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February 1971, Vol 121, No. 2 >
Article | February 1971

Thrombasthenia FREE

Donald G. Corby, MD; Christy L. Zirbel; Annabelle Lindley; Irving Schulman, MD
[+] Author Affiliations

Received for publication July 16, 1970.

Reprint requests to Department of Pediatrics, University of Illinois Hospital, 840 S Wood St, Chicago 60612 (Dr. Schulman).


Am J Dis Child. 1971;121(2):140-144. doi:10.1001/archpedi.1971.02100130094011.
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Two siblings, with a congenital hemorrhagic state characteristic of thrombasthenia, had a history of frequent ecchymoses and prolonged bleeding following minor abrasions. Results of laboratory studies revealed normal platelet counts, long bleeding times, deficient platelet factor 3 availability, and absence of platelet aggregation following the addition of adenosine diphosphate, collagen, and epinephrine. Results of other clotting studies were normal. Three bleeding episodes, two gastrointestinal hemorrhages and one subdural hematoma, were treated with platelet concentrates with clinical and laboratory evidence of restoration of the platelet mediated phase of hemostasis. Platelet infusions appear to be effective in treatment of serious bleeding in these patients.

REFERENCES

1 +
Luscher EF:  Platelets in haemostasis and thrombosis . Brit J Haemat 13:1-9, 1967;.
2 +
Marcus AJ:  Platelet function . New Eng J Med 280:1213-1220,1969;.
3 +
Hardisty RM:  Platelet aggregation and platelet factor 3 availability in bleeding disorders: Experimental biology and medicine , in Hagen E, Wechsler W, Zillike I (eds): Platelets in Hemostasis . New York, S Karger, 1968;, vol 3.
4 +
Glanzmann E:  Hereditare hamorrhagische Thrombasthenie: Ein Beitrag zur Pathologie der Blutplattchen . J Kinderheilk 88:113-120,1918;.
5 +
Born GVR, Cross MJ:  Aggregation of blood platelets . J Physiol 168:178-195,1963;.
6 +
Simone JV, Vanderheiden J, Abilgaard CF:  A semiautomatic one-stage factor VIII assay with a commercially prepared standard . J Lab Clin Med 69:706-712, 1967;.
7 +
Lempert H:  A modified technique for the enumeration of blood platelets . Lancet 1:151-154,1935;.
8 +
Borchgrevink CF, Waaler BA:  The secondary bleeding time: A new method for the differentiation of hemorrhagic diseases . Acta Med Scand 162:363-370, 1958;.
9 +
Hume M:  Platelet entrapment in glass spheres: Clinical and experimental application . Ann Surg 166:59-64, 1967;.
10 +
Quick AJ:  Prothrombin time in hemophilia and in obstructive jaundice . J Biol Chem 109:73-75, 1935;.
11 +
Hardisty RM, Hutton RA:  A kaolin clotting time of platelet-rich plasma: A test of platelet factor 3 availability . Brit J Haemat 2:258-272, 1965;.
12 +
Mustard JF, Hegardt B, Rowseel HC, et al:  Effect of adenosine nucleotides on platelet aggregation and clotting time . J Lab Clin Med 64:548-559, 1964;.
13 +
Hardisty RM, Dormandy KM, Hutton RA:  Thrombasthenia: Studies on three cases . Brit J Haemat 10:371-387, 1964;.
14 +
Weiss HJ:  Analytic review: von Willebrand's disease—diagnostic criteria . Blood 32:668-679, 1968;.
15 +
Weiss HJ:  Platelet aggregation, adhesion, and adenosine diphosphate release in thrombopathia (platelet factor 3 deficiency); comparisons with Glanzmann's thrombasthenia and von Willebrand's disease . Amer J Med 43:570-578, 1967;.
16 +
Hardisty RM, Hutton RA:  Bleeding tendency associated with "new abnormality of platelet behavior." Lancet 1:983-985,1967;.
17 +
Hirsch J, Castelan DJ, Loder PB:  Spontaneous bruising associated with defect in interaction of platelets with connective tissue . Lancet 2:18-21,1967;.
18 +
O'Brien JR:  Platelets: Portsmouth syndrome? Lancet 2:258,1967;.
19 +
Caen JP, Sultan Y, Larrieu MJ:  New familial platelet disease . Lancet 1:203,1968;.
20 +
Sahud MA, Aggler PM:  Platelet dysfunction—differentiation of a newly recognized primary type from that produced by aspirin . New Eng J Med 280:453-459, 1969;.
21 +
Stewart JH, Castaldi PA:  Uremic bleeding: A reversible platelet defect corrected by dialysis . Quart J Med 143:409-423,1967;.
22 +
Thomas DP, Ream VJ, Stuart RK:  Platelet aggregation in patients with Laennec's cirrhosis . New Eng J Med 276:1344-1348, 1967;.
23 +
Wilson PA, MacNicol GF, Douglas AS:  Platelet abnormality in human scurvy . Lancet 1:975-978, 1967;.
24 +
Spaet TH, Lejnieks I, Gaynor E, et al:  Defective platelets in essential thrombocythemia . Arch Intern Med 124:135-141, 1969;.
25 +
O'Brien JR:  Effects of salicylates on human platelets . Lancet 1:779-783,1968;.
26 +
Hermann RG, Frank JD:  Effect of adenosine derivatives and antihistamines on platelet aggregation . Proc Soc Exp Biol Med 123:654-660, 1967;.
27 +
Mills DCB, Robb IA, Roberts GCK:  Release of nucleotides, 5-hydroxytryptamine, and enzymes from human blood platelets during aggregation . J Physiol 195:715-729, 1968;.
28 +
Zieve PD, Solomon HM:  Effect of diuretics on the human platelet . Amer J Physiol 215:650-654, 1968;.
29 +
Hampton JR, Harrison MJG, Honour AJ, et al:  Platelet behavior and drugs used in cardiovascular disease . Cardiovasc Res 1:101-107, 1967;.
30 +
Cronberg S, Nilsson IM, Zellerquist E:  Investigation of a family with members with both severe and a mild degree of thrombasthenia . Acta Pediat Scand 56:189-197, 1967;.
31 +
Hirsch EO, Gardner FH:  The transfusion of human blood platelets . J Lab Clin Med 39:556-595,1952;.
32 +
Yankee RA, Grumet FC, Rogentine GN:  Platelet transfusion therapy: The selection of compatible platelet donors for refractory patients by lymphocyte HL-A typing . New Eng J Med 281:1208-1212,1969;.

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References

1 +
Luscher EF:  Platelets in haemostasis and thrombosis . Brit J Haemat 13:1-9, 1967;.
2 +
Marcus AJ:  Platelet function . New Eng J Med 280:1213-1220,1969;.
3 +
Hardisty RM:  Platelet aggregation and platelet factor 3 availability in bleeding disorders: Experimental biology and medicine , in Hagen E, Wechsler W, Zillike I (eds): Platelets in Hemostasis . New York, S Karger, 1968;, vol 3.
4 +
Glanzmann E:  Hereditare hamorrhagische Thrombasthenie: Ein Beitrag zur Pathologie der Blutplattchen . J Kinderheilk 88:113-120,1918;.
5 +
Born GVR, Cross MJ:  Aggregation of blood platelets . J Physiol 168:178-195,1963;.
6 +
Simone JV, Vanderheiden J, Abilgaard CF:  A semiautomatic one-stage factor VIII assay with a commercially prepared standard . J Lab Clin Med 69:706-712, 1967;.
7 +
Lempert H:  A modified technique for the enumeration of blood platelets . Lancet 1:151-154,1935;.
8 +
Borchgrevink CF, Waaler BA:  The secondary bleeding time: A new method for the differentiation of hemorrhagic diseases . Acta Med Scand 162:363-370, 1958;.
9 +
Hume M:  Platelet entrapment in glass spheres: Clinical and experimental application . Ann Surg 166:59-64, 1967;.
10 +
Quick AJ:  Prothrombin time in hemophilia and in obstructive jaundice . J Biol Chem 109:73-75, 1935;.
11 +
Hardisty RM, Hutton RA:  A kaolin clotting time of platelet-rich plasma: A test of platelet factor 3 availability . Brit J Haemat 2:258-272, 1965;.
12 +
Mustard JF, Hegardt B, Rowseel HC, et al:  Effect of adenosine nucleotides on platelet aggregation and clotting time . J Lab Clin Med 64:548-559, 1964;.
13 +
Hardisty RM, Dormandy KM, Hutton RA:  Thrombasthenia: Studies on three cases . Brit J Haemat 10:371-387, 1964;.
14 +
Weiss HJ:  Analytic review: von Willebrand's disease—diagnostic criteria . Blood 32:668-679, 1968;.
15 +
Weiss HJ:  Platelet aggregation, adhesion, and adenosine diphosphate release in thrombopathia (platelet factor 3 deficiency); comparisons with Glanzmann's thrombasthenia and von Willebrand's disease . Amer J Med 43:570-578, 1967;.
16 +
Hardisty RM, Hutton RA:  Bleeding tendency associated with "new abnormality of platelet behavior." Lancet 1:983-985,1967;.
17 +
Hirsch J, Castelan DJ, Loder PB:  Spontaneous bruising associated with defect in interaction of platelets with connective tissue . Lancet 2:18-21,1967;.
18 +
O'Brien JR:  Platelets: Portsmouth syndrome? Lancet 2:258,1967;.
19 +
Caen JP, Sultan Y, Larrieu MJ:  New familial platelet disease . Lancet 1:203,1968;.
20 +
Sahud MA, Aggler PM:  Platelet dysfunction—differentiation of a newly recognized primary type from that produced by aspirin . New Eng J Med 280:453-459, 1969;.
21 +
Stewart JH, Castaldi PA:  Uremic bleeding: A reversible platelet defect corrected by dialysis . Quart J Med 143:409-423,1967;.
22 +
Thomas DP, Ream VJ, Stuart RK:  Platelet aggregation in patients with Laennec's cirrhosis . New Eng J Med 276:1344-1348, 1967;.
23 +
Wilson PA, MacNicol GF, Douglas AS:  Platelet abnormality in human scurvy . Lancet 1:975-978, 1967;.
24 +
Spaet TH, Lejnieks I, Gaynor E, et al:  Defective platelets in essential thrombocythemia . Arch Intern Med 124:135-141, 1969;.
25 +
O'Brien JR:  Effects of salicylates on human platelets . Lancet 1:779-783,1968;.
26 +
Hermann RG, Frank JD:  Effect of adenosine derivatives and antihistamines on platelet aggregation . Proc Soc Exp Biol Med 123:654-660, 1967;.
27 +
Mills DCB, Robb IA, Roberts GCK:  Release of nucleotides, 5-hydroxytryptamine, and enzymes from human blood platelets during aggregation . J Physiol 195:715-729, 1968;.
28 +
Zieve PD, Solomon HM:  Effect of diuretics on the human platelet . Amer J Physiol 215:650-654, 1968;.
29 +
Hampton JR, Harrison MJG, Honour AJ, et al:  Platelet behavior and drugs used in cardiovascular disease . Cardiovasc Res 1:101-107, 1967;.
30 +
Cronberg S, Nilsson IM, Zellerquist E:  Investigation of a family with members with both severe and a mild degree of thrombasthenia . Acta Pediat Scand 56:189-197, 1967;.
31 +
Hirsch EO, Gardner FH:  The transfusion of human blood platelets . J Lab Clin Med 39:556-595,1952;.
32 +
Yankee RA, Grumet FC, Rogentine GN:  Platelet transfusion therapy: The selection of compatible platelet donors for refractory patients by lymphocyte HL-A typing . New Eng J Med 281:1208-1212,1969;.

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