1/223. Platelet antithrombin deficiency: a new clinical entity. A kindred with a history of multiple thromboses was studied for coagulant abnormalities. A deficiency of serum antithrombin III was found in approximately half of the 13 family members by either coagulant or immunologic assay. No clear relationship between antithrombin iii deficiency and a history of thrombosis was present. Platelet antithrombin assays were also studied in the same subjects. Ten of the 13 members were deficient. None of the remaining three had a history of thrombosis. On the basis of these findings, the hypothesis is proposed that in some cases familial hypercoagulability may be due to a platelet antithrombin deficiency and that the serum antithrombin iii deficiency in some cases is a secondary rather than a primary effect. ( info) |
2/223. Glanzmann's thrombasthenia. Report of two oral surgical cases using a new microfibrillar collagen preparation and EACA for hemostasis. Glanzmann's thromboasthenia is a rare congenital platelet disorder characterized by a prolonged bleeding time, a qualitative platelet defect, and severe hemorrhagic episodes. patients with this disorder have been managed by administration of blood and blood components (most recently, platelet-rich plasma and platelet concentrates) to control hemorrhage resulting from trauma or surgical procedures. The two case reports presented here illustrate the use of a local hemostatic agent (microfibrillar bovine collagen, Avitene) and a systemic fibrinolytic inhibitor (epsilon aminocaproic acid, Amicar) to control postoperative hemorrhage secondary to elective extraction of teeth. The clinical results demonstrate excellent postoperative hemostasis and support recent in vitro observation of platelet adherence to the collagen preparation. This provides an alternate therapeutic modality in the management of patients with Glanzmann's disease and possibly other disorders of platelet function. ( info) |
3/223. The platelets in preleukemia and myelomonocytic leukemia. Ultrastructural cytochemistry and cytogenetics. light and electron microscopic studies of platelets from 16 patients with myelomonocytic leukemia or "preleukemia" revealed major morphologic alterations in 15 and minor ones in 1. Although variable in severity from case to case, the changes present followed a distinct pattern. In most cases there were two platelet populations, one morphologically normal and one morpholigically abnormal. The most salient changes pertained to size (giant forms), shape (the platelets being rounded and probably spheroidal), decrease or absence of the microtubules, and increase in immature elements. A striking feature was the variation in size and shape of the granules, with truly giant forms (up to 2.5 mum) being present. In cytogenetic studies in 14 cases, there was no correlation between the chromosomal changes and the various types of platelet anomalies. ( info) |
4/223. May-Hegglin anomaly and pregnancy. A hypertensive patients with thrombocytopenia is reported who had two pregnancies complicated by preeclampsia and cesarean deliveries without hemorrhage. During her first pregnancy corticosteroids were given for presumed autoimmune thrombocytopenia. Thereafter she was splenectomised. Ten years later May-Hegglin anomaly and renal failure were diagnosed. One of her children had easy bruising. ( info) |
5/223. Sebastian platelet syndrome: two Japanese families originally diagnosed with May-Hegglin anomaly. Ultrastructural studies of granulocytes were performed on two unrelated patients with hereditary thrombocytopenia, giant platelets, and inclusion bodies in granulocytes. Each patient had been diagnosed with May-Hegglin anomaly. In both cases, inclusion bodies in granulocytes consisted of clusters of ribosomes and small segments of rough endoplasmic reticulum. Additional clinical features suggesting Alport syndrome were lacking in these propositi and their family members. These observations imply that the patients were affected not with May-Hegglin anomaly but with Sebastian platelet syndrome. They would thus represent the seventh and eighth families known to carry this hereditary disease. ( info) |
6/223. Acquired von Willebrand's syndrome and thrombopathy in a patient with chronic lymphocytic leukaemia. This paper reports the biological data found during an acquired bleeding disorder occurring in a 65-year-old woman affected with chronic lymphocytic leukaemia. They consists of haemostatic abnormalities which resemble an presently undescribed association of an acquired von Willebrand's syndrome with a thrombopathy. von Willebrand abnormalities were temporarily corrected by infusion of normal cryoprecipitate and reproduced in vitro by the incubation of normal platelets with the patient's IgA. The platelet defect was partially corrected after corticotherapy. The possible relationship of the associated defects is discussed. ( info) |
7/223. A pregnancy complicated with Fechtner syndrome: a case report. A 21-year-old woman was diagnosed with Fechtner syndrome at 15 weeks gestation. She had a familial history of this disorder; her mother, two siblings and maternal grandmother were also affected. She presented with neither bleeding from the genital tract nor symptoms suggestive of placental abruption. Labor progressed uneventfully and resulted in the birth of a healthy female infant weighing 3436 g at 41 weeks of gestation. The puerperium was uneventful for both mother and infant. ( info) |
8/223. Platelets from a patient heterozygous for the defect of P2CYC receptors for ADP have a secretion defect despite normal thromboxane a2 production and normal granule stores: further evidence that some cases of platelet 'primary secretion defect' are heterozygous for a defect of P2CYC receptors. Two unrelated patients with a congenital bleeding diathesis associated with a severe defect of the platelet ADP receptor coupled to adenylate cyclase (P2(CYC)) have been described so far. In one of them, platelet secretion was shown to be abnormal. We recently showed that platelets with the primary secretion defect (PSD; characterized by abnormal secretion but normal granule stores, thromboxane A(2) production, and ADP-induced primary wave of aggregation) have a moderate defect of P2(CYC). Therefore, the interaction of ADP with the full complement of its receptors seems to be essential for normal platelet secretion, and PSD patients may be heterozygotes for the congenital severe defect of P2(CYC). In this study, we describe 2 new related patients with a severe defect of P2(CYC) and the son of one of them, who is to be considered an obligate heterozygote for the defect. The 2 patients with the severe defect had lifelong histories of abnormal bleeding, prolonged bleeding times, abnormalities of platelet aggregation and secretion, lack of inhibition of adenylate cyclase by ADP, and a deficiency of platelet-binding sites for [(33)P]2 MeS-ADP (240 and 225 sites per platelet; normal range, 530 to 1102). The son of one of them had a mildly prolonged bleeding time and abnormalities of platelet aggregation and secretion similar to those found in patients with PSD. In addition, his platelets showed a moderate defect of binding sites for [(33)P]2 MeS-ADP (430 sites per platelet) and of adenylate cyclase inhibition by ADP. This study of a family with the platelet disorder characterized by a defect of the platelet P2(CYC) receptor supports our hypothesis that the full complement of the platelet ADP receptors is essential for normal platelet secretion and that some patients with the common, ill-defined diagnosis of PSD are actually heterozygous for the defect. ( info) |
9/223. Anesthetic implications of the grey platelet syndrome. PURPOSE: To describe the obstetrical anesthetic care provided to two sisters with a rare qualitative platelet disorder, the grey platelet syndrome (GPS). CLINICAL FEATURES: Both patients manifested thrombocytopenia prior to delivery without previous history of a bleeding diathesis or other abnormal laboratory tests of coagulation function. The first required emergency cesarean section due to fetal bradycardia. Due to the thrombocytopenia and the emergency nature of the procedure, general anesthesia was used. During the C-section, 1.5-2 litres of old blood was noted in the abdominal cavity which was attributed to an old splenic capsular tear of unknown etiology. work-up for the thrombocytopenia revealed large platelets on the peripheral smear with abnormal aggregation on platelet function studies. Electron microscopy of the platelets revealed absent alpha granules, diagnostic of GPS. The second patient, the sister of patient #1, presented in a similar fashion. However, at presentation, the platelet count was 112,000 x m(-3) and spinal anesthesia was provided without complication for Cesarean delivery. The same patient presented for a second delivery during which fetal bradycardia necessitated emergency C-section under general anesthesia. Despite administration of six units of platelets, blood loss was 5,200 mL. Her postpartum course was uncomplicated and she and the infant were discharged home on postoperative day #4. CONCLUSION: The primary concerns for the anesthesiologist looking after patients with qualitative platelet defects are related to defective coagulation which influences the need for perioperative replacement of blood products and limits the use of regional anesthesia. ( info) |
10/223. Surreptitious bleeding in surgery: a major challenge in coagulation. Apart from inadequate surgical haemostasis, postoperative bleeding can be related to acquired disorders of platelet number, platelet function or coagulation proteins (e.g. vitamin k deficiency, DIC or liver injury). We highlight our experience with three patients who suffered life-threatening bleeding in the postoperative setting. The three patients - a 47-year-old man and 70- and 74-year-old women -- all had negative histories for excessive bleeding with prior surgeries, and all had normal preoperative PT and aPTT tests. Surgeries were resection of ischaemic bowel, cholecystectomy and coronary artery bypass grafting. All patients experienced unexpected bleeding within the first few postoperative days requiring multiple red cell transfusions and surgical re-explorations. Evaluations within the first 4--7 days after surgery revealed that these three patients had developed prolonged aPTT due to demonstrable factor viii antibodies initially at low titre. One patient was treated with high doses human factor viii, corticosteroids, intravenous gammaglobulin and plasma exchanges. The inhibitor was no longer demonstrable after 6 weeks of such therapy, and he has remained in remission without therapy. The second patient was initially treated with high-dose human factor viii infusions. Five months later, prednisone and 6-mercaptopurine were begun for worsening inhibitor titre and diffuse purpura and subcutaneous haematomas. The factor inhibitor remitted, but the patient died from liver failure related to post-transfusion hepatitis. The third patient was initially managed with high-dose human factor viii. Two months later, worsening inhibitor titre and tongue haematoma was treated with activated prothrombin complex, corticosteroids and cyclophosphamide. Eight years later, she is on no therapy, demonstrates a mild bleeding tendency and has a stable low-titre inhibitor. There have been a few case reports of inhibitors to coagulation factors including factor viii becoming manifest in the postoperative setting but surgery has not been widely recognized as an underlying cause for acquired haemophilia. This paper speculates on pathogenesis and reviews treatment options. This syndrome is remarkable for its abrupt onset in the first few postoperative days and for its substantial morbidity. The problem is potentially reversible with immunosuppressive therapy. Clinicians should be aware of this syndrome, considering acquired haemophilia in patients with unexpected postoperative bleeding. ( info) |