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81 Cards in this Set

  • Front
  • Back
What are two types of Hemorrhagic disorders you can have?
-Primary
-Secondary
What are Primary hemorrhagic disorders?
Disorders of platelet PLUG formation - the INITIAL thing that forms in clotting
What is the characteristic sign of a defect with initial plt plug formation?
Petechial hemorrhages - pinpoint or punctate hemorrhages on SKIN and MUCOUS MEMBRANES
What are multiple petechial subcutaneous hemorrhages sometimes described as?
A RASH
What lab tests are indicative of defects of primary hemostasis - platelet plug formation?
Prolonged BLEEDING times - but there won't be a prolonged PT or PTT
What are 2 classes of disorders of primary hemostasis?
-Vascular lesions
-Platelet disorders
Which of the two is more likely to have an abnormal bleeding time?
Platelet disorders - vascular lesions rarely have abnormal lab findings
What is Simple purpura? Where is it found, on whom?
Easy bruising - esp on the upper thighs, in otherwise healthy persons
What is it called when there are hemorrhagic areas on the backs of hands and forearms of older people?
Senile purpura
Why do older people tend to get senile purpura?
Because of age-dependent atrophy of the vascular supportive tissues
What vitamin deficiency leads to vascular lesions?
Vit C deficiency - scurvy
Where are hemorrhages typically seen in scurvy?
-Gingival
-Muscles
-Subcutaneous tissues
What does Scurvy do to the little hairs on the body? Why?
Makes them twisted and corkscrewy - because of hemorrhagic Perifollicular hyperkeratotic papules that develop
What is a form of hypersensitivity vasculitis that is seen in young children?
Henoch-Schonlein purpura
What is synonymous with hypersensitivity vasculitis?
Leukocytoclastic angiitis
And what causes the purpura in Henoch-Schonlein?
An immune reaction that damages the vascular endothelium
What are 4 clinical findings in Henoch Schonlein?
1. Palpable purpura - Hemorrhagic urticaria
2. Fever
3. Arthralgias
4. GI/Renal involvement
What patient population is associated with Hereditary hemorrhagic telangiectasia?
Osler-Weber-Rendu - in Mormons of Utah
What is the inheritance of Osler-Weber-Rendu?
Autosomal dominant
What is clinically seen in Osler Weber Rendu?
Localized malformations of venules and capillaries of the skin and mucous membranes leading to hemorrhages
What connective tissue disorder can lead to vascular bleeding?
Ehlors-Danlos
What plasma dyscrasia can lead to vascular damage?
Waldenstrom macroglobulinemia
How does Waldenstrom's macroglobulinemia lead to vascular damage?
Due to the hyperviscosity of blood, and also platelet abnormalities
What other systemic condition can cause vascular damage?
Amyloidosis
What are 2 bacterial infections that lead to primary hemorrhagic disorders?
-Rickettsial diseases - RMSF
-Meningococcal diseases
Why do Rocky mtn spotted fever and Meningococcemia cause primary hemostasis disturbance?
Because they involve the vascular endothelium and lead to necrosis and rupture of small blood vessels
What are 2 types of PLATELET disorders that can lead to Primary Hemostasis dysfunction?
1. Thrombocytopenia
2. Platelet FUNCTION abnormality
What are 3 characteristic findings in Thrombocytopenia?
-Cutaneous petechiae
-Intracranial bleeds
-Mucosal oozing
What lab findings are seen in Thrombocytopenia?
-Decreased Plt count
-Increased Bleeding Time
What will cause differences in Megakaryocyte counts in the BM in thrombocytopenia?
Whether it is due to decreased production, or increased destruction.
What are 4 conditions that show decreased Platelet production?
-Radiation
-Acute leukemia - replaces BM
-Myelopthesis
-Aplastic anemia
What are 3 conditions that affect circulating platelets, rather than production?
-Multiple transfusions - dilutes
-DIC - uses them all up
-Splenic sequestration
What is TTP?
Thrombotic thrombocytopenic purpura
What happens in TTP?
Platelets form hyaline microaggregates along the vessels causing narrowing!
What happens as a result of the narrowed vessels?
RBCs get hemolysed - microangiopathic HA
So what are findings on a PBS in TTP?
-Helmet cells
-Schistocytes
-Low platelets
What are 3 other developments often in TTP?
-Transient neurologic abnormlts
-Renal insufficiency
-Fever
What is a CAUSE of TTP?
Deficiency in ADAMTS 13 - vWF metalloproteinase
What is the result of LACKING the metalloproteinase of vWF?
Large very high-MW multimers of vWF which PROMOTES platelet aggregation
What is ITP?
Idiopathic or Immune Thrombocytopenic Purpura
How is ITP different when in Children vs Adults?
Children - follows a viral infection; acute, self limiting

Adults - Chronic
What is ITP CAUSED by?
Auto-antibodies that coat plts and then cause splenic removal
What can cause FETAL ITP?
Maternal antibody crossing the placenta
What are the 2 diagnostic features of ITP?
-Decreased platelets
-Increased Megakaryocytes
What are the 2 hallmark findings in Platelet FUNCTION abnormalities?
-Normal platelet count
-Increased BT
What are 2 things that cause Abnormal Platelet FUNCTION?
-Abnormal plt ADHESION
-Abnormal plt AGGREGATION
What is the disease of abnormal platelet adhesion?
Bernard soulier - lack of GP1b/a receptor for vWF so platelets can't adhere to exposed collagen
What is seen on the PBS in Bernard soulier?
Large platelets
What is an inherited disorder of platelet aggregation?
Glanzmann's thrombasthenia - lack of GP2b/3a so fibrinogen can't crosslink platelets with fibrinogen bridges
What causes ACQUIRED disorder of platelet aggregation?
Aspirin - inhibits COX causing lack of TxA2
What causes 2ndary hemostasis disorders?
Deficiency of CLOTTING factors
How are symptoms of 2ndary hemostasis disorders different from those of Primary?
Involve bleeding from LARGER vessels - not pinpoint/petechiae
What are 4 common manifestations of 2ndry Hemostatic disorders?
-Hemarthroses
-Large hematomas
-Large ecchymoses
-Extensive bleeding in traumas
How are the LAB findings different in 2ndry vs primary hemostatic disorders?
-BT will be NORMAL
-Platelets are usually normal
-PT and/or PTT will be prolonged
What will a prolonged PT indicate?
Deficiency of Fibrinogen, or Factors 2, 5, 7, or 10
What will a prolonged PTT indicate?
Deficiency of ALL factors EXCEPT 7 or 13
What does a prolonged Thrombin Time indicate?
Deficiency of Fibrinogen
What is Classic Hemophilia?
Fx 8 deficiency - Hemophilia A
What is the inheritance pattern of Classic hemophilia?
X-linked
What is diagnostic of Hemophilia A?
Prolonged PTT that corrects with normal plasma
What is indistinguishable from Hemophilia A?
Christmas disease - Fx 9 defic, Hemophilia B
What is the most COMMON cause of a prolonged PT in adults?
Lack of Vit K
What causes the lack of Vit K?
FAT Malabsorption due to:
-Pancreatic disease
-Small bowel disease
What causes lack of Vit K in NEONATES?
Lack of gut flora to make it!
What does Vit K deficiency in neonates lead TO?
Hemorrhagic disease of the Newborn
What does Hemorrhagic dis of the Newborn result in?
Lack of all the Vit K dep clotting factors, so prolonged PT AND PTT!
What is the most COMMON hereditary bleeding disorder?
von Willebrand disease
Where is vWF made?
-Endothelial cells
-Megakaryocytes
What is vWF FOR?
-Carries Fx 8
-Platelet adhesion
So what type of a disorder is LACK of vWF?
Mixed - both primary and secondary hemostatic disorder!
What are the characteristic findings in vWD?
-Abnormal BT - lack adhesion
-Prolonged PTT - lack Fx VIII
What is DIC?
Widespread clotting leading to consumption of Platelets, Fx's 2, 5, 8, and Fibrinogen!
What are the clinical manifestations of DIC?
Thrombosis PLUS hemorrhage
What lab tests will be abnormal in DIC?
ALL OF THEM
-BT prolonged
-PT/PTT
-Thrombin time Tct
-FSP's and D-dimer
When is DIC most COMMONLY seen?
Obstretic complications!
What is a systemic condition that leads to coagulopathy?
LIVER DISEASE
Why does liver disease lead to coagulopathy?
Because ALL clotting factors are made there except vWF!
What lab findings will develop as hepatocellular disease progresses?
PT/PTT/TT will all prolong
What can be used to help alleviate the clotting factor deficiencies in liver disease?
Vit K derivatives
How do Multiple transfusions cause coagulopathy?
Dilutional - if they lack platelets and factors
What is the clinical manifestation of Dilutional coagulopathy?
Persistent bleeding from surgical wounds