Hematology Flashcards
what is pernicious anemia
Pernicious anemia is a type of vitamin B12 anemia. The body needs vitamin B12 to make red blood cells. You get this vitamin from eating foods such as meat, poultry, shellfish, eggs, and dairy products. A special protein, called intrinsic factor (IF), binds vitamin B12 so that it can be absorbed in the intestines.
Why do you not need an iron/tibc in most cases?
ferritin <15 is 100% predictive of iron deficiency. dont need a tibc unless you suspect that the ferritin is an acute phase reactant.
how do homocysteine levels change when B12 is low?
homocysteine will be elevated because b12 is a cofactor for turning homocysteine into methylmalonyl coA
ddx for microcytic anemia
iron deficinecy, thalassemia, anemia of chronic disease, lead poisoning, sideroblastic anemia.
how does alcohol affect bone marrow
suppressed bone marrow. also is an antagonist for folate and thus can cause macrocytic anemia.
8yo boy from Cuba presents to ER with leg pain, jaundice, pale conjunctiva. Hb 82, MCV 89, platelets 321, wbc 8.0 and reitculocyte count of 8.
top diagnoses?
jaundice= hemolysis/bilirubin from blood breakdown.
sickle cell, g6pd hemolysis/spherocytosis/elliptocytosis,
need Hb electrophoresis (look for hemaglobinopathy) May need life long folate therapy.
what does haptoglobin tell you
Haptoglobin is used as an acute-phase marker of red blood cell (RBC) destruction. Its value decreases and may even be absent when RBCs are destroyed at twice the normal rate.
what iron studies would you see in anemia of chronic disease
normal ferritin, normal MCV
low/Normal iron
normal/low tibc
low/normal transferrin saturation
do you have splenomegaly in g5pd deficiency
no. It is an acute hemolytic process set off by drugs or fava beans or genetic.
low retic count anemia would indicate
hemolysis, bleeding, bone marrow issuse.
multiple myeloma classic symptoms
CRAB : calcium elevated, renal dysfunction, anemia, bone disease.
factor 5 leidin, protein c and s deficiencies are considered diseases of
hypercoagulability
PRIMARY vs secondary hemostasis
- platelet plug at site of injury
- coagulation cascade stabilizes and forms a clot and scab.
how does protein C and S deficiency cause hypercoaguability
protien C (vitamin K dependent) typically slows down coagulation by acting on thrombo-modulin to reduce thrombin activity. if there is not enough protein C, thormbin will be consistently activated and can cause clots to form.
explain the pathophysiology of factor v leidin
a misshapen binding site prevents the INactivation of factor 5, so it stays active and activates the coagulation cascade, and there is increased blood clot formation, causing deep vein thrombosis.
makeup of hb F
HbF is fetal, with two alpha and two gamma globin chains
HbA: two alpha two beta (adult)
Hb A2: Hb1A and 2 delta chains. (small amount of adult Hb)
HbH disease
excess beta chains that cause hemoglobin chains to clump together to form bea 4, and causes HbH. Causes hypoxia because it has high affinity and doesnt release O2. Causes splenomegaly.
blood test findings of alpha thalassemia
decreased hemaglobin
microcytic anemia
hypochromic
target cells.
target cells are seen in which heme condition
alpha thalassemia.
type of hemaglobin in hydrops fetalis
BBBB, super high affinity.
aabb= normal adult
aagg= fetal hb
bbbb= hydrops fetalis barts
aabSbS= sickle cell
HbA2= beta thalassemia.
beta thalassemia is a problem with:
beta chains of the aabb hb. it instead has two alpha and 2 delta. there is a missing of one or both beta globin chains.
findings of beta globin chain deficiency (beta thalassemia)
free alpha chains cause its incorrectly paired causes hemolysis and hepatosplenomegaly there jaundice and seocndary hemochromatosis.
ferritin levels in thalassemia
will be high especially if there is hemolysis. this isn’t an iron defiicency problem
what would you see on hb electrphoresis studies in beta thalassemia
high levels of HbA2 (hb containing no beta chains, just 2 a chains).
CML chromosome
associated with philedelphia. treatment with imatinib (TKI)and hydroxyuria
polycythemia vera diagnosis
JAK2 mutation
BM biopsy showing hypercellulairity
High HB.
Need to treat with blood letting and hydroxyurea and low dose ASA + allopurinol
most common lymphoid malignancy in children
ALL. second peak at age 40–bimodal distribution.
malignnat proliferation of lymphoid cells with Reed-Sternberg cells
Hodgkins Lymphoma. Bimodal distribution with peaks at 20 years and up to 50 years.
what type of leukemia are seen in smudge cells
cll. damaged smudge cells are artifacts of damaged lymphocytes on slide prep.
first line therapy is observation or rituximab or ibrutinib.
