This document discusses bleeding disorders and summarizes key points about hemostasis and platelet function. It begins by defining hemostasis and its functions in maintaining a fluid blood state and inducing clotting at sites of injury. Bleeding disorders are then classified based on defects in vascular, platelet, or coagulation systems. Specific inherited and acquired bleeding disorders that result from vascular defects like hereditary telangiectasia and scurvy are described. Platelet disorders are also outlined, distinguishing quantitative issues like thrombocytopenia from qualitative defects. Causes of decreased platelet count or production and increased platelet destruction are summarized.

1. BLEEDING DISORDERS
SUNIL KUMAR.P
Dept. of Haematology
SJMCH
Bangalore
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3. Contents …….
• What is Hemostasis …..?
• Functions …..?
• Bleeding Disorders …..
• Classification of bleeding disorders….
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4. Hemostasis:
• Hemostasis refers to spontaneous arrest of
bleeding caused by injury of small blood
vessels.
• Small vessels are continuously exposed to
trauma during normal activities can not bleed
due to effects of hemostasis.
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5. Functions of hemostasis:
• Maintains blood in a fluid, clot free state in
normal vessel.
• Induce rapid and localized haemostatic plug
at the site of injury.
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6. Mechanism of Hemostasis:
• Hemostasis depends on three components
• Vascular wall
• Platelets
• Coagulation cascade
• Fibrinolytic stage
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7. BLEEDING DISORDERS
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8. DEFINITION
Hemorrhagic disorders are a group of
disorders, of many different causes
characterized by an abnormal tendency of
bleeding due to failure of hemostasis
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9. Clinical Character of Hemorrhagic
Disorders:
• Spontaneous bleeding in the skin, mucous
membrane or internal tissue.
• Extensive or prolonged bleeding following
trauma.
• Bleeding from more than one site.
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10. Causes of Hemorrhagic Disorders
• 1. Hypocoagulation
• 2. Hyper coagulation
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11. Causes of Hypocoagulation
• 1.Due to vascular defects
• 2.Due to platelet defects
• 3.Due to coagulation defects
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12. Bleeding Disorder Terms
• Petechiae-pinpoint size/pinhead size hemorrhagic spots in skin
• Purpura-hemorrhage under the skin, varying in color and duration
• Ecchymosis-purplish patch caused by extravasation of blood into skin,
larger than petechiae
• Epistaxis-nosebleed
• Menorrhagia-excessive menses
• Hematuria-blood in urine
• Hemarthrosis-bleeding into joint
• Hematemesis-vomiting blood
• Hemoptysis-spitting blood
• Melena-blood in stool (occult blood)
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13. Petechiae
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14. Ecchymosis
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15. Hematoma
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16. Bleeding due to vascular defects
• Common characters:
• More common but less severe
• Usually in the form of petechiae or purpura
• Increased bleeding time and frequently
positive tourniquet test.
• Normal platelet count or function.
• Normal CT, APTT, PT and TT
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17. CLASSIFICATION OF VASCULAR
BLEEDING DISORDERS:
• INHERITED
• ACQUIRED
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18. • INHERITED
1
• ACQUIRED
2
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19. The inherited disorders of vascular
system
1. Hereditary telangiectasia
2. Ehlers-Danlos syndromes
3. Marfan syndrome
4. Osteogenesis imperfecta
5. Psedoxanthoma elasticum(AR)
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20. Acquired disorders of vascular system
• 1. Purpura due to decreased connective tissue
• 2. Purpura associated with paraproteins
• 3. Purpura due to vasculitis
• 4. Mechanical purpura
• 5. Purpura simplex
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21. Acquired disorders of vascular system
1. Purpura due to decreased connective tissue
 Senile purpura
 Cushing syndrome
 Corticosteroid therapy
 Scurvy
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22. 2. Purpura associated with paraproteins
 Paraproteinemias
 Amyloidosis
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23. 3. Purpura due to vasculitis
 Allergic purpura (Henoch-Schonlein purpura)
 Infections
 Drugs
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24. 4.Mechanical purpura
 Increased venous pressure
5.Purpura simplex
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25. INHERITED DISORDERS
1. HEREDITARY TELANGIECTASIA
• AD trait
• Characterised by the presence of flat ,red or
purple lesions on mucous membranes
• Due to the lack of elastic fibers in the vessel
wall
• Epistaxis is the most common manifestation
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26. Herediatary telangiectasia
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27. 2.EHLERS –DANLOS SYNDROME
• Autosomal dominant trait
• Defect in collagen synthesis resulting in weak
subendothelial connective tissue and produce
bleeding.
• Extreme fragility of vessels, Easy
bruising,rupture of large vessels,petechiae,GI
bleeding are common.
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28. Ehlers-danlos syndrome
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29. 3.MARFAN SYNDROME
• AD inheritance
• characteristic defects are long
extremities,spidery fingers,dislocation of lens,
and easy bruising.
• Bruising may be caused by abnormalities of
the vessels.
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30. Marfan syndrome
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31. 4.OSTEOGENESIS IMPERFECTA
• Autosomal dominant trait .
• Disorder of the genes for type 1 procollagens
which cause patchy ,defective bone matrix.
• Easy and spontaneous bruising,epistaxis,and
intracranial hemorrhage are the bleeding
symptoms.
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32. 5.PSEUDOXANTHOMA ELASTICUM
• Autosomal recessive manner.
• due to the presence of abnormal elastic
tissue in the skin and all arteries.
• Easy bruising,petechiae and purpura are
common.
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33. ACQUIRED DISORDERS
• 1.SENILE PURPURA
• Usually occur in old age.
• A deficient supportive sub-endothelial
connective tissue leads to easy rupture of
vessels and bleeding occurs.
• Common sites of bleeding are extensor
surface of forearm and hand
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34. Senile purpura
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35. 2.CUSHING SYNDROME
 Related to altered connective tissue support
of the blood vessel wall.
 It may due to abnormalities of
mucopolysaccharides in the supporting tissue
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36. 3.SCURVY
• Caused deficient Vitamin C
• Vitamin C required for vessel collagen integrity
• Acts as “cement” holding endothelial cells
together
• Lack of Vitamin C prevents proper collagen
formation
• Result: bleed and vessel fragility
• Symptoms include gum bleeding, petechiae and
bleeding into tissues and muscles
• Treated with Vitamin C
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37. 4.PARAPROTEIN DISORDERS
• Paraproteins are monoclonal Ig .
• Occurs due to the deposition of protein in the
vascular wall.
• Symptoms (hemostasis) include
purpura,bleeding and thrombosis.
• Bleeding symptoms include
epistaxis,petechiae,and hemorrhage into
organs.
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38. 5.AMYLOIDOSIS
• Due to the deposition of amyloid in the skin
and vascular walls.
• Leads to fragility of the vessel and to
bruising.
• Bleeding into visceral organs can occur
• Thrombosis is common.
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39. ALLERGIC PURPURA
• Usually occur in children.
• The skin lesions begin as urticaria,change to
pink,then to red and hemorrhagic.
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40. INFECTIONS
• Purpura associated with infections can caused by
damage to the blood vessels
(nonthrombocytopenic purpura)
• It is related to nonspecific immune complex
formed by the antigenic agent and its
corresponding antibody.
• This complex attach to either the endothelial
cells or to the underlying subendothelial
structures ,results in inflammation and vasculitis.
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41. DRUG INDUCED
• Aspirin,quinine and warfarins
• Cause vasculitis with the appearance of
ecchymoses in the absence of
thrombocytopenia.
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42. PURPURA SIMPLEX
• Easy bruising syndrome
• Commonly seen in young woman
• Spontaneous small ecchymoses appear mainly
on the skin of the thighs or upper arms-devil’s
pinches.
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PLATELET DISORDERS

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45. PLATELET DISORDERS
• Function in hemostasis is to form the primary
hemostatic plug.
• Platelet plugs are a response to injury to
blood vessels and minimize blood loss.
• Petechiae,epistaxis,GI bleeding,excess
bleeding from superficial wounds,cuts and
easy bruisability are common manifestations
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46. Platelet Disorders Classification:
Thrombocytosis
Thrombocytopathy
Thrombocytopenia
PLT Reference Range = 150 - 450 x109/L
Qualitative PLT Disorders
Quantitative PLT Disorders
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47. Thrombocytopenia and
thrombocytosis
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48. CLASSIFICATION OF PLTELET
DISORDERS
I. Quantitative
• Thrombocytopenia
• Thrombocytosis
2. Qualitative
• Hereditary
• Acquired
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49. QUANTITATIVE PLATELET DISORDERS
• 1.THROMBOCYTOPENIA
• Thrombocytopenia refers to decreased number
of platelet below the lower limit of normal range,
1,50,000/cmm of blood.
• It is the most common cause of abnormal
bleeding.
• Bleeding is common when the count is less than
30,000 – 40,000/cmm of blood.
• Bleedings are associated with prolonged bleeding
time.
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50. Etiology of Thrombocytopenia
• Thrombocytopenia may results from
• Impaired platelet production
• Accelerated platelet destruction
• Dilution
• Splenic sequestration
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51. a) Due to increased destruction
• The platelets in this condition have a
decreased lifespan after they have been
released into the peripheral blood.
• Causes of destruction are of 2 types
 immune destruction
Non immune destruction
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52. Immune destruction
• Mechanism of destruction is phagocytosis of
ab-sensitized platelets by splenic
macrophages.
• Classified into
Idiopathic thrombocytopenia purpura(ITP)
Transplacental thrombocytopenia
alloantibodies-(transfusion or pregnancy)
drugs
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53. ITP
• Increased destruction of platelets by immune
mechanism.
• Also known as autoimmune
thrombocytopenia or immune
thrombocytopenic purpura
• Types
Acute ITP
Chronic ITP
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54. Acute Form
– Most common in children 2 to 6 years
– Self Limited and > 90% remission rate
– easy bruising,petechiae,bleeding from mucous
membranes.
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55. Chronic Form
– Adult disease primarily
– Women more often than men
– Insidious onset with no prodrome
– Symptoms include: easy bruising, prolonged
menses, mucosal bleeding
– Bleeding complications are unpredictable
– Mortality is 1%
– Spontaneous remission is rare
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56. NON - IMMUNE MECHANISM
• Platelets can be destroyed by different ways
1. In situations when thrombin is formed
intravascularly resulting in fibrin formation
and platelet activation and fragmentation.
2. After damage to blood vessel endothelium
so that platelets are activated by surface
contact with subendothelial collagen.
3. By mechanical destruction in patients with
artificial heart valves.
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57. b)Due to decreased platelet
production
• It is characterized by failure of the bone
marrow to deliver adequate no: of platelets to
the peripheral blood.
• It occurs because:
 Megakaryocyte hypoplasia exists in the bone
marrow.
Ineffective thrombopoiesis
Hereditary thrombocytopenic disorder
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58. c) Increased splenic sequestration
• Spleen stores one third of the platelets
produced by the BM in a pool.
• In hypersplenism no: of macrophages are
increased.
• platelets are removed by increased pooling
and by increased phagocytosis.
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59. d) Dilution
• when history of massive hemorrhage
• platelets can be replaced by transfusion of
platelet concentrates
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60. 2) THROMBOCYTOSIS
• Increased no: of platelets are the result of
increased production by the BM since the
lifespan of platelet is not increased.
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61. Based on etiology
• Primary thrombocytosis
• Secondary thrombocytosis
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62. 1) PRIMARY THROMBOCYTOSIS
• In primary thrombocytosis,megakaryocyte
proliferation and maturation bypass the
normal regulatory mechanism.
• Uncontrolled or autonomous production of
megakaryocyte results in a marked increase in
the no: of circulatory platelets.
• The platelet count is usually more than
1000×109 /L
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63. • Hemorrhagic symptoms are present
• Epistaxis ,bleeding from GI tract and from
other mucous membranes can be quite
profuse.
• Excess bruising from minor trauma.
• Prolonged BT
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64. 2)SECONDARY THROMBOCYTOSIS
• Also known as “reactive thrombocytosis”
• In this,platelet count is increased because of
another disease or condition.
• Causes
 Acute hemorrhage
 Surgery
 Malignant disease
 Chronic inflammatory diseases
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65. QUALITATIVE (FUNCTIONAL )
PLATELET DISORDERS
• Abnormalities of any phase of platelet
function(adhesion,aggregation or release) may
lead to the prolonged or abnormal formation of a
primary hemostatic plug.
• Common manifestations are
petechiae,superficial bruises in the skin,bleeding
from mucous membranes such as nose or
abnormal vaginal bleeding and prolonged
bleeding from cuts
• Platelet count is usually normal.
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66. • Platelet functional disorder are classified into
 hereditary disorders
 acquired disorders
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67. HEREDITARY DISORDERS OF PLATELET
FUNCTION
• Autosomal recessive
• Abnormalities in function can be related to
affected portion of the platelet ultrastructure
• it is classified into
 disorders of platelet adhesion
 disorders of platelet aggregation
 disorders of platelet secretion
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68. a) Disorders of platelet adhesion
• Adhesion to collagen requires the presence of
both adequate amounts of functional von
Willebrand factor and the presence of
glycoprotein Ib on the platelet membrane.
• Von Willebrand factor serves as a “bridge”
binding the platelet via glycoprotein Ib to
collagen .
• Adhesion to collagen is the major mechanism
initiating platelet function
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69. • Two hereditary disorders in which platelets
fail to adhere to collagen are :
1. BERNARD-SOULIER DISEASE
-there is a decrease in amount or abnormal
function of fractions of platelet membrane
glycoprotein, including glycoprotein Ib.
-also called “giant platelet syndrome”
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70. • 2. VON WILLEBRAND DISEASE
- Characterized by a decreased production
of von Willebrand factor or by an abnormal
molecule.
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71. b) Disorders of platelet aggregation
• Platelet aggregation requires the presence of
fibrinogen and glycoprotein IIb /IIIa receptor on
the platelet membrane.
• In the absence of one or more of these
components,platelets will not interact with one
another to produce either primary or secondary
aggregation.
• eg:Glanzmann thrombasthenia(platelet of
patients are deficient in the glycoprotein IIb/IIIa
complex)
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72. c) Disorders of platelet secretion
• It is a heterogenous group of defects in the
mechanisms of release from platelet granules
or membranes.
• they include defects in the content of the
dense granules(storage pool disease),defect in
the mechanism of release from the granules.
• Gray platelet syndrome-absence of α granules
causes platelet to appear agranular on
peripheral blood smear.
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73. AQUIRED DISORDERS OF PLATELET
FUNCTION
• It is induced in a variety of conditions and
with the ingestion of certain drugs.
• UREMIA
• -the pathogenesis is related to the
accumulation of waste products in the blood
• ecchymoses ,GI bleeding ,and hemorrhage
into serous cavities are the manifestations.
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74. HEMATOLOGIC DISORDERS
• It is characterized by myeloproliferative
disorders,chronic myelogenous
leukemia,polycythemia vera and essential
thrombocythemia ,are characterized by
thrombocytosis ,by abnormalities of platelet
morphology and in some cases by
megakaryocyte morphology.
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75. DRUGS
• Many drugs have been shown to contribute
to platelet dysfunction.
• eg:s
• Aspirin
• Alcohol
• Antibiotic agent like carbenicillin
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76. • thank you……
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