Heme- Anemia, Coag, Malignancy Flashcards
X-linked recessive heme disorders
Hemophilias
G6PDD
Thalassemia is a(n) ______ inherited disorder
autosomal recessive
Sickle Cell Anemia is a(n) ______ inherited disorder
autosomal recessive
Hereditary Spherocytosis is a(n) ______ inherited disorder
autosomal dominant
Von Willebrand Disease is a(n) ______ inherited disorder
autosomal dominant
Hemophilia A is a(n) ______ inherited disorder
x-linked recessive
MCC of Anemia worldwide
Iron Deficiency Anemia
MCC of iron defic anemia
Blood loss
-GI, menstrual, blood donation
hypochromic microcytic on smear
decreased serum Fe, serum ferritin
increased TIBC
Iron Deficiency Anemia
c/o weakness, irritability, decreased concentration microcytic hypochromic anemia exam with glossitis w/o dx
Vit B6 defic anemia
dx via plasma pyridoxal phosphate
plasma pyridoxal phosphate used to dx what defic?
Vit B6
how to dx folate def anemia
RBC folate level
patho of aplastic anemia
bone marrow failure from suppression/injury to hematopoietic stem cells
pancytopenia and bone marrow bx that demonstrates hypocellularism
aplastic anemia
micryocytic hypochromic anemia
micryocytosis that is out of proportion to the anemia
Thalassemia
Alpha thalassemia is d/t gene ______
deletions
more alpha globin chains not transcribed means worse sxs
Heinz bodies
G6PDD
fava beans, antimalarials, sulfa, ASA
G6PDD
hyperchromic microcytosis with spheroctyes and increased retic on peripheral smear
splenomegaly
heredity spherocytoss
hemophilia A is deficiency of factor ______
inherited ______
prolonged_____
tx with ______
A=8 xlinked recessive prolonged PTT tx with factor VIII, DDAVP for minor bleeds cox2 (Celebrex) for joint pain
hemophilia B is deficiency of factor ______
inherited ______
prolonged_____
tx with ______
B=9
inherited linked recessive
prolonged PTT
tx with factor IX, DDAVP for minor bleeds
Christmas disease
hemophilia B, factor 9 def
“Hemophlia C”
factor XI def
hemophilia “C” is deficiency of factor ______
inherited ______
prolonged_____
tx with ______
C= 11 AUTOSOMAL recessive, Ashkenazi Jews prolonged PTT tx with FFP aminocaproic acid for procedures
MC inherited bleeding disorder
von Willebrand Dis
von Willebrand had what kind of bleeding?
platelet type:
petechial, gingival bleeding, epistaxis bruising
tx vWF dis
DDAVP
~vwf Concentrate
TMA pentad
microagniopathic hemolytic anemia thrombocytopenia fever renal insuff neruo abnl
adamst13
TTP
tx TTP
plasma exchange immed
NO platelets
monitor warfarin with
PT/INR
reversal of heparin
protamine sulfate
monitor heparin with
aPTT
MC inheritable cause of hypercoag
Factor V Leiden
how to dx sickle cell
electrophroesis
tx PV
phlebotomy
hydroxyurea
ASA
JAK2 mutation
PV
pruritus following warm shower
PV
Philadelphia chromosome
CML
isolated lymphocytosis
smudge cells
CLL
auer rods
AML
bone marrow hypercellular with blass
(+) sudan black esterase strain
AML
terminal deoxynuceltidyl transferase
ALL
MC leukemia of children
ALL
Reed Sternberg cells
Hodgkin lymphoma
bence jones proteins in urine
MM
xray with punched out lytic lesions
MM