Flashcards in heme/onc Deck (88):
low reticulocyte count indicates what?
bone marrow failure or diminished hematopoiesis
clinical features of iron deficiency anemia in adolescents
spoon shaped nails and diminished attention and ability to learn
low serum ferritin results in INCREASED/DECREASED transferrin?
increased transferrin and decreased transferrin saturation
what should iron be ingested with?
vitamin C to enhance intestinal iron absorption
anemia characterized by defective synthesis of one of the Hgb Chains?
type of thalasemia prevalent in south east asians?
two alpha glob in genes are deleted and have mild anemia
a thalassemia minor
three alpha glob in genes are deleted - patients have severe anemia at birth .
Hemoglobin H disease
what is hemoglobin Barts ?
seen in three alpha glob in genes deletion
binds oxygen very strongly doesn't release it to tissue
four alpha glob in genes deleted resulting in only hgb harts formation
bone marrow hyperplasia
maxillary hyperplasia w/ prominent cheekbones and skull deformities
b thalassemia major
what population has b thalassemia major?
what is the treatment of beta thalassemia major?
lifelong transfusions and splenectomy
what is a complication of beta thalassemia major? what drug can help it?
Beta thallasemia minor is commonly misdiagnosed as what?
iron deficiency anemia
whmild asymptomatatic anemia w/ hemoglobin level 2-3 below age appropriate norms and target cells and anisocytosis?
beta thalassemia minor
characterized by prussian blue staining resulting from accumulation of iron in mitochondria of RBC precursors in bone marrow ?
macrocytic megaloblastic anemias are characterized with MCV > ?
what are two major causes of macrocytic megaloblastic anemia?
folic acid and vitamin B 12 deficiency
vitamin b 12 must first combine with what?
instrinsic factor secreted by gastric parietal cells
where is vitamin b 12 absorbed?
what is up w/ tongues in vitamin b 12 deficiency?
smooth red tongue and neurology manifestations
what is the treatment of vitamin b 12 def?
monthly IM vitamin b 12 injections
spectrin defect causing RBC membrane instability autosomal dominant?
splenomegaly, pigmentary gallstones, aplastic crises and high retic count ?
what studies are used for hereditary spherocytosis ?
osmotic fragility studies
tx of hereditary spherocytosis
transfusions and splenectomy after 5 years of age
what are 2 enzyme deficiencies of RBS?
pyruvate kinase and G6PD
what does blood smear show in G6PD deficiency?
bite cells and hemighosts/ heinz bodies
what are some triggers of hemolysis in G6PD
fava beans, infection, drugs
positive direect coombs test indicates?
autoimuno hemolytic anemia
tx for autoimmune hemolytic anemia
corticosteroids and transufision
occurs when mom is Rh negative has Rh positive baby and forms Rh antibodies and subsequent pregnancies result in hemolysis of Rh positive babies? with a strongly positive coombs test
Rh hemolytic disease
this type of alloimmune hemolytic anemia can occur in the first pregnancy when mom is blood group O and fetus is blood group A, B, or AB? Moms antibodies are passed downy o baby causing hemolysis ?
ABO hemolytic disease
what is management for alloimmune hemolytic anemia?
phototherapy and exchange transfusion
single amino acid substitution of valine for glutamic acid on number 6 position of B glob in chain of Hgb
what leads to distorted RBC shape in SS?
polymerization of HgB
2 genes for Hgb S?
1 gene for Hgb S
what is the hemoglobin makeup for those w/ SS trait?
Hgb A, Hg S and some HgF
diagnosis of SS disease?
what should you always suspect in a person presenting w/ priapism?
parvovirus B 19 can cause?
aplastic crisis ( SLAPPED CHEEKS)
leading cause of death from SS disease?
infection (sepsis or meningitis)
red blood life span SS
hemoglobin in SS
hematocrit in SS
reticulocyte count in SS
abc count in SS
platelet count in SS
bili in ss
blood smear in SS
sickled cells, target cells, howell jolly bodies
bone marrow in SS
treatment in SS?
hydroxyurea, oral penicillin, daily folic acid, routine immunizations, serial transcranial doppler ultrasound
other name for fanconi anemia?
congenital aplastic anemia
inheritance for fanconi anemia
what are the skeletal abnormalities of falcon anemia?
absence or hypoplasia of the thumb and radius
what are lab findings in pancytopenia?
decreased RBCs, leukocytes and platelets
sulfonamides, anticonvulstants, chloramphenical, HIV, EBV and CMV chemicals and radiation may cause ?
acquired aplastic anemia
increase in RBC's relative to total blood volume
apparent increase in RBC mass caused by a decrease in plasma volume
what is the most common cause of relative polycythemia
prolonged PTT and normal PT w/ hemarthroses?
hemophilia A ( factor 8 deficiency)
management of hemophilia A
no hemarthrosis, mucosal bleeding, menorrhagia
vvon wile brand disease
diagnosis of vwf ?
ristocetin cofactor assay
management of vwf?
factor 9 deficiency
what is vitamin K responsible for?
factors II, VII, IX and X proteins C and S
what is PTT and PT in vitamin K deficiency?
prolonged in BOTH
tx for vitamin K def?
IM vitamin K prevents hemorrhagic disease
accelerated fibrinogenesis and fibrinolysis
thromboycytopenia, prolongation of PT and PTT, reduction in clotting factors and elevated fibrin (D - DIMER)
thrombocytopenia, unusually small platelets, excezma and defects in T and B cell immunity
wiscott aldrich syndrome
how can you distinguish between tar and fanconi?
TAR has a thumb !
what is the treatment for ITP?
what causes ITP?
what is the treatment for chronic ITP
when mom has thrombocytopenia
neonatal immune mediated thrombocytopenia
when mom doesn't have thrombocytopenia but still passes antibodies against fetuses platelets
kasabach merrit syndrome
when large hemangiomas sequester and destroy platelets
diminished ability of platelets to aggregate and forma clot bc of deficient adhesive glycoprotein IIB/IIIa
absent platelet membrane glycoproteins and vwf?
bernard soulier syndrome
noncyclic neutropenia with mild infections w/ low ANC
chronic benign neutropenia of childhood
kostmann syndrome ( severe congenital agranulocytosis
recurrent fever, oral ulcers, stomatitis diagnosis?
cyclical neutropenia diagnosed by serial neutrophil counts
short stature, immunodeficiency fine hair and neuropenia
cartilage hair hypoplasia syndrome