State-of-the-Art Review: Vascular Thrombohemorrhagic Disorders: Hereditary and Acquired

Bick RL Hereditary and acquired vascular bleeding disorders [chapter 87]. In: Bick RL , Bennett JM , Brynes RK , eds. Hematology: Clinical and Laboratory Practice. St. Louis : Mosby Publishers; 1993:1325.

Bick RL Clinical evaluation of the patient with hemorrhage [chapter 86]. In: Bick RL , Bennett JM , Brynes RK , eds. Hematology: Clinical and Laboratory Practice. St. Louis : Mosby Publishers ; 1993:1317.

Bick RL Vascular disorders associated with thrombohemorrhagic phenomenon [chapter 3]. In: Bick R , ed. Disorders of Hemostasis and Thrombosis. New York: Thieme, Inc: 1985:44.

Bick RL Vascular bleeding disorders [chapter 3]. In: Bick R. ed. Disorders of Thrombosis and Hemostasis: Clinical and Laboratory Practice. Chicago: ASCP Press; 1992:35.

Alshameeri RS , Mammen, EF Clinical experience with the Thrombostat 4000 . Semin Thromb Hemost 1995;21:1.

Kundo SK Heilmann EJ , Sio R. , et al. Description of a in-vitro platelet function analyzer: PFA-100. Semin Thromb Hemost 1995;21: 106. .

Johnson SA , Falls EF Ehler-Danlos syndrome: A clinical and genetic study. Arch Dertmatol 1949;60:82.

Sucheti A. , Szemere J. , Bernstein B. , et al. Chronic pain is manifestation of the Ehlers-Danlos syndrome , J Pain Symptom Manage 1997; 14:88.

Robert; HR , Kroncke FG Tests of platelet activity: Application to clinical diagnosis. In: Brinkhous KM. Shermer RW , Mostofi FK , eds. The Platelet. Baltimore: Williams & Wilkins Co; 1971:365.

Dolan AL , Mishra MB , Chambers JB , et al. Clinical and echocardiographic survey of the Ehlers-Danlos syndrome . Br J Rheumatol 1997:36:459.

McIntosh LJ , Mallett VT , Frahm JD , et al. Gynecotogic disorders in women with Ehlers-Danlos syndrome. J Soc Gynecol Investig 1995;2:559.

Millar AJ Ghilbert RD , Brown, RA , et al. Abdominal aortic aneurysms in children. J Pediatr Surg 1996;31:1624.

Oyen O. , Clausen OP , Brekke IB , et al. Spontaneous rupture of the renal artery in a patient with the Ehlers-Danlos syndmme. Eur J Vasc Endovasc Surg 1997;13:509.

Schievink, WI Genetics of intracranial aneurysms. Neurosurgery 1997;40:651.

Sonesson B. , Hansen F. , Lanne T. The mechanical properties of elastic arteries in Ehlers-Danlos syndrome. Eur J Vasc Endovasc Surg 1997; 14:258.

Paradisi M. , Guibilie L. , Canzona F. , et al. Ehlers-Danlos syndrome type I: Ultrastructural study. Minerva Pediatr 1997;49: 215.

Greenspan DS , Northrup H. , Au KS , et al. COL5A1: Fine genetic mapping and exclusion as a candidate gene in families with nail-patella syndrome, tuberous sclerosis 1, hereditary hemorrhagic telangiectasia and Ehlers—Danlos syndrome type II. Genomics 1995;25;737.

Michalickova K. , Susic M. , Willing MC , et al. Mutations of the alpha2(V) chain of type V collagen impair matrix assembly and produce Ehlers-Danlos syndrome type I. Hum Mol Genet 1998; 7:249.

Wenstrup, RJ , Langiand, GT Willing, MC , et al, A splice-junction mutation in the region of COL5A1 that codes for the carboxyl propeptide of pro alpha 1(V) chains results in the gravis form of the Ehlers-Danlos syndrome (type 1). Hum Mol Genet 1996;5:1733.

De Paepe A , Nuytinck L. , Hausser I. , et al. Mutations in the COL5A gene are causal in the Ehlers-Danlos syndrome I and II, Am J Hum Genet 1997;60:547.

Angels CH , van Dongen P. Boers GH , et al. Ehlers-Danlos syndrome type IV: Phenotype variation. Ned Tijdschr Geneeskd 1997;141:296.

Eitchanittoff H. , Cribier A. , Letac B. Peripheral and coronary artery dissections in a young woman. A rare case of type IV Ehlers-Danlos syndrome. Arch Mal Coeur Vaiss 1997;90:841.

Gelbmann CM , Kollinger M. , Gmeinweiser J. , et al. Spontaneous rupture of liver in a patient with Ehlers-Danlos disease, type IV, Dig Dis Sci 1997;42:1724.

Lauwers G. , Nevelsteen A. , Daenen G. , et at. Ehters-Dantos syndrome type IV:, A heterogenous disease, Ann Vase Surg 1997;11: 178.

Schwarze U. , Goldstein JA , Byers PH Splicing defects in the COL3A gene: Marked preference for 5' (donor) splice-size mutations in patients with exon-skipping mutations and Ehlers-Dantos syndrome Type IV. Am J Hum Genet 1997;61:1276.

Liu X. , Wu H. , Byme M. , et al. Type III collagen is crucial for collagen fibrillogenesis and for normal cardiovascutar development. Proc Natl Accad Sci USA 1997;94:1852.

Anderson DW , Thakker-Varia S. , Tromp G. , et al. A glycine (415)-to-serine substitution results in impaired secretion and decreased thermal stability of type III procollagen in a patient with Ehlers-Dantos syndrome type IV. Hum Mutat 1997;9:62.

Smith LT , Schwarze U. , Goldstein J. , et al. Mutations in the COL3A gene result in the Ehlers-Danlos syndrome type IV and atterations in the size and distribution of the major collagen fibrils of the dermis. J Invest Dermatol 1997 ;108:241.

Pajunen L. , Soukas M. , Hautala T. , et at. A slice-site mutation that induces exon skipping and reduction in lysyl hydroxylase mRNA levels but does not create a nonsense codon in Ehlers-Danlos syndrome type IV. DNA Cell Biol 1998; 17:117.

Pousi B. , Hautata T. Hyland JC , et al. A compound heterozygote patient with Ehlers-Danlos syndrome type IV has a deletion in one allete and a splicing defect in the other allele of the lysyl hydroxylase gene. Hum Mutat 1998 ; 11:55.

Yeowell HN , Walker LC , Murad S. , et al. A common duplication in the lysyl hydroxylase gene of patients with Ehters-Dantos syndrome type IV results in preferential stimulation of lysyl hydroxytase activity and mRNA by hydralazine. Arch Biochem Biophys 1997;347:126.

Yeowell HN , Walker LC Ehlers-Danlos syndrome type IV results from a nonsense mutation and a splice-site-mediated exon-skipping mutation in the lysyl hydroxylase gene . Proc Assoc Am Physicians 1997;109:383.

Byers PH , Duvic M. Atkinson M , et al. Ehlers-Danlos syndrome type VIIA and VIIB result from splice-junction mutations or genomic deletions that involve exon 6 in the COL1A1 nd COL1A2 genes of type I collagen. Am J Med Genet 1997 ;72:94.

Kuivaniemi H. , Peltonen L. , Kivirikko KI Type IX Ehlers-Danlos syndrome and Menkes syndrome: The decrease in lysyl oxidase activity is associated with a corresponding deficiency in the enzyme protein. Am J Hum Genet 1985;37:798.

Stine KC , Becton DL DDAVP therapy controls bleeding in Ehlers-Danlos syndrome. J Pediatr Hematol Oncol 1997;19:156.

Anderson M. , Pratt-Thomas RH Marfan's syndrome. Am Heart J 1953;46:911.

Futcher PH , Southworth H. Arachnodactyly and its medical complications. Arch Intern Med 1938;61:693.

Toriello HV , Glover TW , Takahara K. , et al. A translocaction interrupts the COL5AI gene in a patient with Ehlers-Danlos syndrome and hypomelanosis of Ito. Nat Genet 1996;13:361.

Fleischer KJ , Nousari HC , Anhalt GJ , et al. Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan's syndrome . Ann Thorac Surg 1997;63:1012.

Reinhardt DP , Chalberg SC , Sakai LY The structure of function of fibrillin. Ciba Found Symp 1995;192:128.

Gillinov AM , Zehr KJ , Redmond JM , et al. Cardiac operations in children with Marfan's syndrome: Indications and results. Ann Thorac Surg 1997;64:1140.

Millar AJ Gilbert RD , Brown RA , et al. Abdominal aortic aneurysms in children. J Pediatr Surg 1996;31:1624.

Sekine S. , Abe T. , Kuribayashi R. , et al. Aortic root syndrome. Cardiovasc Surg 1996;4:635.

Lipscomb KJ , Smith JC , Clarke B. , et al. Outcome of pregnancy in women with Marfan' syndrome. Br J Obstet Gynaecol 1997 ; 104:201.

Dotrelova D. , Karel I. , Clupkova E. Retinal detachment in Marfan's syndrome: Characteristics and surgical results. Retina 1997; 17:390.

Schievink WI , Parisi JE , Piepgras DG , et al. Intracranial aneurysms in Marfan's syndrome: An autopsy study. Neurosargery 1997 ;41:866.

Sullivan CE Influence of maxillary constriction on nasal resistance and steep apnea severity in patients with Marfan's syndrome. Chest 1996;110:1184.

Cistulli PA , Sullivan CE Sleep apnea in Marfan's syndrome: Increased upper airway collapsibility during sleep. Chest 1995; 108:631.

Cole WG The molecular pathology of osteogenesis imperfecta. Clin Orthop 1997;343:235.

De Paepe A , Nuytinck L. , Raes M. , et al. Homozygosity by descent for a COL1A2 mutation in two sibs with severe osteogenesis imperfecta and mild clinical expression in the heterozygotes. Hum Genet 1997;99:478.

Lund AM , Nicholls AC , Schwartz M. , et al. Parenteral mosiacism and autosomal dominant mutations causing structural abnormalities of collagen I are frequent in families with osteogenesis imperfecta type III/IV. Acta Paediatr 1997 ;86:711.

Bouvier M. , Colson F. , Noel E. , et al. Two new case reports of reflex sympathetic dystrophy syndrome in patients with osteogenesis imperfecta: Review of the literature. Rev Rhum Engl Ed 1997;64:202.

Korkko J. Ala-Kokko L. , De Paepe A. , et al. Analysis of the COL1 A 1 and COL1A2 genes by PCR amplification and scanning by comformation-sensitive gel electrophoresis identifies only COLIAI mutations in 15 patients with osteogenesis imperfecta type I: Identification of common sequences of null-aliele mutations. Am J Hum Geatet 998:62:98.

Forlino A. , D'amato E. , Valli M. , et al, Phenotypic comparison of an osteogenesis imperfecta type IV proband with a de novo alpha2(I) Gly 922 to Ser substitution in type I collagen and an unrelated patient with an identical mutation. Biochem Mol Med 1997;62:26.

Williams CJ , Smith RA , Ball RJ , et al. Hypercalcaemia in osteogenesis imperfecta treated with pamidronate. Arch Dis Child 1997 :76:169.

Bembi B. , Parma A. , Bottega M. , et al. Intravenous pamidronate treatment in osteogenesis imperfecta . J Pediatr 1997;131:622.

Landsmeer-Beker EA , Massa GG , Maaswinkel-Mooy PD , et al. Treatment of osteogenesis imperfecta with the biphosphanate olpadronate (dimethylaminohydroxypropylidene bisphosphonate). Eur J Pediatr 1997; 156:792.

Albright JA , Miller EA Osteogenesis imperfecta. Clin Orthop 1981;159:2.

Myrmel T. , Christensen O. , Lunde P. Cardiac manifestations in osteogenesis imperfecta. A case report and therapeutic implications. Tidsskr Nor Laegeforen 1997; 117:519.

Gilbert GJ Spontaneous dissections. Neurology 1997 ;48:1475.

Seibel BM , Briedman IA , Schwartz SO Hemorrhagic disease in osteogenesis imperfecta: Studies of platelet function defect. Am J Med 1957;22:315.

Polimer IJ Pseudoxanthoma elasticum and gastrointestinal hemorrhage. J Maine Med Assoc 1967;58:76.

Struk B. , Neldner KH Rao VS , et al. Mapping of both autosomal recessive and dominant variants of pseudoxanthoma elasticum to chromosome 16p13:1. Hum Mol Genet 1997;6:1823.

Swart J. , Tijmes NT Proteoglycan alterations in skin fibroblast cultures from patients affected with pseudoxanthoma etasticum. Cell Biochem Funct 1996;14:111.

Piccoli A. , Ricciardi D. , Santucci A. , et at. Elevated levels of plasma endothelin-1, von Willebrand factor, and urinary albumin excretion in three relatives with pseudoxanthoma elasticum Thromb Haemost 1996;76:278.

Spinzi G. , Strocchi E. , Imperial G. , et al. Pseudoxanthoma elasticum: A rare cause of gastrointestinal bleeding . Am J Gastroenterol 1996,91:1631.

Fred HL , Hariharan R. Hematemesis in a woman with skin, eye, and heart abnormalities. Hosp Pract 1995:30:28.

Okamura T. , Yamamoto M. , Ohta K. , et al. A case of ruptured cerebral aneurysm associated with fenestrated vertebral artery in osteogenesis imperfecta. No Shinkei Geka 1995;23:451.

Cailleux N. , Hachulla E. , Perez-Cousin M. , et al. Pseudoxanthoma elasticrum: A rare cause of leg artery diseases in young adults. J Mal Vasc 1997;22:51:

Hirano T. , Hashimoto Y. , Kimura K. , et al. Lacunar brain infarction in patients with pseudoxanthoma elasticum . Rinsho Shinkeigaku 1996;36:633.

Gurwood AS , Mastrangelo DL Understanding angioid streaks. J Am Optom Assoc 1997:68:309.

Janotka H. , Hess J. , Wlodarczyk J. Angioid streaks. Pathogenesis and the clinical picture. Klin Oczna 1995:97:299.

Guba SC , Fink LM , Fonseca V. Hyperhomocystinemia: An emerging and important risk factor tor thromboembolic and cardiovascular disease. Am J Clin Pathol 1996;105:709.

Rees MM , Rodgers GM Homocystinemia: Association of a metabolic disorder with vascular disease and thrombosis. Thromb Res 1993;71:337.

Kang SS , Zhou J. , Wong Pwk , et al. Intermediate homocystinemia: A thermolabile variant of methylenetetrahydrofolate reductase. Am J Hum Genet 1988;43:414.

Frosst P. Blom HJ , Milos R. A candidate genetic risk factor for vascular disease: A common mutation in methylenetetrahydrofolate reductase . Nat Genet 10:111.

Mudd SH , Levy HL , Skovby F. Disorders of transsulfuration [chapter 23]. In: Schriver CR , Beaudet AL , Sly WS , et al, eds. The Metabolic Basis of Inherited Disease. New York: McGraw-Hill; 1995:1279.

Fenton WA , Rosenberg LE Inherited disorders of cobalamine transport and metabolism. In: Schriver CR , Beaudet AL , Sly WS , et al, eds. The Metabolic Basis of Inherited Disease. New York: McGraw-Hill; 1995:3129.

Valle D. Pai GS , Thomas GH , et al. Homocystinuria due to cystathionine beta-synthetase deficiency: Clinical manifestations and therapy. Johns Hopkins Med J 1980;146:110.

Boers Ghj , Smals AG , Trijbels FJ Heterozygosity for homocystinuria in premature peripheral and cerebral occlusive arterial disease. N Engl J Med 1985 ;313:709.

Carey MC Donovan DE , Fitzgerald O. , et al. Homocystinuria. I. A clinical and pathologic study of nine subjects in six families. Am J Med 1968;45:17.

Harker LA , Ross R. , Slichter SJ , et al. Homocysteine-induced arteriosclerosis: The role of endothelial cell injury and platelet response in its genesis. J Clin Invest 1976;58:731.

Di Minno G. , Davi G. Margaglione M. Abnormally high thromboxane biosynthesis in homozygous homocystinuria: evidence for involvement and probucol-sensitive mechanism. J Clin Invest 1993;92:1400.

Ratnoff OD Activation of Hageman factor by L-homocystine. Science 1968; 162:1007.

Rodgers GM , Conn MT Homocysteine: An atherogenic stimulus reduces protein C activation by arterial and venous endothelial cells. Blood 1990;75:895.

Lentz SR , Sadler JE , Inhibition of thrombomodulin surface expression and protein C activation by the thrombogenic agent bomocystine. J Clin Invest 1991;88:1906.

Harpel PC. Zhang X. , Borth W. Homocysteine and hemostasis: Pathogenetic mechanisms predisposing to thrombosis. J Nutr 1996;126:1285.

Anderson A. , Isaksson A. , Hultberg B. Homocysteine export from erythrocytes and its implication for plasma testing. Clin Chem 1992;38:1311.

Brenton DP , Cusworth DC , Gaull GE Homocystinuria: Metabolic studies on three patients. J Pediatr 1965;67:58.

Kottke-Marchant K. , Green R. , Jacobsen DJ , et al. High plasma homocysteine: A risk factor for arterial and venous thrombosis in patients with normal coagulation profiles . Clin Appl Thromb He-mast 1997;3:239.

Cocceri S. Palareti G. Premature vascular disease in homocystinemia . Haemostasis 1989;19:4.

Burbank MK , Spittell JA Tumor of Mood and lymph vessels [chapter 2]. In: Juergens JL , Spittell JA , Fairbairn JA , eds. Peripheral Vascular Diseases . Philadelphia: WB Saunders; 1980:679.

Takahashi T. , Katoh H. , Dohke M. , et al. A giant hepatic hemangioma with secondary portal hypertension: A case report of successful surgical treatment. Hepatogastroenterology 1997;44: 1212.

Greif F. Zifroni A. Madhala Cig. et al. Giant cavernous hemangioma of the liver. Harefuah 1996;131:471.

Allen PW , Enzinger FM Hemangiomata of skeletal muscle: Analysis of 89 cases. Cancer 1972;28:8.

Kasabach HH , Merritt KK Capillary hemangioma with extensive I purpura . Am J Dis Child 1940;59:1016.

Bick RL , Arun B. , Frenkel EP Disseminated intravascular coagulation: Clinical and pathophysiological mechanisms and manifestations. Haemonstasis 1999;29:111.

Valls C. Rene M. , Gil M. , et al. Giant cavernous hemangioma of the liven Atypical CT and MR findings . Eur Radiol 1996;6:448.

Edgerton MT The treatment of hemangiomas: With special reference to the role of steroid therapy. Ann Surg 1976 ; 183:517.

Castello MA , Ragni G. , Antimi A. , et al. Successful management with interferon alpha-2a after prednisone therapy failure in an infant with a giant cavernous hemangioma. Med Pediatr Oncol 1997;28:213.

Lobato R. , Martinez L. Leal N. , et al. Hemangiomas and vascular malformations. Review and update. Cir Pediatr 1997;10;119.

Bick RL , Pegram MD Giant cavernous hemangioma and deep vein thrombosis: A clinical syndrome . Blood 1991;78:493.

Hans FM Multiple hereditary telangiectasia causing hemorrhage (hereditary hemorrhagic telangiectasia). Bull Johns Hopkins Hosp 1909 ;20:63.

Osler W. On multiple hereditary telangiectasia with recurrent hemorrhages. Quart J Med 1907;:53.

Osier W. On telangiectasia circumscripta universalis. Bull Johns Hopkins Hosp 1901;12:33.

Plauchu H. , Bideau A. Epidemiologie et constitution d'un fegistre de population a propos d'une concentration geographique d'une maladie rare. Population 1984;4:765.

Vase P. , Grove O. Gastrointestinat lesions in hereditary hemorrhagic teiangiectasia . Gastroenterology 1986:91:1079.

Shovlin CL , Hughes Jmb , Tuddenham Egd. A gene for hereditary hemorrhagic telangiectasia maps to 9q33-34. Nat Genet 1994;6:197.

Yamashita H. , Ichijo H. , Grimsby S. Endogulin forms a heterodimeric complex with the signaling receptors for transforming growth factor-β J. J Biol Chem 1994;269:1995.

Marchuk DA The molecular genetics of hereditary hemorrhagic telangiectasia. Chest 1997; 111:79s.

Berg JN , Gallione CJ , Stenzel TT , et al. The activin receptor-like kinase 1 gene: Genomic structure and mutations in hereditary hemorrhagic telangiectasia type 2. Am J Hum Genet 1997;61:60.

Hashimoto K. , Pritzker M. Hereditary hemorrhagic telangiectasia : An electron microscope study. Oral Surg Oral Med Oral Pathol 1972;34:751.

Jahnke V. Ultrastructure of hereditary telangiectasia. Otolaryn— gology 1970;91:262.

Menefee MG , Plessa HC , Glueck HI Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease): An electron microscopic study of the vascular lesions before and after therapy with hormones. Arcli Otolaryngol 1975;101:246.

Berg JN , Guttmacher AE , Marchuk DA Clinical heterogeneity in hereditary hemorrhagic telangiectasia. J Med Genet 1996;33:256

Askin MP , Lewis BS Push enteroscopic cauterization: Long-term follow-up of 83 patients with bleeding small intestinal angiodysptasia Gastrointevt Etidosc 1996:43:580.

Velegrakis GA , Prokopakis EP , Papadakis CE , et al. Nd:YAG laser treatment of recurrent epistaxis in heredity hemorrhagic telangiectasia. J Otolaryngol 1997;26:384.

Shay SM Hereditary hemorrhagic telangiectasia: What the otolaryngologist should know . Am J. Rhinol 1997; 11:55.

Osler W. On a family form of recurrent epistaxis associated with telangiectasia of the skin and mucous membranes. Bull Johns Hopkins Hosp 1901 12:33,

Berry DL , DeLeon FD Endometrial ablation for severe menorrhagia in a patient with hereditary hemorrhagic telangiectasia A case report. J Reprod Med 1996;41:183.

Hodgsum CH , Kaye RL Pulmonary arteriovenous fistula and hereditary hemorrhagic telangiectasia. Dis Chest 1963 ;43:449.

Wang HC , Yang PC Kuo SH , et al, Pulmonary arteriovenous malformation: Analysis of 10 cases. J Formos Med Assoc 1998; 97:97.

Mccaughan JS Jr , Hawley PC , LaRosa JC , et at. Photodynamic therapy to control life-threatening hemorrhage from hereditary hemorrhagic telangiectasia. Lasers Sur Med 1996; 19:492.

Dutton JA , Jackson JE , Hughes JM , et al. Pulmonary arteriovenous malformations: Results of treatment with coil embolization in 53 patients. AJR Am J Roentgenol 1995; 165:1119.

Tripathy U. , Kaul S. , Bhosle K. , et al. Pulmonary arteriovenous fistula with cerebral arteriovenous malformation without hereditary hemorrhagic telangiectasia. Unusual case report and literature review. J Cardiovasc Surg 1997;38:677.

Buscurini E. Buscarini L. Dunesiao C. et al. Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: Doppler sonogruphic screening in a large family . J Hepatol 1997 ;26:111.

Mukusn C. Nakamura K. , Chijiiwa Y. et al. Liver failure caused by hepatic angiodysptasia in hereditary hemorrhagic telangiectasia, Am J Gastroenterol 1998;93:471.

Fitz-Hugh T. Sptenomegaty and hepatic enlargement in hereditary hemorrhagic tetangiectasia. Am J Med Sci 1931 ,181:261.

Press OW , Ramsey PG Central nervous system infections associated with hereditary hemorrhagic telangiectasia . Am J Med 1984;77:86.

Adams HP , Subbiah B. , Bosch EP Neurological manifestations of hereditary hemorrhagic telangiectasia . Arch Neurol 1977;34: 101.

Roman G. , Fisher M. , Pert DP , et al. Neurological manifestations of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease): Report of two cases and review of the literature . Ann Neurol 1978;4:130.

Baroudet S. Houdart E , Boissonnet H. , et al. Cerebromeningeal hemorrhages in Rendu-Osler disease. Two cases treated by embolization. Presse Medicale 1997;26:1622.

Weiner RL Gamma knife radiosurgery treatment of AVMs. Clin Appl Thromb Hemost 1995;1:179.

Bick RL , Fekete LF Hereditary hemorrhagic telangiectasia and associated defects in hemostasis . Blood 1978;52:179.

Quick AJ Telangiectasia. In: Quick AJ , ed. Hemorrhagic Diseases and Thrombavis. Philadelphia : Lea and Febiger; 1966:285.

McDervitt TJ , Toh AS Epistaxis: Management and prevention. Laryngoscope 1967 ;47:1109.

Ryan AJ Control of bleeding in familial telangiectasia. Merider , Hosp Bull 1958;7: 1.

Bick RL Hereditary hemorrhagic telangiectasia and disseminated intravascular coagulation: A new clinical syndrome. In: Walz DA, McCoy LE, eds. Contributions to Hemostasis . Ann NY Acad Sci 1981;370:851.

Steel D. , Bovill EG , Golden E. Hereditary hemorrhagic telangiectasia: A family study. Am J Clin Pathol 1988:90:274.

Bick RL Disseminated intravascular coagulationi Pathophysiological mechanisms and manifestations. Semin Thromb Hemost 1998 :24:3.

Stitch MH Carbazochrome salicylate therapy in hereditary hemorrhagic telangiectasia . NY State J Med 1959,59:2725.

Koch HJ , Escher GL , Lewis JS Hormonal management of hereditary hemorrhagic telangiectasia. JAMA 1952;149:1376.

Quick AJ Hemostasis then and now [chapter 1]. In: Quick AJ , ed. The Hemorrhagic Diseases and the Pathology of Hemostasis. Springfield, IL: Charles C. Thomas Publishers: 1974:3.

Lachner H. Hemostatic abnormalities associated with paraprotein abnormalities. Semin Hematol 1973;10:125.

Bick RL Alterations of hemostasis in malignancy [chapter 101]. In: Bick RL, Bennett JM, Brynes RK. Hematology,: Clinical and Laboratory Practice . St. Louis: Mosby Publishers ; 1993:1583.

Glaspy JA Disturbances in hemostasis in patients with B cell malignancies. Semin Thromb Hemost 1992;18:440.

Bick RL , Strauss JF , Frenket EP Thrombosis and hemorrhage in oncology patients. Hematol Oncol Clin North Am 1996;10:875,

Gallo GM , Picken M. , Buxbaum J. The spectrum of monoclonal immunoglobulin deposition disease associated with immunocytic disorders. Semin Hematol 1989;26:234.

Cawley LP , Minard BJ Amyloidosis [chapter 49]. In: Bick RL, Bennett JM, Brynes RK. Hematology. Clinical and Laboratory Practice. St. Louis : Mosby Publishers; 1993 :729.

Kunkel LA Acquired circulating anticoagulants. Hemeitol Oncol Clin North Am 1992;6:1341.

Triplett DA , Bang NU , Harms CS , et al. Mechanisms of acquired factor X deficiency in primary amyloidosis. Blood 1977 50:285.

152, Galbraith PA , Sharma N. Parker WL , et al. Acquired factor X deficiency. Altered plasma antithrombin activity and association with amyloidosis. JAMA 1974 ;230:1658.

Glenner GG Factor X deficiency and systemic nmytoidosis . N Engl J Med 1977;297:108.

Howell M. Acquired factor X deficiency associated with systemaiized amyloidosis: Report of a case. Blood 1963;21:739.

Krause JR Acquired factor X deficiency and amyloidosis. Am J Clin Pathol 1977;67:170.

McPherson RA , Onstad JW , Vgoretz RT , et al. Coagutopathy in amyloidosis: Combined deficiency of factor IX and X. Am J Hematol 1977:3:225.

Furie B. Green E. Furie BC Syndrome of acquired factor X deficiency and systemic amyloidosis . N Engl J Med 1977:297:81.

Stefanini M. , Mednicoff IB Demonstration of antivessel antibodies in serum of patients with anaphylactoid purpura and polyarteritis nodosa. J Clin Invest 1954;33:967.

Dixon FJ , Vazques JJ , Weigle WD , et al. Pathogenesis of serum sickness. Arch Pathol 1958;65:18.

Pierson KK Leukocytociastic vasculitis viewed as a phase of immune-mediated vasculopathy, Setitin Thromb Hemost 1984;10: 196.

Markowitz AS , Lang CF Streptococcal related glomerulonephritis . J Immunol 1964;92,565.

Cochrane CG Mediators of the arthus and related syndromes. Prog Allergy, 1967; 11: 1.

Freedman P. , Meister NP , Lee HJ , et al. The renal response to streptococcal infections. Medicine 1970 ;49:433.

Parish WE Studies on vasculitis: I. Immunoglobulins, beta-1-C, C-reactive protein, and bacterial antigens in cutaneous vasculitis lesions. Clin Allergy 1971;1:97.

165, Parish WE Studies on vasculitis: II. Some properties of complexes formed of antibacterial antibodies from persons with or without cutaneous vasculitis lesions. Clin Allergy 1971;1:111.

Tanaseanu S. , Purice S. The systemic vasculitides. Med Interna 1989;27:167.

Bigby M. , Stem RS , Arndt KA Allergic cutaneous reactions to drugs. Prim Care 1989;16:713.

Ashton N. The eye in malignant hypertension. Trans Am Acad Ophthalmol Otolaryngol 1972;76:17.

Farber EM , Hines EA , Montgomery H. , et al. Arterioles of skin in essential hypertension. J Invest Dermatol 1947;9:215.

Kazmier FJ , Didisheim P. , Fairbanks VF , et al. Intravascular coagulation and arterial disease. Thromb Diath Haemorrh 1969;36 (Suppl):295.

Ryan TJ The investigation of vasculitis. In: Ryan TJ , ed. Microvascular Injury. Philadelphia : WB Saunders; 1976:333,

Morris RN , Vassaill P. , Beller FK , et al. Immuno-fluorescent studies of renal biopsies in the diagnosis of toxemia of pregnancy. Obstet Gynecol 1964 ;24:32.

Robboy SJ , Mihm MC , Coleman RW , et at. The skin in disseminated intravascular coagulation: Prospective analysis of 36 cases. Br J Dermatol 1973;88:221.

Bick RL Disseminated intravascular coagulation: Objective clinical and laboratory diagnosis, treatment and assessment of therapeutic response. In: Weiss K. Sterz F, eds. Thrombosis and hemostasis in emergency medicine. Semin Thromb Hemost 1996; 22:69.

Bondy PK Disorders of the adrenal cortex [chapter 221. In: Wilson JD , Foster DW , eds. Textbook of Endocrinology. Philadelphia: WB Saunders; 1985:816.

Kaklamani VG , Vaiopoulos G. , Kaklamanis PG Behcet's disease. Semin Arthritis Rheum 1998;27:197.

Haim S. , Sobel JD Freidman-Bimbauin R. Thrombophlebitis and a cardinal symptom of Beheet's syndrome. Acta Derm Venereol 1974:54:299.

Lee SK , Lee J. Behcet's disease: A rheumatologic perspective. Yonsei Med J 1997;38:395.

Zierhut M. , Saal J. , Pleyer U. , et al. Beheet's disease: Epidemiology and eye manifestations in German and Mediterranean patients. German J Ophthalmol 1995;4:246.

Nazzarro P. Cutaneous manifestations of Behcet's disease: Clinical and histological findings. Proc Int Symp Behcets Disease, Karger, Basel; 1966:15.

Farah S. , Al-Shubaili A. , Montaser A. , et at. Beheet's syndrome: A report of 4 patients with emphasis on neurological manifestations. J Neurol Neurosurg Psychiatry 1998;64:382.

Bayraktar Y. , Balkanci F. , Bayraktar M. , et al. Budd-Chiari syndrome: A common complication of Behcet's disease. Ain J Gastroenterol 1997 ;92:858.

Roguin A. , Edetstein S. Edoute Y. Superior vena cava syndrome as a primary manifestation of Beheet's disease . Angiology 1997; 48:365.

Kroger K. , Ansasy M. Rudofsky G. Postoperative thrombosis of the superior eavai vein in a patient with primary asymptomatic Behcet's disease. A case report, Angiology 1997;48:649.

Han DS , Kim JB , Lee OY , et al. A case of Behcet's syndrome with superior vena cava syndrome. Korean J Intern Med 1998; 13:72.

Sagdic K. , Oxer ZG , Saba D. , et al. Venous lesions in Behcet's disease . Eur J Vasc Endovasc Surg 1996;11:437.

Lenk N. , Ozet G. , Alli N. , et al. Protein C and protein S activities in Behcet's disease as risk factors of thrombosis. lnt J Dermatol 1998 ;37:124.

Hills EA Behcet's syndrome with aortic aneurysms. Br Med J 1967;4:152.

Sakuri M. , Miyaji T. Behcet's disease from the point of view of vascular pathology. Folia Ophthalm Japan 197 1;22:903.

Chajek T. , Fainard M. Behcet's disease with decreased fibrinolysis and superior vena caval occlusion . Br Med J 1973; 1:782.

Jorizzo JL , Abernethy JL , White WL , et al. Mucocutaneous criteria for the diagnosis of Behcet's disease: An analysis of clinicopathologic data from multiple international centers. J Am Acad Dermatol 1995;32:968.

Mangetsdorf HC , White WL , Jorizzo JL Behcet's disease. Report of twenty-five patients from the United States with prominent mucocutaneous involvement. J Am Acad Dermatol 1996;34:745.

Dominguez LN , Irvine AR Fundus changes in Behcet's disease . Trans Am Ophthalmol Soc 1997;95:367.

Ciftci OU , Ozdemir O. Cataract extraction in Behcet's disease. Acta Ophihalmol Scand 1996;74:74.

Ohno E. , Ohtsuka E. , Watanabe K. , et al. Behcet's disease associated with myelodysplastic syndromes. A case report and a review of the literature, Cancer 1997;79:262.

Yano K. , Seguchi K. , Migita K. , et at. Behcet's disease complicated with myelodysplastic syndrome: A report of two cases and review of the literature. Clin Rheumarol 1996; 15:91.

Celik G. , Kalaycioglu O. , Durmax G. Colchicine in Behcet's disease with major vessel thrombosis. Rheumatol Int 1996;16:43.

Kaklamani VG Vaiopoulos G. , Kaklamanis PG Behcet's disease. Semin Arthritis Rheum 1998;27:197.

Tseng S. , Pak G. , Washenik K. et al. Rediscovering thulidomide: A review of its mechanism of action, side effects, and potential uses. J Am Acad Dermatol 1996;35:969.

Behrendt PM , Epstein SE , Marrow AG Postperfusion nonthrombocytopenic purpura: An uncommon sequel of open-heart surgery. Am J Cardiol 1968;22:631.

Bick RL Hemostasis defects in cardiac surgery and use of cardiac devices. Piffare R , ed. Cardiac Surgery: Principles and Practice. Philadelphia: Belfus ; 1999.

Calabrese LH , Michel BA , Bloch DA The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis . Arthritis Rheum 1990;33:1108.

Criep LH , Cohen CG Purpura as a manifestation of penicillin sensitivity. Ann Intern Med 1951;34:1219.

Nalbandian RM , Mader IJ , Barrett JL , et al. Petechiae, ecchymoses, and necrosis of skin induced by coumarin congeners, JAMA 1965; 192:603 )

Nalbandian RM , Beller FK , Hamp AK , et al. Coumarin necrosis of the skin treated successfully with heparin. Gynecology 1971 ; 38:395.

Nudetman HL , Kempson RL Necrosis of the breast; A rare complication of anticoagulant therapy. Am J Surg 1966;111:728

Cole MS , Minifee PK , Wolma FJ Coumarin necrosis: A review of the literature. Surgery 1988;103:271.

Becker CG Oral anticoagulant therapy and skin necrosis, In: Wessler S. Becker CG , Nemerson Y , eds. The New Dimensions of Warfarin Prophylaxis. New York: Plenum Press ; 1987:217.

Triplett DA Current recommendations for warfarin therapy: Use and monitoring. Med Clin North Am 1998 ;82:601.

Conrad J. , Horellou MH , van Dreden P. Homozygous protein C deficiency with late onset and recurrent coumarin-induced skin necrosis. Lancet 1992-.339:743.

Hyman BT Landas SK, Ashman RF. Warfarin-related purple toes syndrome and cholesterol microembolization. Am J Med 1987;82:1233,

Samama MM Laboratory control of anticoagulant therapy [chapter 103]. In: Bick RL , Bennett JM , Brynes RK , eds. Hematology: Clinical and Laboratory Practice. St. Louis : Mosby Publishers; 1993:729.

Francis RB Acquired purpura fulminans. Semin Thromb Hemost 1990;16:310.

Adcock DM , Bronza J. , Marler RA Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis. Semin Thromb Hemost 1990; 16:333.

Walenga J. , Bick RL Heparin associated thrombocytopenia and other adverse effects of heparin therapy. Cardiol Clin 1998;2:123.

Rodgers RP , Levin J. A critical reappraisal of the bleeding time . Semin Thromb Hemost 1990; 16:1.

Burns ER , Lawrence C. Bleeding time: A guide to its diagnostic and clinical utility. Arch Pathol Lab Med 1989; 113:1219.

Rodgers Rfc , Levin J. A critical reappraisal of the bleeding time . Semin Thromb Hemost 1990;16:1.

Bick RL , Adams T. , Schmalhorst WR Bleeding times, platelet adhesion, and aspirin. Am J Clin Pathot 1976;65:65.

Davis KA Complement, immune complexes and systemic lupus erythematosus. Br J Rhetimatol 1996;35:5.

Bentwich Z. , Beverley Pcl , Hammarstrom L. Laboratory investigations in clinical immunology: Methods, pitfalls, and clinical indications. Clin Immunol Immunopathol 1988;49:478.

Anderson CL , Stillman WS Raji cell assay for immune complexes evidence for detection of Rap-directed immunoglobulin G antibody in patients with systemic lupus erythematosus. J Clin Invest 1980;66:353.