Hemonc Flashcards
Heparin to warfarin bridge is necessary because
Proteins c and s have shorter half lives than other factors leading to paradoxical hypercoagulability
For rapid reversal of warfarin give
Ffp or otherwise vit K
For reversal of heparin give
Protamine sulfate
Mech of lmwh (enoxaparin)
Mainly inhibits factor Xa
In hemophilia, pt is __, ptt is ___ and bleeding time is ___
Ptt is prolonged
Pt and bleeding time is normal
What is cryoprecipitate
Mix of factor VIII and fibrinogen more concentrated than ffp
Mild hemophilia can be treated with
Desmopressin
How does desmopressin work to tx hemophilia
Releases factor VIII from endothelial cells
Best initial test to dx hemophilia
Mixing study-mix pt plasma with normal plasma which will correct the ptt
Most accurate test for hemophilia
Obtain specific factor assays for factors VII, VIII, IX, XI and XII
Most accurate dx test for vwd
Decreased agglutination seen on ristocetin cofactor assay
In vwd, what will happen to bleeding time, pt, ptt, and platelet count
Increased bt
Increased ptt (possibly ) due to low factor viii levels
Normal pt and platelet counts
Tx for vwd
Desmopressin
Ocps can be used for menorrhagia
Aspirin ___ risk of bleeding in vwd pts
Increases
Mech of factor V Leiden
Hypercoagulable state
Factor v is resistant to inactivation by protein C
Hypercoagulable state with skin or tissue necrosis following warfarin admin. What am I?
Protein c or s deficiency
Aps is often associated with what two conditions
Sle and rheumatoid arthritis
What tests confirm aps
Lupus anticoagulant and anticardiolipin antibody
Tx of dvt or pe in pts with hypercoagulable state
Heparin followed by 3-6 mos warfarin (longer if it’s a repeated event)
Tx of hit
Discontinue heparin then start argatroban or lepirudin
Dic is characterized by what two things
Thrombosis and hemorrhage
Etiology of dic
Deposition of fibrin in small blood vessels leading to thrombosis and end organ damage; also depletion of clotting factors and platelets leading to bleeding diathesis
Three common associations with dic
Obstetric complications, sepsis and pancreatitis
In dic factor Viii is
Depressed
Three causes of microangiopathic hemolytic anemia
Hus
Ttp
Dic
Etiology of ttp
Deficiency of vwf cleaving enzyme adamts-13 resulting in large vwf multimers that aggregate platelets and create platelet microthrombi. Rbcs are thus fragmented.
Low plt count, microangiopathic hemolytic anemia, neuro changes (delirium, seizure, stroke), impaired renal function, fever. Think
Ttp
Severe increases in creatinine level are more suggestive of hus or ttp
Hus
Platelet transfusion in ttp is
Contraindicated
Tx for ttp
Plasma exchange
Etiology of itp
IgG antibodies formed against platelets
In itp, bone marrow production of platelets is __ and there are __ megakaryocytes in the marrow
Up
Increased
Differential of thrombocytopenia
Hit shoc
Hit or hus Itp Ttp or treatment (meds) Splenomegaly Hereditary (wiskott Aldrich) Other (malignancy) Chemo
Tx of itp if plt count >30,000
None
Tx of itp if plt count <30,000
Corticosteroids or ivig
Thrombocytopenia, Increassd creatinine and schistocytes acutely in a school aged kid, think
Hus
Causes of microcytic anemia
Tails
Thalassemia Anemia of chronic dz Iron def Lead poisoning Sideroblastic anemia
Is tibc high or low in anemia of chronic disease? How about ferritin?
Low and high
Is tibc low or high in iron def anemia? How about ferritin?
Tibc high
Ferritin low
Etiology of anemia of chronic disease
Body hides iron in form of ferritin to limit bacterial proliferation
How does sideroblastic anemia present
Low hemoglobin, high serum iron, basophilic stippling (in lead poisoning) and ringed sideroblasts
Hypersegmented neutrophils are a characteristic finding of
Megaloblastic anemia
Vit b12 def has __ mma and __ homocysteine; folate def has __ mma and __ homocysteine
High and high; normal and high
Hereditary spherocytosis is due to a deficiency in
Spectrin or ankyrin
Tx of spherocytosis
Splenectomy
Why can sickle pts have polyuria or nocturia
Due to sickling happening in inner medulla Of kidneys
Etiology of aplastic anemia
Failure of blood cell production due to destruction of bone marrow cells
Cafe au lait spots, short stature, radial/thumb hypoplasia/aplasia. What am I?
Fanconi anemia
Tx of aplastic anemia
Blood transfusion and stem cell transplantation
How does hydroxyurea work
It stimulates production of fetal hemoglobin
Polycythemia Vera is due to
Clinal proliferation of a pluripotent marrow stem cell due to a jak2 mutation
Epo in pcv
Normal
Tx of pcv
Hydroxyurea and ifn
Telltale sign of porphyria
Pink urine
What happens to reflexes in porphyria
Areflexia
5 ps of acute intermittent porphyria
Painful abdomen, port wine colored urine, polyneuropathy, psych disturbances, precipitated by drugs (cyp450 inducers, alcohol, starvation)
Auer rod think
AML
Most common childhood malignancy
ALL
AML on peripheral smear
Large myeloblasts with round or kidney shaped nuclei
Smudge cells are characteristic of
CLL
Philadelphia chromosome t(9,22) is associated with
CML
Which leukemia tends to present at age <13
ALL