A Morphometric Analysis of Platelet Dense Granules of Patients with Unexplained Bleeding: A New Entity of Delta-Microgranular Storage Pool Deficiency

被引:15
作者
Gunning, William T., III [1 ]
Raghavan, Meera [2 ]
Calomeni, Edward P. [1 ]
Turner, James N. [3 ]
Roysam, Bodri [4 ]
Roysam, Shantala
Smith, Mary R. [5 ]
Kouides, Peter A. [6 ]
Lachant, Neil A. [7 ]
机构
[1] Univ Ohio, Dept Pathol, Toledo, OH 43614 USA
[2] Kansas City Univ Med & Biosci, Kansas City, MO 64106 USA
[3] New York State Dept Hlth, Albany, NY 12203 USA
[4] Univ Houston, Dept Elect & Comp Engn, Houston, TX 77204 USA
[5] Univ Toledo, Dept Med, Toledo, OH 43614 USA
[6] Rochester Gen Hosp, Mary Gooley Hemophilia Ctr, Rochester, NY 14621 USA
[7] Univ Rochester, Wilmot Canc Inst, Med Ctr, Rochester, NY 14642 USA
关键词
unexplained bleeding; platelet storage pool deficiency; platelet dense granules; CONGENITAL DISORDERS; ADENINE-NUCLEOTIDES; RELEASE REACTION; AGGREGATION; TIME; ULTRASTRUCTURE; HEREDITARY; SECRETION; DIAGNOSIS; DISEASE;
D O I
10.3390/jcm9061734
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
One thousand and eighty patients, having prolonged bleeding times, frequent epistaxis, menorrhagia or easy bruising or other bleeding manifestations, and excluding those with von Willebrand's disease, were evaluated for platelet dense granule deficiency. The mean diameter of platelet dense granules was determined for all patients using image analysis. Four hundred and ninety-nine had "classic" dense (delta) granule storage pool deficiency (delta-SPD). Five hundred and eighty-one individuals (53.8%) were found to have a normal mean number of dense granules, but for some of these patients, the dense granules were smaller than for the controls. Of the patients having a normal number of dense granules, 165 (28.4%) were found to have significantly smaller granules than the platelets obtained from the control subjects. Their average granule diameter was 123.35 +/- 0.86 nm, that is more than three standard deviations below the mean of the control data. Total delta-granule storage pool volumes (TDGV)/platelet were calculated using these measurements. Individuals with delta-SPD had half the number of granules (2.25 +/- 0.04 DG/PL) and storage pool volume (3.88 +/- 1.06 x 10(6)nm(3)) when compared to our control data (4.64 +/- 0.11 DG/PL; 10.79 x 10(6)nm(3)+/- 0.42). Individuals having a bleeding history but a normal average of small dense granules had a calculated storage pool volume statistically different than controls and essentially the same storage pool volume as patients with delta-SPD. We have identified a sub-classification of delta-SPD that we have defined as micro-granular storage pool deficiency (delta-MGSPD).
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页数:15
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共 63 条
  • [1] ATP-ADP COMPARTMENTATION IN STORAGE POOL DEFICIENT PLATELETS - CORRELATION BETWEEN GRANULE-BOUND ADP AND THE BLEEDING-TIME
    AKKERMAN, JWN
    NIEUWENHUIS, HK
    MOMMERSTEEGLEAUTAUD, ME
    GORTER, G
    SIXMA, JJ
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 1983, 55 (01) : 135 - 143
  • [2] HEMATOCRIT AND BLEEDING-TIME - AN UPDATE
    ANAND, A
    FEFFER, SE
    [J]. SOUTHERN MEDICAL JOURNAL, 1994, 87 (03) : 299 - 301
  • [3] THE STORAGE POOL DEFICIENCY IN PLATELETS FROM HUMANS WITH THE CHEDIAK-HIGASHI-SYNDROME - STUDY OF 6 PATIENTS
    APITZCASTRO, R
    CRUZ, MR
    LEDEZMA, E
    MERINO, F
    RAMIREZDUQUE, P
    DANGELMEIER, C
    HOLMSEN, H
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 1985, 59 (03) : 471 - 483
  • [4] POTENTIATION OF UREMIC BLEEDING BY HEREDITARY STORAGE POOL DISEASE
    BERTY, RM
    ZEIGLER, ZR
    BRUNS, FJ
    [J]. AMERICAN JOURNAL OF KIDNEY DISEASES, 1992, 19 (04) : 326 - 330
  • [5] Blanchette V S, 1991, Baillieres Clin Haematol, V4, P291, DOI 10.1016/S0950-3536(05)80162-3
  • [6] QUANTITATIVE-EVALUATION OF MEPACRINE LABELED HUMAN PLATELET DENSE BODIES IN NORMALS AND IN CASES OF PERIPHERAL THROMBOCYTOPENIA
    BONEU, B
    CARANOBE, C
    CAPDEVILLE, J
    ROBERT, A
    BIERME, R
    [J]. THROMBOSIS RESEARCH, 1978, 12 (05) : 831 - 839
  • [7] Electron microscopy examination of platelet whole mount preparations to quantitate platelet dense granule numbers: Implications for diagnosing suspected platelet function disorders due to dense granule deficiency
    Brunet, J. G.
    Iyer, J. K.
    Badin, M. S.
    Graf, L.
    Moffat, K. A.
    Timleck, M.
    Spitzer, E.
    Hayward, C. P. M.
    [J]. INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, 2018, 40 (04) : 400 - 407
  • [8] BUCHANAN GR, 1976, BLOOD, V47, P941
  • [9] ULTRASTRUCTURE OF NEGATIVELY STAINED PLATELETS - SOME PHYSIOLOGIC IMPLICATIONS
    BULL, BS
    [J]. BLOOD-THE JOURNAL OF HEMATOLOGY, 1966, 28 (06): : 901 - +
  • [10] Carr ME, 1997, AM J MED SCI, V313, P176