutrophil maturation cannot be ruled out.
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PMID:Iron and copper requirements for proliferation and differentiation of a human promyelocytic leukemia cell line (HL-60). 777 91

Agranulocytosis developed in a 63-year-old patient with myelodysplasia 6 weeks after commencing treatment with the oral iron chelator deferiprone (L1, 1,2-dimethyl-3-hydroxypyrid-4-one, CP20) at a daily dose of 79 mg/kg. This was the 3rd case of agranulocytosis (neutrophils 0 x 10(9)/l) in clinical trials of L1 at the Royal Free Hospital. The neutrophil count recovered 7 days after stopping L1 and commencing G-CSF at a dose of 300 micrograms daily. Three other patients with milder degrees of neutropenia (neutrophils < 1.5 x 10(9)/l) have also been observed in our trials. The case histories of these 4 patients are described here; other reported cases of neutropenia or agranulocytosis are reviewed. Based on worldwide long-term clinical trials the incidence of agranulocytosis is about 1.6% and of neutropenia 2%.
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PMID:Deferiprone-associated myelotoxicity. 781 10

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease, whose origin seems to lie in a acquired defect in the membrane of the pluri-potential hematopoietic cell. Chronic or intermittent acute hemolytic syndrome is the most frequent clinical manifestation, although in the literature there are also some references to the leukocytic and immunologic disorders of this disease. In this paper, we present the case of a 63-year-old patient with NPH who developed severe neutropenia and sustained febrile syndrome. In the past four years, she had suffered frequent episodes of fever and leukopenia, which apparently disappeared spontaneously. In the physical exploration, we observed hepatosplenomegaly. The hemogram showed mild iron deficiency anemia (hemoglobin 10.8 g/dl), severe neutropenia (neutrophil 0.3 x 10(9)/l) and significant reticulocytosis (610 x 10(9)/l). Iron deposits were greatly reduced in the marrow. Simultaneously to a new febrile episode and isolation of Escherichia coli in the urine, there was a severe anemization (hemoglobin 5 g/dl) and a significant thrombopenia (platelets 30 x 10(9)) resulting in a positive hemosiderinuria and sucrose test. The study of the leukocytic function showed a defect in the neutrophil chemotaxis, although a normal phagocytic capacity and microbicidal activity. In the following nine months, the patient had several severe infections, with intense but transitory pancytopenia, which always improved when treating the infection with antibiotics. The patient died due to a septic shock twelve months after the diagnosis. Recurrent febrile episodes and severe neutropenia are very rare in the PNH (less than 4% of the cases). The cause of these disorders is still unknown.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Recurrent infections, severe neutropenia and neutrophil chemotaxis defect in paroxysmal nocturnal hemoglobinuria]. 786 56

The clinicohaematological findings of acute state (Group A, 30 patients) and chronic state (Group B, 34 patients) of falciparum malaria in paediatric patients are compared. The children with chronic falciparum malaria were apyrexic and presented with features of moderate to severe anaemia with hepato-splenomegaly. Greater severity of anaemia and haemolysis, higher incidence and severity of neutropenia, atypical lymphocytosis, monocytosis and thrombocytopenia were observed in patients with chronic falciparum malaria as compared to patients with acute falciparum malaria despite lesser degree of parasitaemia in the former as compared to the latter. While mechanical destruction of parasitised RBC's, ineffective and dysplastic erythropoiesis either due to unmasking of border line dierty folic acid deficiency or otherwise, transient hypoplasia of bone marrow, impaired utilization of iron and immune destruction of RBCs with hypersplenism may be the mechanisms for anaemia, transient hypoplasia of bone marrow and hypersplenism may be the factors responsible for thrombocytopenia and neutropenia.
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PMID:Clinico-haematological profile in acute and chronic Plasmodium falciparum malaria in children. 796 80

In a significant proportion of patients with acute myeloid leukemia (AML), a series of hematological alterations--including refractory anemia, neutropenia, thrombocytopenia, abnormal iron metabolism, and elevated levels of blast cells both in peripheral blood and bone marrow--are observed before the diagnosis of AML is made. This preleukemic state has called the attention of several investigators around the world, since it represents a way to study the origin and progression of leukemia in man. During the past 5 years, major advances in the molecular and cellular biology of this disease have been achieved. It is now known that preleukemia is a clonal disorder that arises from a malignant transformation at the level of primitive pluripotent hemopoietic stem cells. The hemopoietic progenitors in preleukemic patients have abnormal responses to hemopoietic regulators, thus, they do not seem to follow the controlled proliferation observed in the hemopoietic system under normal conditions. The mechanisms of cell differentiation and maturation are also altered, leading to the production of immature (blast) cells, instead of the development of fully mature erythrocytes, granulocytes, platelets and lymphocytes. Several oncogenes, such as C-FMS and RAS, have been found to be structurally altered in a significant proportion of preleukemic patients, suggesting that they may be involved in the pathogenesis of the disease. In spite of the advances made during the last few years, major questions regarding the biology of this hematological disorder are still unanswered.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Human preleukemia: cellular, molecular and clinical aspects. 811 54

A 33 year-old man with undiagnosed neuropathy showing mental retardation and involuntary movements has been nourished for a long period by total parenteral nutrition (TPN) because of frequent vomiting and repeated aspiration pneumonitis. After ten months' TPN, macrocytic anemia and neutropenia developed and iron preparation was administered without hematological improvement. Bone marrow examination revealed normocellular marrow without features of megaloblastosis and dysplasia. In some erythroblasts and immature myeloid cells, vacuoles were observed and mature granulocytes were reduced in the bone marrow. Both serum copper and ceruloplasmin were very low (12 micrograms/dl and 7mg/dl, respectively). Thus, oral administration of copper sulfate resulted in marked increase of reticulocytes and subsequent improvement of anemia and neutropenia within two months. Copper deficiency is a rare condition, but during an unusual nutrition such as TNP, hematological abnormality due to copper deficiency must be noticed to occur.
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PMID:[Anemia and neutropenia due to copper deficiency during long-term total parenteral nutrition]. 849 15

We have reported that lactoferrin, a 77-kDa iron-binding glycoprotein found in secondary neutrophil granules, provides a useful marker of fecal leukocytes in fecal specimens from patients with inflammatory diarrhea (R. L. Guerrant, V. Araujo, E. Soares, K. Kotloff, A. A. M. Lima, W. H. Cooper, and A. G. Lee, J. Clin. Microbiol. 30:1238-1242, 1992). In order to determine the usefulness of this marker of neutrophilic inflammation in different body fluids, we examined blood, gingival swabs, sputum, and saliva using antilactoferrin antibodies (lactoferrin latex agglutination [LFLA]). LFLA titers in whole blood samples were < or = 1:4 in all eight samples from patients with neutropenia (absolute neutrophil count [ANC] = < 150 polymorphonuclear cells [PMNs] per microliter), < or = 1:8 in samples from 13 individuals with moderate leukocyte counts (ANC = 150 to 8,000), and 1:8 to 1:32 in samples from six patients with neutrophilia (ANC > 8,000). While the overlap precludes a useful role in the identification of neutropenia, these data confirm that lactoferrin titers of > 1:100 indeed indicate inflammation in fluid specimens. On quantitative elution of lactoferrin from gingival swabs, all 7 patients with dental plaque had titers of 1:200 to 1:400; 9 of 12 patients with clinical gingivitis had LFLA titers of 1:200 to 1:1,600, while all 7 individuals with healthy gums and teeth and 4 edentulous patients had LFLA titers of < or = 1:100. Eight purulent sputum samples had titers of > or = 1:400 (7 were 1:1,600) while 11 normal saliva samples showed titers of < or = 1:100. Lactoferrin titers in sputum, gingival swabs, and whole blood correlate with the presence of neutrophils or inflammation in these specimens and may offer a convenient rapid test for inflammatory processes.
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PMID:Correlation of lactoferrin with neutrophilic inflammation in body fluids. 857 44


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