hemeatology Flashcards
sickle cell anemia soundbite, questions to ask, tx, causes of sickling
autosomal recessive disorder where a point mutation (Valine for Glutamic acid) causes sickling of RBCs and hemolysis
2) hospitalizations, txs, causes of crisis
3) Hydroxyurea increases HbF which prevents sickling, bone marrow transplant
4) periop/causes of sickling -
- low o2
- dehydraion
- acidosis
-exercise
-infection
-cold weather
-other medical diseases
- physical/psyc stress
-prego
avoid pain in these patients
complications of sickle cell anemia
1) aplastic crisis - b19
dactylitis - swelling offhand and feet
acute chest syndrome, pulmonary capillaries -
aseptic necrosis of femoral head
autosplenctomy
increased risk of infection due to encapsulated organisms because of autosplenctomy
pain crisis
splenic sequestration
how does heparin and LMWH (lovanox) work?
indirect thrombin inhibitors by up regulating antithrombin III which blocks prothrombin to thrombin.
see clotting pathway sheet for warfarin, heparin, mgmt of warfarin and heparin with TE, and other things related to the clotting pathways - memorize this stuff
see before.
Hemophilia soundbite, types, dx, types
x linked recessive bleeding disorder
Hemophilia A - factor 8 (AAeight)
Hemophilia B - factor 9 (benign, B9)
3) dx - family history, intrinsic pathway will be longer (PTT), and can measure factor levels if below 40% for the respective then is that hemophilia
4) types severe - less than 1% of factor
moderate - 1-5%
mild 5-40% factor
tx for hemophilia
1) discuss with hematologist
2) avoid cox inh, NSAIDs and asa
3) give recombinant factors and maintain for 1-2 weeks after procedure
4) txa - topical? (inh plasminogen which helps clots remain stable)
5) DDAVP - increases factor 8 and vwf (use in vwd too)
vonWillebrand Disease soundbite, what is vonwillebrand factor, types
1) platelet bleeding disorder due to deficiency or dysfunction of von Willebrand factor (usually congenital but can be acquired) often autosomal dominant inheritance.
2) vWF responsible for mediating platelet adhesion, adhesion to the endothelium and prevention of degradation of factor 8
3) Type 1 - partial quantitative
type 2 - qualitative (further divided)
type 3- total, near total quantitative (autosomal recessive)
vwd labs
CBC,pt put usually normal
ristocetin test
vWF antigen will see decrease
Factor 8 activity will see decrease.
treatment for types of vwd
type 1 - ddavp releases vwf stores from the endothelial cells and increases factor 8 (can be used in hemophilia 8 as well) dosage: 0.3 micrograms/kg over 20 min IV, intranasal 150 micrograms spray each nostril. risk of hyponatremia and siezures so watch free water intake, subcu is an option too
type 2 - ddavp for some type 2
Cryoprecipitate can be used for all forms of vwd - has factor 8,9,vwf, fibrinogen, fibronectin
Factor 8 concentrate is rich in vWF
- humate P (vwf/factor 8) concentrate
- Amicar - blocks plasminogen
pro-thrombotic diseases, thrombophilic diseases, mechanisms of each and general treatment of these diseases/with the surgical management
1) antithrombin III def - antithrombin III inhibits thrombin, factor 10a and 9a. therefore when there is a def of antithrombin 3 there is increased coagulation.
2) protein C deficiency - protein c inhibits factor 5,8,13 therefore if it is missing then increased coagulation.
factor V leiden - factor V (5) is a cofactor to xa, and is cleaved by protein c,
3) factor V leiden resists cleavage by protein c causing a hypercoagulable state.
most problematic forms have some version of anticoagulation (warfarin etc) that may need to be managed for surgery.
multiple myeloma soundbite, labs, comorbidities, tx
A plasma cell malignancy characterized by production of monoclonal immunoglobulin and bone destructive lesions
labs: protein electrophoresis detects monoclonal proteins
comorbidities - anemia, bone pain, renal failure due to light chain nephropathy, hypercalcemia due to osteolysis
tx: prolia/denosumab, stem cell transplant, chemo, radiation focal bone lesions not responsive to chemo.
lymphoma soundbite, types, tx
lymphoma is a malignancy of lymphocytes that arises in lymph nodes
both with b symptoms of night sweats, fevers, weight loss.
types:
Hodgkins - characterized by the reed sterner malignant cells, tx with chemo/rads
non Hodgkins - cells express CD20 - chemo/rads/stem cell transplant for tx
microcytic, microcytic and normocytic anemias
microcytic - iron def, and thalassemia,
macrocytic - folate and b vitamin def. , alcoholic liver disease leading to folate def
normocytic - anemia of chronic disease, acute blood loss (hemorrhage), aplastic anemia,
hemolytic anemias - autoimmune hemolytic anemia, and hereditary spherocytosis (also a hemolytic anemia)
Thalassemia soundbite, types
autosomal recessive anemic disorders due to the abnormal production of globin chains
types are alpha and beta
