Lecture: Platelet and Hemostatic Disorder Flashcards
Assess the amount of platelets
Quantitative
Defective platelet adhesion, secretion and aggregation
Qualitative
Caused by clonal proliferation of hematopoietic cells; Primary bone marrow defect
Essential Thrombocytosis
Do not manifest bleeding tendencies; result of underlying conditions
Secondary Thrombocytosis
Chronic blood loss due to ineffective regulation of thrombopoiesis by iron
Iron-deficiency Anemia
Autoimmune disorder (rheumatoid
fever/arthritis)
Chronic Inflammatory Disease
Bruising; large, irregularly-shaped hemorrhage
Ecchymosis
Known as stress platelets, appear in compensation for thrombocytopenia
Reticulated platelets
Increase risk of significant bleeding
< 50,000 μL
Risk of severe spontaneous bleeding
< 10,000 μL
Decreased in number of megakaryocytes; due to bone marrow aplasia/hypoplasia
Thrombocytopenia: Decreased Production
Bone marrow is infiltrated by fibrotic tissue (tumor) which infect the cells
Myelophthisis
Kills cancer cell but also damages normal cells
Radiation / chemotherapy
50% of patient only has 6 months to live; difficult for recovery due to bone marrow damage
Aplastic anemia
Thiazide
Chlorothiazide
Chloramphenicol
Bone Marrow Suppressants
Production is good; circulation is defective
Thrombocytopenia: Ineffective Production
Abnormal platelet structure; increased destruction
Hereditary Thrombocytopenia
FAB M6
Di Guglielmo’s Syndrome (erythroleukemia)
Involved in development of all nuclear cell maturation
Vitamin B12 or Folate
Dohle-like inclusions in neutrophil; large/giant platelets
May-Hegglin Anomaly
Overproduction of platelet being stored in spleen; not enough in circulation
Hypersplenism/Splenomegaly
Vascular shunting
Hypothermia
Platelets (primary hemostasis) and coagulation factor (secondary hemostasis); no bone marrow defect but in circulation
Thrombocytopenia: Consumption - Combined
Excessive clot formation and clot
lysis repetitively
Disseminated Intravascular Coagulation
Appearance of ultra large vWF (activates platelets which then causes platelet plug formation)
Thrombotic thrombocytopenic purpura
(TTP); Moschkowitz Syndrome
Infection of Shiga-like toxin (1 and 2), E. coli O157:H7, Shiga toxin (produced by Shigella spp.) – which causes abnormal consumption due to G.I. bleeding
Hemolytic Uremic Syndrome
Production of antiplatelets Ab (alloantibodies and autoantibodies)
Thrombocytopenia: Immune Destruction
Platelet autoantibodies
Idiopathic thrombocytopenic purpura (ITP)
ITP: Children; during infection & post-vaccination
Acute ITP
ITP: Adult; during platelet
autoantibodies
Chronic ITP
Multiple transfusion; develops antibodies from foreign body fluid (blood) like donor to recipient
Post-transfusion purpura
Maternal Ab; neotanal immune-mediated thrombocytopenia; mother produces antibody against infections while having a fetus - mother develop IgG against fetus which has inherited father antigen
Isoimmune neonatal purpura
Develop antibody from self
Autoimmune
Develop antibody from
different species
Alloimmune
Mimics ITP
HIV infection
Physical manifestation; enlarged spleen; larger number of platelet sequestrated
Gaucher’s Disease
Rare bone marrow cancer
Myelofibrosis/Osteomyelofibrosis
Seen after multiple blood transfusion (prolonged platelet storage that are dysfunctional/function-depleted)
Dilutional Thrombocytopenia
Adhesion disorder; autosomal recessive disorder affecting the membrane receptor, GpIb
Bernard-Soulier Syndrome
Aggregation disorder; autosomal recessive disorder affecting the receptor complex GpIIb-IIIa preventing the binding of fibrinogen
Glanzmann’s Thrombastenia
Caused by mutation in the gene that controls production of melanosomes, platelet dense bodies, and lysosomes
Hermansky-Pudlak Syndrome
Characterized by giant cytoplasmic granules and decreased ADP released; manifestations include ocular, neurologic, and skin abnormalities, possible neutropenia, normal platelet count but diminished aggregation response
Chediak-Higashi Syndrome
X-linked disorder highlighted by thrombocytopenia, recurrent infections, eczema, and a predisposition to secondary leukemia or lymphoma
Wiskott-Aldrich Syndrome
Characterized by the finding of large platelets that lack α-granules producing a gray appearance on a Wright-stained smear
Gray Platelet Syndrome
Characterized by abnormal proteolysis of α-granules and selective defective aggregation with epinephrine
Quebec Platelet Disorder
Defect in thromboxane generation by abnormal response to arachidonic acid; platelet count may be normal
Cyclooxygenase Deficiency
Defect in platelet coagulant activity caused by impaired transmembrane migration of the procoagulant; PS resulting in the absence of Xa-binding sites
Membrane Phospholipid Deficiency
Platelets will not adhere due to lacking VWF
Von Willebrand’s Disease
No fibrinogen
Afibrinogenemia
Defective or abnormal fibrinogen
Dysfibrinogenemia
Also known as “acetylsalicylic acid”;
inhibits cyclooxygenase
Aspirin Intake
Platelet dysfunction results from coating of the platelet membrane by paraprotein and does not depend on the type of paraprotein present
Multiple Myeloma and Waldenstrom’s
Macroglobulinemia
Induces thrombocytopenia and a severe platelet function defect that assumes major importance in relation to post-surgical bleeding – platelet count is low
Cardiopulmonary Bypass Surgery
Reduced platelet adhesion, abnormal platelet aggregation (in response to ADP, epinephrine, and thrombin), abnormal phospholipid availability, and reduced procoagulant activity
Liver Disease
“Devil’s pinches”; Skin fragility; Every time you bump on the chair – vessels injured
Simple Purpura
Release tissue thromboplastin
Extrinsic problem
Excessive collagen
Keloid
Sudden increase on body’s pressure –
extravasation of RBCs
Mechanical Purpura
Emotional trauma (accident, loss, injury); Hormonal changes in the body; creating permeability to blood vessels
Psychogenic Purpura
Brown spots; As we grow old, formation of collagen slows down – normal process in the body
Senile Purpura
Progressive pigmented purpura; cayenne pepper purpura appearance
Schamberg’s Purpura
Self-induced trauma
Factitious Purpura
Caused by bacterial, fungal, viral and parasitic microorganisms; releases toxins which damages blood vessels
Infectious Purpura
Unique infectious purpura disorder affecting the vessel
Purpura fulminans
Caused by Neisseria meningitis; Excessive lysis and formation
Waterhouse-Friedrichsen Syndrome
Allergic vasculitis (IgA) involves the skin affecting, GIT, kidneys, heart, joint problems and CNS (autoimmune process); common in children, might exist as single disease or may coexist
Henoch-Schonlein Purpura
Abdominal pain, secondary to GIT bleeding
Henoch Purpura
Secondary to joint pain, rheumatoid arthritis, loss of cartilage in joints
Schonlein Purpura
Causes gum bleeding or gingivitis
Scurvy (Vitamin C Deficiency)
Lack of insulin/inhibitors of insulin; increase concentration of sugar in blood; slows down blood flow and destroy blood vessels
Diabetes Mellitus
Causes abnormal enlargement of muscle/vessel, increased testosterone; described by difference in face, shoulders and pink or purple stretch marks
Cushing Syndrome
Proteins are large and big substances and destroy blood vessels, second to dysproteinemia
Protein C Deficiency
Inability to convert procollagen to collagen; hyperextensible
skin, hypermobile joints, joint laxity, fragile tissues, and a bleeding tendency, primarily subcutaneous hematoma formation
Ehler’s Danlos Syndrome
Elastic fibers and blood vessels are not elastic enough; elastic fiber calcification, prone to fragility
Pseudoxanthoma Elasticum
Elongated extremities and fingers, not proportional to patient bodies; mutation in the FBN1 gene that codes fibrillin
Marfan Syndrome
Soft and fragile, incomplete formation of bone; mutation in type I collagen genes
Osteogenesis Imperfecta
The vascular defect of this disorder is characterized by thin-walled blood vessels with a discontinuous endothelium; most common blood vessel disorder; lesions on skin, lips and tongue; thin-dilated vessels; caused by Iron Deficiency Anemia
Hereditary Hemorrhagic-Telangiectasia
Another term for Hereditary Hemorrhagic-Telangiectasia
Rendu-Osler-Weber Sydrome
Vascular tumor which blocks functions of blood vessels and destroy them; “Kasabach-Merritt Syndrome”; associated with acute or chronic DIC and MAHA
Congenital Hemangiomata
The deposition of abnormal quantities of amyloid protein in tissues; heavy metal poisoning
Amyloidosis
Excessive destruction of own cells by your own antibody; can be caused by either allergic reaction or drug-induced endothelial damage; drug-induced purpura
Autoimmune Vascular Purpura
