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In patients with autoimmune diseases, the simultaneous occurrence of lupus anticoagulant and blood coagulation factors inhibitors is infrequent and is associated with hemorrhagic events. In these cases, the initial approach requires a thorough interpretation of coagulation laboratory tests and mixing studies to reach a definitive diagnosis. We report the case of a patient with systemic lupus erythematosus and Sjogren’s syndrome who presented with hemorrhagic diathesis caused by circulating inhibitors against factors VIII and XI coexisting with lupus anticoagulant. The inhibitors eradication was made with rituximab, achieving good results.

Coagulation factor XIII (F-XIII) deficiency is a rare disorder characterized by hemorrhagic diathesis, leading to defective wound healing and prolonged bleeding after trauma. This report describes tooth extraction in a 29-year-old man with F-XIII deficiency. A year earlier, he had experienced odontorrhagia and had been transported to hospital after extraction of the left mandibular third molar. Extensive blood examination was therefore performed this time, revealing F-XIII deficiency (activity level, 25%). Therefore, concentrated F-XIII was administered to increase F-XIII activity before extraction, increasing the F-XIII activity level to 61%. Teeth were then extracted under local anesthesia. Subsequent wound healing was favorable without odontorrhagia. In this time, we described the extremely rare report of F-XIII deficiency diagnosed by examinations immediately before dental treatments that included tooth extraction.

Sustaining hemostasis in clinical hemorrhages is a challenging task and requires extensive effort to stabilize medically hard-to-treat traumatic injuries. Several hemostatic agents are preferred to control external and internal bleedings, yet commercially available products are not sufficiently effective or fast-acting to achieve hemostasis in extreme cases. Ankaferd Blood Stopper (ABS) is a herbal extract traditionally used as a hemostatic agent. Recent studies have shown that ABS could be utilized successfully as a hemostatic agent for the management of clinical hemorrhages when conventional methods were ineffective. This review serves as a basis to provide recent findings on several applications of ABS, specifically preclinical, biological, and clinical studies both in vitro and in vivo. Another section focuses on the ultrastructural morphology and protein network formation of ABS in an effort to understand the hemostatic mechanisms of this unique agent at tissue level.

In 1955, animal studies suggested that thrombocytopenia was not the initiating cause of hemorrhage. Coagulation studies in vitro revealed the correction of coagulation by fresh platelets. Platelets, freshly collected with use of nonwettable surfaces, corrected thrombocytopenia, controlled associated hemorrhage, and prevented death from bleeding. Thus, in vitro and animal studies can be misleading (bench to bedside). Careful clinical observations, elaborated by in vitro studies, which create hypotheses testable in the clinic, lead to therapeutic advances (bedside to bench and back). Platelet replacement for thrombocytopenia prevents the hemorrhagic diathesis and has been universally practiced for over 50 years.

Resumen Objetivos La osteogenesis imperfecta es una enfermedad hereditaria con una baja prevalencia, pero gran impacto en la calidad de vida de los pacientes. Se presenta la experiencia en el manejo perioperatorio de estos pacientes en nuestro hospital. Pacientes y metodos Estudio descriptivo retrospectivo de las intervenciones realizadas en nuestro centro durante los anos 1991 a 2009, en los pacientes con esta enfermedad. Se analizan los datos demograficos, variante de la enfermedad, alteraciones asociadas, procedimientos quirurgicos, tipo de anestesia y complicaciones intraoperatorias y postoperatorias. Resultados Desde el ano 1991 hasta el 2009 se han realizado un total de 105 intervenciones en 29 pacientes con osteogenesis imperfecta (37,9% mujeres, 62,1% varones), con una edad que oscila entre 1 a 25 anos. El tipo mas frecuente de osteogenesis imperfecta fue el tipo III (65,5%). El 93% de los pacientes no presentaron enfermedades asociadas. Dos pacientes eran alergicos al latex. El 62% de las intervenciones transcurrieron sin complicaciones. Se registro un caso de hipertermia no maligna y una fractura de femur contralateral en el quirofano. Conclusiones La osteogenesis imperfecta es una enfermedad de baja prevalencia que precisa un tratamiento multidisciplinar. Es necesario un adecuado conocimiento de las alteraciones tanto esqueleticas como extraesqueleticas de esta enfermedad para un correcto tratamiento perioperatorio.

The current status of knowledge of the uremic hemorrhagic diathesis is reviewed. Discussions of clinical features, classification and pathogenesis, retention products and hemostatic defects, and therapy are included.

H emophilia is an X-linked hereditary hemorrhagic diathesis that is caused by a deficiency of either factor VIII or factor IX and is characterized by spontaneous or minimally provoked hemorrhage. Musculoskeletal manifestations of hemophilia include hemarthroses, peripheral neuropathies caused by nerve compression, and hemophilic pseudotumors of bone and muscle. We report an unusual case of multiple

Eight patients with spontaneous intracranial bleeding secondary to hemophilia A, afibrinogenemia, and thrombocytopenia are described. The clinical picture was that of increased intracranial pressure of gradual onset with variable signs and symptoms. Both the hemostatic defect and the intracranial bleeding were proved by laboratory investigations and CT scanning. Mortality was 75% (six of eight patients). The two remaining patients recovered completely following combined medical and surgical treatment, and are alive and well.

A case of multiple myeloma presenting as a hemorrhagic diathesis is reported. Heretofore, reports of bleeding disorders seen with multiple myeloma have been rare in the dental literature. The classic features of the disease are reviewed, as well as the mechanisms of associated hemorrhagic dyscrasias.

n -Butyl-cyanoacrylate is an effective hemostatic agent. Optimal efficiency depends on local application via a spray to achieve a thin and elastic film. Construction of a spray applicable to the oral cavity is illustrated using a plastic ampule with Histoakryl ® as reservoir. This spray has been used in 27 cases of prolonged bleeding to achieve hemostasis. In 18 cases the patients suffered from different hemorrhagic diatheses and general hemostatic procedures would normally have been necessary to achieve hemostasis. In nine patients without hemorrhagic diathesis, the cyanoacrylate spraying replaced more complicated local procedures. In 24 cases the cyanoacrylate spraying was a sufficient hemostatic treatment.

One of the most vital functions of the vertebrates is to maintain hemostasis through coagulation and fibrinolysis. This is achieved by the interaction of vascular walls with blood components such as cells and proteins (coagulation factors, immunoglobulins, and complement). Deficiency of coagulation factors results in bleeding disorders. The immune system, in response to foreign proteins or defective self proteins, produces antibodies triggering an Ag-Ab interaction with the related host of different reactions including abnormal coagulation leading to hemorrhagic diathesis.

Summary The results of the study of hemostasis in experimental hemoblastosis in monkeys were obtained on 108 animals (P. hamadryas and M. ardoides). 42 monkeys of the same species were used as controls. The changes in the blood coagulation system appeared early, preceding the development of clinical symptoms of the disease, and had the same trend in both monkey species. The deepness of alterations in blood, coagulation was aggravated in proportion to severeness of thesymptoms of the main process of hemoblastosis. Thrombocyte-megakaryocytograms of monkeys showed increased percentage of degenerative and involutive forms. The dynamic function of thrombocytes and general coagulologic tests were disturbed, prothrombin consumption was decreased, and fibrinolytic activity was increased. With the onset of clinical remission, hemostasis was not accompanied by full restoration of thrombocytogram and the coagulogram. Some experimental monkeys showed hemorrhagic diathesis, more often of the light type. The development of the haemorrhagic syndrome in the severe type was accompanied by more pronounced hemostatic changes coming to the functional alterations in thrombocytes and megakaryocytes, and also to changes in I and III stages of blood coagulation.

A 14-year-old Japanese boy suffering from Glanzmann's thrombasthenia required extraction of two permanent teeth and one primary tooth. This report deals with the details of the procedure and discusses the effect of platelet transfusion and local management in tooth extraction of patients with this hemorrhagic diathesis.

A carrier of hemophilia B was found to have an unusually low factor IX level of 13 per cent. Her history of previous bleeding and the hospital course following elective dental extractions were consistent with a mild hemorrhagic diathesis. The patient is a member of a rare kindred of hemophiliacs. The mean level of factor IX in 12 carriers in this kindred was 42 per cent, with a range of 13 to 100 per cent. This patient represents the sixth reported case in which a female carrier of factor IX deficiency was symptomatic.

A case of gingival hemorrhage secondary to uremia has been reported, and the unsuccessful attempt at local control has been described. The probable mechanism of the hemorrhagic diathesis of uremia has also been described. This bleeding tendency is due to an altered pathway of urea metabolism with consequently increased levels of GSA, which inhibits platelet function. In view of the increased number of persons with chronic renal disease who are surviving as a result of repeated dialyses on the artificial kidney, the dentist must be acutely aware of the possible consequences of uremia.

The acquired clotting defect of pregnancy associated with some degree of hypofibrinogenemia has been discussed and reviewed within the context of the controversies surrounding the various etiologic hypotheses. Additional types of cases have been presented in which this syndrome may be encountered. It is apparent that in certain of these patients the pathophysiology encompasses many variables and these must be recognized if the treatment is to be entirely effective.

A simple test requiring only a small amount of capillary blood was utilized in making multiple determinations of prothrombin values in newborn infants. The test as described is accurate enough for practical purposes. Treatment of expectant mothers with menadione (2-methyl-1, 4-naphthoquinone) during the last days of pregnancy or during labor virtually eliminated the hypoprothrombinemia and tendency toward pathologic bleeding as a result of hemorrhagic disease which otherwise may occur during the neonatal period. The incidence of retinal hemorrhages in the newborn was markedly reduced in infants of mothers treated with menadione during labor or prior to labor. Our results suggest that the reduction is greatest in the cases in which treatment is instituted before the onset of labor. These findings are of particular interest in their possible relationship to intracranial hemorrhage. The introduction of formula feedings during the first few days of life counteracted hypoprothrombinemia. The mechanism of this action is still not known. Three infants with hemorrhagic disease of the newborn were rapidly cured by administration of vitamin K. In two cases this was administered intramuscularly, and in one case by mouth.

Resume Les donnees experimentales et cliniques etablissent de facon formelle la superiorite des plaquettes fraiches sur le sang conserve dans le traitement des maladies plaquettaires. Des plaquettes viables peuvent etre apportees sous forme de sang frais, de plaquettes fraiches ou de plasma frais riche en plaquettes preleve par plasmapherese. Les indications du sang frais et plus exactement de plaquettes viables sont formelles : 1) en cas d'hemorragie importante. 2) lors de la periode pre et per-operatoire en cas de splenectomie. Les indications sont moins pressantes mais peuvent egalement etre posees en l'absence d'hemorragie dans les thrombopenies par aplasie medullaire aigue et, d'une facon generale, toutes les fois que le nombre des plaquettes est extremement bas.

Five patients with meningococcal sepsis who died with a rapidly progressing hemorrhagic diathesis secondary to intravascular consumption coagulopathy (IVCC) associated with irreversible shock are discussed in detail. Review of the microscopic pathology showed gram-negative diplococci within necrotic dermal endothelial cells, extensive necrosis of vascular endothelium without bacterial invasion in extradermal sites and free floating intraluminal fibrin-leukocyte-platelet clots. It is postulated that the meningococcal organism and especially the endotoxin it produces initiates accelerated intravascular clotting primarily by injurying the vascular endothelium. It is suggested that IVCC may be a frequent occurrence in meningococcemia and should be looked for in all patients with this infection since early diagnosis and initiation of proper therapy may prevent death secondary to fulminating hemorrhage and irreversible shock.

Ethynylestradiol (19-nor-17α-pregna-1,3,5,(10)-trien-20-yne-3,17-diol) was administered po to groups of 4 mature female beagle dogs for up to 95 days at levels of 0.04, 0.2, 1.0, or 5.0 mg/kg per day. 0.04 mg/kg induced persistent signs of estrus but had no hematologic effect. A dose-related neutrophil leukocytosis occurred at doses of 0.2 mg/kg and above, and there was a thrombocytopenia at 1.0 and 5.0 mg/kg. The latter was moderate and showed cyclic fluctuations at 1.0 mg/kg, but was marked at 5.0 mg/kg, resulting in a hemorrhagic diathesis. Terminal bone marrow samples from affected animals showed myeloid hyperplasia, depression of megakaryocytes with the appearance of abnormal forms and eventual overall depletion in the most severely affected dogs. Some depression of erythropoietic activity was also noted at 1.0 and 5.0 mg/kg.

Two unrelated female subjects with a hemorrhagic diathesis associated with pure AHGdeficiency are presented. The father of one girl has classical hemophilia; the mother of the other is a “carrier”. Pedigree analyses indicate no reason for believing that either of these girls has a genetic constitution other than heterozygous for the hemophilia gene von Willebrand's disease was excluded by appropriate laboratory studies. Possible genetic mechanisms which might have resulted in severe AHG deficiency in these patients are discussed.

Two cases of severe abruptio placentae have been presented. In each case, a hemorrhagic diathesis was evident and fibrin breakdown products were demonstrated qualitatively and quantitatively in the serum. The role of these products in interfering with platelet function is suggested as being important in the development of the syndrome. These findings suggest that the hemorrhagic diathesis associated with abruptio placentae is the result of abnormalities in both blood coagulation and platelet function.

INTRODUCTION IN YOUNG birds there are many hemorrhagic conditions with poorly understood mechanisms. In addition to hemorrhages associated with infectious diseases (fowl-pest, Newcastle disease, fowl-cholera, septicemia, blue-comb disease . . . ), parasites (coccidiosis) and intoxication [heavy metals, fungi, soybean oil meal extracted with trichloroethylene (Eveleth and Goldsby, 1953) and underheated soybean oil meal, (Balloun and Johnson, 1953)], there are two types of hemorrhages having a non-precise etiology. The first is characterised by profuse hemorrhages with considerable increase in the blood clotting time and “quick” time test (Goldhaft and Wernicoff, 1954; Cover et al., 1955). This condition is considered as being due to a vitamin K deficiency. The second is a syndrome characterised by numerous, small, localised hemorrhagic lesions, normal prothrombin-time (Wartell, 1957), and hemogram modifications due to reticulohistiocytary lesions. Until now, the cause of this condition has been unknown. Observation of cases of hemorrhagic diathesis of this second category . . .

I N 1940 Lozner, Jolliffe and Taylor’ presented the first documented case of a hemorrhagic diathesis associated with a circulating anticoagulant. Hematologically, the most prominent feature of the case was the presence of a prolonged coagulation time. Since that initial report a total of thirteen cases have been described in the literature. Conley2 and Dieter3 each presented a case of prolonged clotting time associated with a circulating anticoagulant arising spontaneously in a non-hemophiliac male. Munro4 and Van Creveld6 reported the phenomenon of the failure of hemophiliac patients to respond to fresh blood or plasma transfusions. All of these patients had responded to previous therapy with a marked reduction in the clotting time. Hewlett and Haden reported two females, seven months and one year postpartum, respectively, with a prolonged coagulation time and a circulating anticoagulant, neither patient having received blood transfusions previously. Dreskin and Rosenthal’ reported a similar experience in a thirty year old female seven weeks postpartum. In all cases the coagulation time was significantly prolonged and an anticoagulant was identified. These cases may be divided into three categories; (1) cases in which the anticoagulant arose de novo; (2) those which occurred in the postpartum state and (3) those which developed in patients who had previously received multiple whole blood transfusions. We are presenting the case of a twenty-seven year old female who, three months postpartum, developed her first manifestations of a bleeding tendency. This subsequently became severe and the patient had a massive gastrointestinal hemorrhage. Hematologic studies revealed a markedly prolonged clotting time associated with the presence of a circulating anticoagulant.

Prolonged bleeding time and alteration in clotting factors were observed in a patient with polycystic renal disease and cerebral artery aneurysm who received pharmacological doses of dextran-40. It is suggested that these abnormalities may have been aggravated by selective retention of the higher mo

T H E multitude of observations67 ‘1 25 concerning abnormal hemorrhagic states during pregnancy usually refers to the syndrome of afibrinogenemia or hypofibrinogenemia. The pathogenetic concept of a defibrinating syndromez5 in which fibrinogen is consumed as the resuit of the autoextraction of thromboplastin has been substantiated by a variety of experimental observations, but the part which fibrinolysis plays in such cases remains open to discussion. This is partly because, in the cases observed to date, exact determinations of fibrinolytic activity have not been made. In only a few cases has blood been taken and investigated at frequent intervals in the acute phase, In addition, clinical determination of fibrinolysis is not yet satisfactory and particularly the socalled rapid methods (clot observation test and others)16 give no indication of any associated fibrinolytic activity.23 Therefore, whether, and to what extent, fibrinolysis is a reactive process to the pathologic fibrin deposition or whether it is primarily of pathogenetic importance in hemorrhagic diathesis associated with pregnancy remains an open question. Clinical and analytical findings, in a case of abnormal obstetric bleeding recently ob

1. Blood plasma fibrinogen determinations were performed on ten selected women having abruptio placentae, the specimens being taken before, during, and/or following delivery. These studies were made in order to evaluate the potentiality for development, in these cases, of a hemorrhagic diathesis state. 2. The two mild cases showed no defibrination. The four moderately severe cases showed varying degrees of defibrination, from mild to severe, the two with fibrinogen levels lower than 90 mg. per cent developing hemorrhagic diatheses. In all of the four severe cases there was marked defibrination and in three of them abnormally great tendencies to bleed were present. 3. Since the defibrination tends to continue until delivery, and to clear up following delivery, it must be caused by the abruption state, since delivery chiefly accomplishes abolition of this state. These studies also demonstrated that the defibrination state may continue or grow worse in some of the moderately severe cases as well as in the severe ones, and that it is therefore wise to hasten their delivery if need be. 4. The evidence that the hemorrhagic state occasionally occurring in cases of abruptio placentae is due principally to depletion of the fibrinogen is further substantiated by our findings. Dangerous degrees of defibrination can occur within one hour following the occurrence of abruption. 5. These patients were all successfully treated with blood transfusion, given as quickly as needed and in amounts sufficient to replace the blood loss and to help restore whatever disruption of the blood coagulation mechanism might have occurred. 6. The second part of their treatment was termination of the abruption state through delivery. The obstetrician must see that this is accomplished before there is depletion of the patient's blood coagulation mechanism to an irretrievably low degree. If the apoplectic, overdistended uterus in a severe case is unable to go into labor so that it can deliver itself of its pregnancy, a section should be performed. The section should be commenced, in the severe cases, just as soon as there is some bettering of the patient's condition by rapid blood transfusion.

1. 1. Bleeding diathesis occurred in six of twelve animals after addition of a donor liver and its cause is probably related to implantation of the donor liver. 2. 2. Measurable levels of endogenous heparin were found in six of twelve animals after liver allografts. Five of the six animals had a hemorrhagic diathesis. 3. 3. Removal of the host liver had no influence on the appearance of a bleeding diathesis, active fibrinolysin, or endogenous heparin. 4. 4. Endogenous heparin may contribute to the hemorrhagic diathesis. 5. 5. Heparin was found three days after a liver allograft in one animal. That animal did not have bleeding diathesis. 6. 6. These observations may be another manifestation of the immune reaction in liver homotransplantation.

A case of Von Willebrand's disease in a sixty-two year old man is reported. The major feature of this patient's hemorrhagic diathesis was recurrent gastrointestinal bleeding. The diagnosis of Von Willebrand's disease is discussed, and the literature concerning gastrointestinal bleeding in this disease is briefly reviewed.