blood disorders
DESCRIPTION
Blood disorders . What is hematology?. Hematology is the study of blood and is concerned mainly with the formed elements in the blood. The formed elements in the blood include: - PowerPoint PPT PresentationTRANSCRIPT
![Page 1: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/1.jpg)
Blood disorders
![Page 2: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/2.jpg)
What is hematology?
• Hematology is the study of blood and is concerned mainly with the formed elements in the blood.
• The formed elements in the blood include:– The white blood cells (leukocytes) which include the
neutrophils, eosinophils, basophils, monocytes, and lymphocytes (.
– The red blood cells (erythrocytes)– The platelets (thrombocytes)
• All of the formed elements in the blood are derived from same pluripotential stem cell in the bone marrow
![Page 3: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/3.jpg)
What is hematology continued
• Erythrocytes function in the transport of oxygen to the tissues.
• Leukocytes function in both specific (immune responses) and non-specific defenses against foreign invasion.
• Thrombocytes function in hemostasis or blood clotting.
![Page 4: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/4.jpg)
• Hemostasis • Disorders of bleeding • Anemia • Blood malignancies
![Page 5: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/5.jpg)
Hemostasis
![Page 6: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/6.jpg)
definition
• Maintenance of fluidity of blood while in vessel and formation of hemostatic plug on vascular injury
![Page 7: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/7.jpg)
Balance between clot formation and bleeding is maintained
![Page 8: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/8.jpg)
Hemostasis involves
• Clot formation • Anti clotting mechanisms
![Page 9: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/9.jpg)
At a site of a vascular injury
1.Vasoconstriction
2.Primary hemostatic plug formation
3.Secondary hemostasis due to activation of coagulation cascade by tissue factor and phospholipid via extrinsic pathway- the end result being fibrin which traps the cells in the blood forming a clot
![Page 10: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/10.jpg)
![Page 11: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/11.jpg)
Vasoconstriction
• due to local neural response, and release of endothelin from the endothelium vessels constricted
![Page 12: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/12.jpg)
Primary hemostatic plug formation
due to platelet adhesion activationdegranulation(ADP, TXA2) recruitment of other platelets
![Page 13: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/13.jpg)
• In a site of vessel wall injury platelets in circulation comes in to contact with the ECM
• On contact with ECM constituents, platelets undergo 3 reactions:
1) ADHESION and shape change2) SECRETION (release reaction)3) AGGREGATION
![Page 14: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/14.jpg)
• PLATELET ADHESION
• To sub-endothelial ECM constituents• Bridged by vWF, a product of endothelial cells
![Page 15: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/15.jpg)
• PLATELET SECRETION
• Occurs soon after adhesion• Platelets release ADP and calcium• ADP activation of platelets is essential for
platelet aggregation, further release of ADP
![Page 16: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/16.jpg)
Platelet aggregation
• product of platelet set up a reaction leading to build-up of an enlarging platelet aggregate, the primary hemostatic plug
![Page 17: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/17.jpg)
• Vascular and platelet responses are important in reducing bleeding but their activity is limited.
• To arrest bleeding the proper ‘clot’ should be formed
• This is brought about by the clotting cascade
![Page 18: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/18.jpg)
Coagulation cascade
• The coagulation cascade is essentially a series of enzymatic conversions, turning inactive proenzymes into activated enzymes and culminating in the formation of thrombin.
• Thrombin then converts the soluble plasma protein fibrinogen into the insoluble fibrous protein fibrin.
• This results in formation of the definitive clot
![Page 19: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/19.jpg)
![Page 20: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/20.jpg)
Anti clotting mechanism• Once activated the coagulation cascade must be restricted to the local site
of vascular injury to prevent clotting of the entire vascular tree.• Regulated by natural anticoagulants
• Anti thrombin III• Protein C and Protein S• Tissue palsminogen
• With onset of coagulation cascade, fibrinolytic cascade is also activated to limit the the size of final clot
• Primarily accomplished by plasmin
![Page 21: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/21.jpg)
![Page 22: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/22.jpg)
Disorders of hemostasis
• Clot formation inappropriately -thrombosis
• Bleeding disorders
![Page 23: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/23.jpg)
Bleeding disorders
![Page 24: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/24.jpg)
Types of skin bleeds –terminology
• Petechie - Minute (1- to 2-mm) hemorrhages into skin, mucous membranes, or serosal surfaces
![Page 25: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/25.jpg)
Types of skin bleeds –terminology
• Purpuras - Slightly larger i.e 3- to 5-mm hemorrhages are called purpuras
![Page 26: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/26.jpg)
Types of skin bleeds –terminology
• Ecchymoses - Larger i.e 1- to 2-cm or more subcutaneous hematomas (bruises)
![Page 27: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/27.jpg)
Bleeding disorders
• Vessel wall disorders • Platelet disorders • Coagulation disorders
![Page 28: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/28.jpg)
Vessel wall disorders
• Defective collagen due to connective tissue disorders, vitamin C deficiency
![Page 29: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/29.jpg)
Platelet disorders
• Low platelet count (thrombocytopenia )• Platelet function disorders
![Page 30: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/30.jpg)
Causes of thrombocytopenia • Decreased platelet production-bone marrow disorders like cancers,aplastic
anemia,-drugs, infections • Increased destruction-immune thrombocytopenic purpura-DIC-HUS• Enlarged spleen
![Page 31: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/31.jpg)
Coagulation disorders
• Hemophilia A• Hemophilia B• Vitamin K deficiency• Von Willebrand Disease
![Page 32: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/32.jpg)
Platelet and vessel wall defects usually present as• skin and mucous membranes-
Petechie,Ecchymosis• Gum bleeding and epistaxis• Menorrhagia• Gastrointestinal bleeding• Intracranial bleeding
![Page 33: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/33.jpg)
Clotting factor disorders may present as
• Bleeding Into joints - Haemarthroses• Into deep tissues – Hematoma• Muscle bleeds
![Page 34: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/34.jpg)
Coagulation disorders
• Hemophilia A• Hemophilia B• Vitamin K deficiency• Von Willebrand Disease
![Page 35: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/35.jpg)
Question
• why does vitamin K deficiency give rise to bleeding?
![Page 36: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/36.jpg)
Hemophilia A & Bclinically similar:occur in approximately 1 in 5,000 male birthsaccount for 90% of congenital bleeding
disordersHemophilia A is approximately 5 times more
common than B
![Page 37: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/37.jpg)
Etiology Inherited as a sex linked recessive trait with bleeding
manifestations only in males genes which control factor VIII and IX production are
located on the x chromosome; if the gene is defective synthesis of these proteins is defective
female carriers transmit the abnormal gene A disease of males
![Page 38: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/38.jpg)
![Page 39: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/39.jpg)
Classification
% normal factor level
Causes of bleeding
Severe < 1% bleeding after trivial injury or spontaneous
Moderate
1 - 5% bleeding after minor injury; occasional spontaneous bleeds
Mild 6 - 20 % following major trauma, surgical or dental procedures
![Page 40: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/40.jpg)
Diagnosis
• Atypical bleeding at circumcision or bruising at neonatal vaccines
• Toddlers with lip bleeding or unusual bruising when learning to walk
• Hx of affected males on mother’s side• Elevated PTT• Factor assays
![Page 41: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/41.jpg)
Clinical Features – Joint Bleeds Joints (Hemarthrosis)
Knees, ankles and elbows most common sites begin as the child begins to crawl and walk
Single joint bleed: stiffness, swelling, pain With repeated bleeding into same jt---arthropathy->
stiffness and contractures
![Page 42: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/42.jpg)
![Page 43: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/43.jpg)
Clinical Features – Muscle Bleeds
Bleeding into muscle or soft tissueSites: calfSymptoms: pain, swelling, muscle spasmComplications: nerve compression,
contracture
![Page 44: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/44.jpg)
Other Sites of Hemorrhage
AbdomenGI tractIntracranial bleedsAround vital structures in the neck
Can cause death…
![Page 45: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/45.jpg)
• They have high risk of HIV,Hep B and Hep C due to repeated transfusion of blood products
![Page 46: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/46.jpg)
Management Specific Hemophillia AFac viii preparations CryoDDAVP
Hemophillia BFac ix CPP
![Page 47: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/47.jpg)
General
Avoid NSAIDsAvoid contact sports Avoid IM injections Good dental careEducation – life long managementAcute and long term management of
musculoskeletal problems
![Page 48: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/48.jpg)
Von Willabrand disease
Read…..
![Page 49: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/49.jpg)
Investigations in bleeding disorders
• Bleeding time-vessel wall and paltelet defects detected
• Prothrombin time (PT)-prolonged in disorders of the extrinsic pathway
• Activated partial thromboplastin time(APTT) –prolonged in intrinsic pathway disorders
![Page 50: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/50.jpg)
![Page 51: Blood disorders](https://reader036.vdocuments.us/reader036/viewer/2022081502/568163c2550346895dd4e711/html5/thumbnails/51.jpg)
Thank you…..