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Hematological Abnormalities in a Family

J. WORTH ESTES, MD; ROBERT J. CAREY, MD; RAJENDRA G. DESAI, MD, PhD

Arch Intern Med. 1965;116(6):889-893.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

ABNORMALITIES of hemostasis have been described in patients having heritable disorders of connective tissue, namely the Ehlers-Danlos syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, and Marfan's syndrome.^1-20 Qualitative platelet defects have been found in osteogenesis imperfecta ^13,14 and subjects with the May-Hegglin anomaly, whereas factor VIII (antihemophilic factor, AHF) deficiency has been associated with Marfan's syndrome.^15,16 This report is concerned with several members of a family with Marfan's syndrome in whom morphological, functional, and biochemical abnormalities of platelets and polymorphonuclear neutrophils were observed.

Methods

Various hematological tests, namely hemoglobin determination, hematocrit, total and differential white cell counts, and platelet counts (phase contrast method) were done by routine methods. Coagulation tests included clotting time, tourniquet test, prothrombin time, prothrombin consumption, thromboplastin generation test (TGT) using platelet substitute,²¹ and recalcification time of platelet-rich plasma²² to estimate platelet factor 3 (thromboplastic activity). Clot retraction was estimated semiquantitatively 24 hours after blood was withdrawn.²⁸ A few . . . [Full Text PDF of this Article]

Author Affiliations
BOSTON

From the Department of Hematology, University Hospital, Boston.

Footnotes
Submitted for publication March 20, 1965; accepted Aug 13.

Reprint requests to 750 Harrison Ave, Boston, Mass 02118 (Dr. Estes).

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