Skip to main content

Disorders of Hemostasis

  • Chapter
Dermatology

Abstract

Hemostasis is a complex problem. Endothelial cells, megakaryocytes and platelets, clotting factors, fibrinolysis components and their inhibitors are all intertwined in a system designed to rapidly seal off vessel damage without producing extensive clotting or occlusion. Normally, there is a delicate balance between the factors favoring hemostasis and those opposing it. If the hemostatic system is inappropriately activated, a thrombosis results. If on the other hand the system fails to function effectively, hemorrhage occurs. Since the skin is often the first site where hemorrhage is seen, dermatologists must have a basic understanding of hemostasis. We have deliberately simplified this chapter, leaving out rare disorders and only touching on the basic scientific aspects of platelet function, the coagulation cascade, and fibrinolysis.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 74.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Bibliography

General

  • Baselga E, Drolet BA, Esterly NB (1997) Purpura in infants and children. J Am Acad Dermatol 37:673–705

    Article  PubMed  CAS  Google Scholar 

  • Hirsh J, Weitz JI (1999) New antithrombotic agents. Lancet 353:1431–1436

    Article  PubMed  CAS  Google Scholar 

  • Kottke Marchant K (1994) Laboratory diagnosis of hemorrhagic and thrombotic disorders. Hematol Oncol Clin North Am 8:809–853

    Google Scholar 

  • Manco-Johnson MJ (1997) Disorders of hemostasis in childhood: risk factors for venous thromboembolism. Thromb Haemost 78:710–714

    PubMed  CAS  Google Scholar 

  • Piette WW (1994) The differential diagnosis of purpura from morphlogic perspective. Adv Dermatol 9:3–9

    PubMed  CAS  Google Scholar 

  • Shopnick RI, Brettler DB (1996) Hemostasis: a practical review of conservative and operative care. Clin Orthop 328:34–38

    Article  PubMed  Google Scholar 

Platelet-Related Hemostatic Diseases

  • Blanchette VS, Rand ML (1997) Platelet disorders in newborn infants: diagnosis and management. Semin Perinatol 21:53–62

    Article  PubMed  CAS  Google Scholar 

  • Rodgers GM (1999) Overview of platelet physiology and laboratory evaluation of platelet function. Clin Obstet Gynecol 42:349–359

    Article  PubMed  CAS  Google Scholar 

Wiskott-Aldrich Syndrome

  • Aldrich RA, Steinberg AG, Campbell DC (1954) Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea. Pediatrics 13:133–139

    PubMed  CAS  Google Scholar 

  • Haddad E, Cramer E, Riviere C et al. (1999) The thrombocytopenia of Wiskott Aldrich syndrome is not related to a defect in proplatelet formation. Blood 94:508–518

    Google Scholar 

  • Kuska B (1996) Wiskott-Aldrich syndrome is a “wonderful mystery”. J Natl Cancer Inst 88:1258–1261

    Article  PubMed  CAS  Google Scholar 

  • Ochs HD (1998) The Wiskott-Aldrich syndrome. Semin Hematol 35:332–345

    PubMed  CAS  Google Scholar 

  • Wiskott A (1937) Familiärer angeborener Morbus Werlhoffi? Mschr Kinderh 68:212–216

    Google Scholar 

Thrombotic Thrombocytopenic Purpura

  • Chen DK, Kim JS, Sutton DM (1999) Thrombotic thrombocytopenic purpura associated with ticlopidine use: a report of 3 cases and review of the literature. Arch Intern Med 159:311–314

    Article  PubMed  CAS  Google Scholar 

  • George IN, Gilcher RO, Smith IW et al. (1998) Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: diagnosis and management. J Clin Apheresis 13:120–125

    Article  PubMed  CAS  Google Scholar 

  • Hayward CP, Sutton DM, Carter WH et al. (1994) Treatment outcomes in patients with adult thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Arch Intern Med 154:982–987

    Article  PubMed  CAS  Google Scholar 

  • Moake J (1998) Moschcowitz, multimers and metallopro-tease. N Engl J Med 339:1629–1631

    Article  PubMed  CAS  Google Scholar 

  • Musio F, Bohen EM, Yuan CM et al. (1998) Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum 28:1–19

    Article  PubMed  CAS  Google Scholar 

Hemolytic-Uremic Syndrome

  • Gasser C et al. (1955) Hämolytisch-urämische Syndrome: Bilaterale Nierenrindennekrosen bein akuten erworbenen hämolytischen Anämien. Schweiz Med Wschr 85:905–909

    PubMed  CAS  Google Scholar 

  • Siegler RL (1995) Hemolytic uremic syndrome in children. Curr Opin Pediatr 7:159–163

    Article  PubMed  CAS  Google Scholar 

  • Stewart CL, Tina LU (1993) Hemolytic uremic syndrome. Pediatr Rev 14:218–224

    Article  PubMed  CAS  Google Scholar 

Immune-Mediated Acquired Thrombocytopenia

  • Johnson JR, Samuels P (1999) Review of autoimmune thrombocytopenia: pathogenesis, diagnosis, and management in pregnancy. Clin Obstet Gynecol 42:317–326

    Article  PubMed  CAS  Google Scholar 

  • Kiefel V, Freitag E, Kroll H et al. (1996) Platelet autoantibodies (IgG, IgM, IgA) against glycoproteins IIb/IIa and Ib/IX in patients with thrombocytopenia. Ann Hematol 72:280–285

    Article  PubMed  CAS  Google Scholar 

  • Pulkrabek SM (1996) Platelet antibodies. Clin Lab Med 16:817–835

    PubMed  CAS  Google Scholar 

Idiopathic Thrombocytopenic Purpura

  • George JN, Woolf SH, Raskob GE (1998) Idiopathic thrombocytopenic purpura: a guideline for diagnosis and management of children and adults. American Society of Hematology. Ann Med 30:38–44

    Article  PubMed  CAS  Google Scholar 

  • Karpatkin S (1997) Autoimmune (idiopathic) thrombocytopenic purpura. Lancet 349:1531–1536

    Article  PubMed  CAS  Google Scholar 

  • Law C, Marcaccio M, Tarn P et al. (1997) High-dose intravenous immune globulin and the response to splenectomy in patients with idiopathic thrombocytopenic purpura. N Engl J Med 336:1494–1498

    Article  PubMed  CAS  Google Scholar 

Secondary Immune-Mediated Thrombocytopenia

  • Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V et al. (1997) Successful therapy with danazol in refractory autoimmune thrombocytopenia associated with rheumatic diseases. Br J Rheumatol 36:1095–1099

    Article  PubMed  CAS  Google Scholar 

  • Boumpas DT, Austin HA, Fessier BJ et al. (1995) Systemic lupus erythematosus: emerging concepts, part 1: renal, neuropsychiatrie, cardiovascular, pulmonary, and hematologic disease. Ann Intern Med 122:940–950

    PubMed  CAS  Google Scholar 

  • Diehl LF, Ketchum LH (1998) Autoimmune disease and chronic lymphocytic leukemia: autoimmune hemolytic anemia, pure red cell aplasia, and autoimmune thrombocytopenia. Semin Oncol 25:80–97

    PubMed  CAS  Google Scholar 

  • Yashiro M, Nagoshi H, Kasuga Y et al. (1996) Evans syndrome associated with Graves’ disease. Intern Med 35:987–990

    Article  PubMed  CAS  Google Scholar 

HIV-Associated Immune Thrombocytopenia

  • Fabris F, Sgarabotto D, Zanon E et al. (1993) The effect of a single course of alpha-2B-interferon in patients with HIV-related and chronic idiopathic immune thrombocytopenia. Autoimmunity 14:175–179

    Article  PubMed  CAS  Google Scholar 

  • Lord RV, Coleman MJ, Milliken ST (1998) Splenectomy for HIV-related immune thrombocytopenia: comparison with results of splenectomy for non-HIV immune thrombocytopenic purpura. Arch Surg 133:205–210

    Article  PubMed  CAS  Google Scholar 

  • Louache F, Vainchenker W (1994) Thrombocytopenia in HIV infection. Curr Opin Hematol 1:369–372

    PubMed  CAS  Google Scholar 

Infection-Related Thrombocytopenia

  • Al-Majed SA, al-Momen AK, al-Kassimi FA et al. (1995) Tuberculosis presenting as immune thrombocytopenic purpura. Acta Haematol 94:135–138

    Article  PubMed  CAS  Google Scholar 

  • Lotric-Furlan S, Strie F (1995) Thrombocytopenia — a common finding in the initial phase of tick-borne encephalitis. Infection 23:203–206

    Article  PubMed  CAS  Google Scholar 

  • Mayer JL, Beardsley DS (1996) Varicella-associated thrombocytopenia: autoantibodies against platelet surface glycoprotein V. Pediatr Res 40:615–619

    Article  PubMed  CAS  Google Scholar 

  • Yamaguchi S, Kubota T, Yamagishi T et al. (1997) Severe thrombocytopenia suggesting immunological mechanisms in two cases of vivax malaria. Am J Hematol 56:183–186

    Article  PubMed  CAS  Google Scholar 

Medication-Related Immune Thrombocytopenia

  • Aster RH (1999) Drug-induced immune thrombocytopenia: an overview of pathogenesis. Semin Hematol 36:2–6

    PubMed  CAS  Google Scholar 

  • Kelton JG (1998) Dealing with drug-induced thrombocytopenia. Hosp Pract Off Ed 15:37–48

    Article  Google Scholar 

Heparin-Associated Thrombocytopenia

  • Demasi R, Bode AP, Knupp C et al. (1994) Heparin-induced thrombocytopenia. Am Surg 60:26–29

    PubMed  CAS  Google Scholar 

  • Gross AS, Thompson FL, Arzubiaga MC et al. (1993) Heparin-associated thrombocytopenia and thrombosis (HATT) presenting with livedo reticularis. Int J Dermatol 32:276–279

    Article  PubMed  CAS  Google Scholar 

  • Walenga JM, Bick RL (1998) Heparin-induced thrombocytopenia, paradoxical thromboemboHsm, and other side effects of heparin therapy. Med Clin North Am 82:635–658

    Article  PubMed  CAS  Google Scholar 

  • Warkenin TE, Levine MN, Hirsh J et al. (1995) Heparin-induced thrombocytopenia in patients treated with low-molecular-weight heparin of unfractionated heparin. N Engl J Med 332:1330–1335

    Article  Google Scholar 

Thrombocythemia

  • Barbui T, Finazzi G (1999) Clinical parameters for determining when and when not to treat essential thrombocythemia. Semin Hematol 36:14–18

    PubMed  CAS  Google Scholar 

  • Griesshammer M, Bangerter M, Sauer T et al. (1999) Aetiology and clinical significance of thrombocytosis: analysis of 732 patients with an elevated platelet count. J Intern Med 245:295–300

    Article  PubMed  CAS  Google Scholar 

  • Kutti J, Wadenvik H (1996) Diagnostic and differential criteria of essential thrombocythemia and reactive thrombocytosis. Leuk Lymph 22:41–45

    Article  Google Scholar 

  • Murphy S, Peterson P, Hand H et al. (1997) Experience of the Polycythemia Vera Study Group with essential thrombocythemia: a final report on diagnostic criteria, survival, and leukemic transition by treatment. Semin Hematol 34:29–39

    PubMed  CAS  Google Scholar 

  • Tefferi A, Hoagland HC (1994) Issues in the diagnosis and management of essential thrombocythemia. Mayo Clin Proc 69:651–655 Vora AJ, Lilleyman JS (1993) Secondary thrombocytosis. Arch Dis Child 68:88–90

    Google Scholar 

  • Yohannan MD, Higgy KE, al Mashhadani SA et al. (1994) Thrombocytosis. Etiologic analysis of 663 patients. Clin Pediatr Phila 33:340–343

    Article  PubMed  CAS  Google Scholar 

Pseudo-von Willebrand Syndrome

  • Kunishima S, Heaton DC, Naoe T (1997) De novo mutation of the platelet glycoprotein lb alpha gene in a patient with pseudo-von Willebrand disease. Blood Coagul Fibrinolysis 8:311–315

    Article  PubMed  CAS  Google Scholar 

  • Marchese P, Saldivar E, Ware J et al. (1999) Adhesive properties of the isolated amino-terminal domain of platelet glycoprotein Ib alpha in a flow field. Proc Natl Acad Sci USA 961:7837–7842

    Article  Google Scholar 

Disorders of Coagulation

Hemophilia

Von Willebrand Disease

  • Lethagen SR (1995) Pathogenesis, clinical picture and treatment of von Willebrand’s disease. Ann Med 27:641–651

    Article  PubMed  CAS  Google Scholar 

  • Rinder MR, Richard RE, Rinder HM (1997) Acquired von Willebrand’s disease: a concise review. Am J Hematol 54:139–145

    Article  PubMed  CAS  Google Scholar 

Hepatic Defects

  • Mammen EF (1994) Coagulation defects in liver disease. Med Clin North Am 78:545–554

    PubMed  CAS  Google Scholar 

  • Rereira SP, Langley PG, Williams R (1996) The management of abnormalities of hemostasis in acute liver failure. Semin Liver Dis 16:403–414

    Article  Google Scholar 

Vitamin K Deficiency

Gammopathies

  • Appenzeller P, Leith CP, Foucar K et al. (1999) Cutaneous Waldenstrom macroglobulinemia in transformation. Am J Dermatopathol 21:151–155

    Article  PubMed  CAS  Google Scholar 

  • Finder KA, McCollough ML, Dixon SL et al. (1990) Hyper-gammaglobulinemic purpura of Waldenstrom. J Am Acad Dermatol 23:669–676

    Article  PubMed  CAS  Google Scholar 

  • Kaneko Y, Murakami H, Matsushima T et al. (1993) Idiopathic thrombocytopenic purpura and monoclonal gammopathy of undetermined significance accompanied by urinary bladder carcinoma. Clin Exp Rheumatol 11:217

    PubMed  CAS  Google Scholar 

  • Keren DF (1993) Coagulation disorders in patients with monoclonal gammopathies. Hematol Oncol Clin North Am 7:1153–1159

    PubMed  CAS  Google Scholar 

  • Miyagawa S, Fukumoto T, Kanauchi M et al. (1996) Hyper-gammaglobuliaemic purpura of Waldenstrom and Ro/SSA autoantibodies. Br J Dermatol 134:919–923

    Article  PubMed  CAS  Google Scholar 

  • Robert F, Mignucci M, McCurdy SA et al. (1993) Hemostatic abnormalities associated with monoclonal gammopathies. Am J Med Sci 306:359–366

    Article  PubMed  CAS  Google Scholar 

  • Torok L, Borka I, Szabo G (1993) Waldenstrom’s macro-globulinaemia presenting with cold urticaria and cold purpura. Clin Exp Dermatol 18:277–279

    Article  PubMed  CAS  Google Scholar 

Disseminated Intravascular Coagulation

  • Baglin T (1996) Disseminated intravascular coagulation: diagnosis and treatment. BMJ 312:683–687

    Article  PubMed  CAS  Google Scholar 

  • Barraud S (1904) Über extremitätengangrän im jugendlichen Alter nach Infektionskrankheiten. Deut Zschr Chir 74:237–297

    Article  Google Scholar 

  • Bick RL (1994) Disseminated intravascular coagulation. Objective laboratory diagnostic criteria and guidelines for management. Clin Lab Med 14:729–768

    PubMed  CAS  Google Scholar 

  • Hardaway RM, Williams CH (1996) Disseminated intravascular coagulation: an update. Compr Ther 22:737–743

    PubMed  CAS  Google Scholar 

  • Levi M, Ten-Cate H (1999) Disseminated intravascular coagulation. N Engl J Med 341:586–592

    Article  PubMed  CAS  Google Scholar 

Purpura Fulminans

  • Bergmann F, Hoyer PF, D’Angelo SV et al. (1995) Severe autoimmune protein S deficiency in a boy with idiopathic purpura fulminans. Br J Haematol 89:610–614

    Article  PubMed  CAS  Google Scholar 

  • Darmstadt GL (1998) Acute infectious purpura fulminans: pathogenesis and medical management. Pediatr Dermatol 15:169–183

    Article  PubMed  CAS  Google Scholar 

  • Kreuz W, Veldman A, Escuriola-Ettinghausen C et al. (1998) Protein C concentrate for meningococcal purpura fulminans. Lancet 351:986–987

    PubMed  CAS  Google Scholar 

  • Smith OP, White B (1999) Infectious purpura fulminans: diagnosis and treatment. Br J Haematol 104:202–207

    Article  PubMed  CAS  Google Scholar 

Waterhouse-Friderichsen Syndrome

  • Locker GJ, Wagner A, Peter A et al. (1995) Lethal Waterhouse-Friderichsen syndrome in posttraumatic asplenia. J Trauma 39:784–786

    Article  PubMed  CAS  Google Scholar 

  • Ryan CA, Wenman W, Henningsen C et al. (1993) Fatal childhood Waterhouse-Friderichsen syndrome. Pediatr Infect Dis J 12:250–251

    Article  PubMed  CAS  Google Scholar 

  • Varon J, Chen K, Sternbach GL (1998) Rupert Waterhouse and Carl Friderichsen: adrenal apoplexy. J Emerg Med 16:643–647

    Article  PubMed  CAS  Google Scholar 

Kasabach-Merritt Syndrome

  • Enjolras O, Wassef M, Mazoyer E et al. (1997) Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr 130:631–640

    Article  PubMed  CAS  Google Scholar 

  • Ettlinger JJ, Fleming PJ, Joffe HS et al. (1996) Cavernous haemangioma with Kasabach-Merritt syndrome: treatment with alpha-interferon. J R Soc Med 89:55–56

    Google Scholar 

  • Schulz AS, Urban J, Gessler P et al. (1999) Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomtosis of spleen and pancreas. Eur J Pediatr 158:379–383

    Article  PubMed  CAS  Google Scholar 

  • Velin P, Dupont D, Golkar A et al. (1998) Neonatal Kasabach-Merritt syndrome healed by complete surgical excision of the angioma. Arch Pediatr 5:295–297

    Article  PubMed  CAS  Google Scholar 

Senile Purpura

Factitial Purpura

  • Shelley ED, Shelley WB, Talanin NY (1997) The mysterious case of the premenstrual purple chin. Cutis 60:147–149

    PubMed  CAS  Google Scholar 

Paroxysmal Finger Hematoma

Pigmented Purpuric Dermatoses

  • Ratnam KV, Su WP, Peters MS (1991) Purpura simplex (inflammatory purpura without vasculitis): a clinicopatho-logic study of 174 cases. J Am Acad Dermatol 25:642–647

    Article  PubMed  CAS  Google Scholar 

  • Smoller BR, Kamel OW (1991) Pigmented purpuric eruptions: immunopathologic studies supportive of a common immunophenotype. J Cutan Pathol 18:423–427

    Article  PubMed  CAS  Google Scholar 

Progressive Pigmented Purpura

  • Ghersetich I, Lotti T, Bacci S et al. (1995) Cell infiltrate in progressive pigmented purpura (Schamberg’s disease): immunophenotype, adhesion receptors, and intercellular relationships. Int J Dermatol 34:846–850

    Article  PubMed  CAS  Google Scholar 

  • Kano Y, Hirayama K, Orihari M et al. (1997) Successful treatment of Schamberg’s disease with pentoxifylline. J Am Acad Dermatol 36:827–830

    Article  PubMed  CAS  Google Scholar 

  • Nagata K, Danno K, Tanaka S (1999) Unilateral Schamberg disease in a 14-year-old Japanese boy. J Dermatol 26: 348–351

    PubMed  CAS  Google Scholar 

  • Reinhold U, Seiter S, Ugurel S et al. (1999) Treatment of progressive pigmented purpura with oral bioflavonoids and ascorbic acid: an open pilot study in 3 patients. J Am Acad Dermatol 41:207–208

    Article  PubMed  CAS  Google Scholar 

Purpura Annularis Teleangiectoides

  • Honda M, Saijo S, Tagami H (1997) Majocchi’s disease in a newborn baby: a familial case. Br J Dermatol 137:655–666

    Article  PubMed  CAS  Google Scholar 

  • Kim HJ, Skidmore RA, Woosley JT (1998) Pigmented purpura over the lower extremities. Purpura annularis telan-giectoides of Majocchi. Arch Dermatol 134:1477–1480

    Article  PubMed  CAS  Google Scholar 

Lichenoid Purpuric Dermatitis

  • Krizsa J, Hunyadi J, Dobozy A (1992) PUVA treatment of pigmented purpuric lichenoid dermatitis (Gougerot-Blum). J Am Acad Dermatol 27:778–780

    Article  PubMed  CAS  Google Scholar 

  • Lotti T, Ghersetich I, Panconesi E (1994) Why should we use PUVA treatment in pigmented purpuric lichenoid dermatitis. J Am Acad Dermatol 30:145

    Article  PubMed  CAS  Google Scholar 

Eczematid-Like Purpura

Lichen Aureus

Gardner-Diamond Syndrome

  • Anderson JE, DeGoff W, McNamara M (1999) Autoerythro-cyte sensitization (psychogenic purpura): a case report and review of the literature. Pediatr Emerg Care 15: 47–48

    Article  PubMed  CAS  Google Scholar 

  • Berman DA, Roenigk HH, Green D (1992) Autoerythrocyte sensitization syndrome (psychogenic purpura). J Am Acad Dermatol 27:829–832

    Article  PubMed  CAS  Google Scholar 

  • Gardner FH, Diamond LK (1955) Autoerythrocyte sensitization. A form of purpura producing painful bruising following autosensitization to red blood cells in certain women. Blood 10:675–690

    PubMed  CAS  Google Scholar 

  • Gomi H, Miura T (1994) Autoerythrocyte sensitization syndrome with thrombocytosis. Dermatology 188:160–162

    Article  PubMed  CAS  Google Scholar 

  • Moll S (1997) Psychogenic purpura. Am J Hematol 55: 146–147

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 2000 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Braun-Falco, O., Plewig, G., Wolff, H.H., Burgdorf, W.H.C. (2000). Disorders of Hemostasis. In: Dermatology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-97931-6_23

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-97931-6_23

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-97933-0

  • Online ISBN: 978-3-642-97931-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics