heme Flashcards
What is a heinz body?
precipitants formed in the red cell by the denatured oxidized hemoglobin. denatured usually thru oxidative damage
What diseases do you see heinz bodies?
G6PDehydrase deficiency, NADPH oxidase deficiency, alpha thalasemia or asplenia
type of stain for heinz body?
cresyl violet
hallmark of Waldenstrom macroglobulinemia
the presence of monoclonal IgM spike seen on electrophoresis in beta or gammaglobulin region.
heinz bodies in G6PD lead to
bite cells
general description of pnh
an acquired clonal stem cell disorder where the red blood cell membrane is sensitive to lysis by complement. Test with sugar water test and ham test.
how does serum ferritin look in a person with iron def anemia vs a person with anemia of chronic disease? (both are microcytic)
feritin would be low in a person with iron def but ferritin would be normal to elevated in a person with anemia of chronic disease.
how does serum iron look in a person with iron def vs ACD vs sideroblastic anemia?
iron would be decreased in both iron def and ACD but markedly increased in a person with sideroblastic anemia
Bernard Soulier is characterized by?
thrombocytopenia (easy bruising, nosebleeds, mucosal bleeding, menorrhagia), prolonged bleeding time and LARGE platelets.
Does Bernard Soulier respond to Ristocetin?
no, it responds to ADP, epinephrine, and collagen. (no response to ristocetin means that platelets do not aggregate in its presence). this is because since GP1b is missing (the anchor for vWF, there will be no aggregation).
In what disease is platelet glycoprotein IIa/IIIb complex deficient?
Glanzmann thrombasthenia. leads to defective platelet aggregation.
Von willenbrand disease is characterized by
prolonged BT,PTT mildly elevated, Platelets normal, PT normal. No response to ristocetin (means platelets do not aggretate in its presence).
What do the platelets in Glanzmann thrombasthenia respond to?
normal response to ristocetin, decreased response to ADP, epinephrine, and collagen
How does Glanzmann thrombasthenia present?
As a platelet dysfunction: excessive bleeding petechiae, ecchymoses, high bleeding time, PT, PTT, and platelet counts are normal. GIIb/IIIa is either deficient or dysfunctional.
How would I treat a bleeding pt that has Glanzmann?
Platelet transfusion
LAP in Leukomoid Reaction
High LAP in Leukomoid Re-AC-tion
LAP in CML
Low. Cancer makes you feel down, SLAP DOWN. LAP is down.
direct Coombs Test tests for….
autoimmune hemolytic anemia
In certain diseases an individual’s blood may contain IgG antibodies that can specifically bind to antigens on the RBC surface membrane, and their circulating RBCs can become coated with IgG alloantibodies and/or IgG autoantibodies. Complement proteins may subsequently bind to the bound antibodies and cause RBC destruction. The direct Coombs test is used to detect these antibodies or complement proteins that are bound to the surface of red blood cells;
Cold Agglutinin pathogens
Chlamydia, Mycoplasma - leads to walking pneumonia –> autoimmune hemolytic anemia.
cold agglutinins, which are usually due to IgM antibodies that react with polysaccharide antigens on the RBC surface at temperatures below the core temperature of the body and can be associated with both RBC agglutination and hemolytic anemia.
Warm Agglutinin
(AIHA) due to the presence of warm agglutinins is almost always due to IgG antibodies that react with protein antigens on the red blood cell (RBC) surface at body temperature.
Drugs that lead to autoimmune hemolytic anemia
penicillin, cephalosporins, sulfa drugs, quinine, NSAIDs
symptoms of PNH?
thrombosis and morning hematuria
what is HIT?
an immune complex mediated disorder that manifests as thrombosis and decreased platelet count (30,000-55,000) 4-10 days after heparin is started.
HIT is caused by the formation of abnormal antibodies that activate platelets.
direct acting thrombin inhibitors for use instead of heparin?
argatroban and lepirudin
itp presentation
thrombocytopenia, possibly post viral, or drug use
ttp/hus
pentad of thrombocytopenia, hemolytic anemia, renal probs, altered mental status, fever.
hus - e coli O157
theory of warfaren induced necrosis
factor c and s (anticoagulants) are depleted faster/first so this causes an imbalance with 2, 7, 9, 10 (clotting factors/coagulants) so clotting keeps blood from reaching skin and the necrosis occurs
what is bernard soulier
deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor, which is important in clot formation.
the indirect coombs test tests
The indirect Coombs test is used in prenatal testing of pregnant women, and in testing blood prior to a blood transfusion. It detects antibodies against RBCs that are present unbound in the patient’s serum. In this case, serum is extracted from the blood sample taken from the patient. Then, the serum is incubated with RBCs of known antigenicity; that is, RBCs with known reference values from other patient blood samples. If agglutination occurs, the indirect Coombs test is positive.
treatment for acute intermittent porphyria
hematin
treatment for ITP
prednisone until platelet count goes back up. then taper off.
splenectomy, ultimate definitive therapy
platelet transfusions are very rare because they survive for less than a few hours
IVIG is too expensive and only used for a person who wont stop bleeding
