Heme/Onc Boards Flashcards
Med study review for heme and onc sections
What is Wisckott-Aldrich?
X-linked; thrombocytopenia with small plts, eczema, immunodeficiency
What is TAR syndrome?
thrombocytopenia with absent radii (nml thumbs); high risk for bleeding in neonatal period due to plts < 30k
common medical history ass’d with ITP
recent viral illness or immunization (within past 1-6 wks)
blood smear findings in ITP
megathrombocytes
giant platelets on blood smear
Bernard-Soulier
blood counts in ITP
low plt, nml Hgb, nml WBC
bone marrow findings in ITP
normal to increased megakaryocytes
1st line tx of ITP
IVIG
TTP cause
ADAMTS13 antibodies
Kasabach-Merritt syndrome
destruction of plts in vacsular tumors (hemangioma) of the skin, liver, or spleen; aka hemangioma- thrombocytopenia syndrome
clinical features of vWD
AD; mild to moderated bleeding in mucosal surfaces or with procedures, heavy menses.
lab eval for vWD
decreased vWF antigen with proportional decreased in Factor 8 activity, decreased activity measured by ristocentin cofactor assay
treatment for vWD
DDAVP, aminocaproic acid, transexamic acid
thombocytopenia, abnormal platelets than do not aggregate in response to ristocetin but do aggregated in response to ADP, epi, or collagen
Bernar-Soulier syndrome
nml plt count, poor plt aggregation in response to ADP, epi, and collagen
Glanzamann Thrombasthenia
differentiate primary hemostatic problem from coag problem
1 = petechiae, coag = deep bleeds (hemearthrosis)
prolonged PT, nml PTT
aqu or ihn F 7 problem, milk vit K def, mild liver dz, therapeutic warfarin
prolonged PTT, nml PT, corrects with mixing study
F 8, 9 , 11, 12 def
prolonged PTT, nml PT, does not correct with mixing study
inhibitor present, like SLE antiCoag
What is hemophilia A, what does PT and PTT look like with this dz
F8 def, prolonged PTT with nml PT
X-linked factor clotting defs
8 & 9
AR factor clotting defs
11, 12, 13, 5,7, 10
what med can you give a kid with mild hemophilia A prior to tooth ext & what test should be done prior
DDAVP and DDAVP challenge
What is hemophilia B, how do you tx it?
F 9 def, tx with F9 concentrate
which F def doesn’t result in bleeding
F 12 def
vit K dep coag factors
10, 9, 7, 2, prot C, prot S
Skin necrosis with warfarin and why?
prot C def - no prot C to serve as anti-thrombotic agent during transient hypercoaguable state during initiation of warfarin, bridge with heparin
Cause of neonatal purpura fulminans in infancy
homozygous protein C or S def
single most common childhood malignancy
ALL
Synds at increased risk for ALL
downs, Ataxia telang, bloom, fanconi anemia, NF
pallor and limp in a child
ALL
CBC findings in ALL presentation
anemia, reticulocytopenia, +/- neutrophilia
BM bx with leukemic lymphoblasts
ALL
most impt predicitive factor for 2nd remission in ALL
length of time of 1st remission
Synds at increased risk for AML
downs, fanconi anemia, kostmann, PNH, bloom, NF
orbital or epidural chloroma
AML - collection of leukemia cells
auer rod
AML
t(9;22)
Philadelphia chromosome, CML
<2yo, splenomegaly, elevated fetal Hgb, thrombocytopenia, rash
juvenile myelomonocytic leukemia
tx for aplastic anemia (2)
immunosuppression with ATG, cyclosporine, + steroids; or BMT
blood products given to aplastic anemia pts should be _________ to prevent ________
leukoreduced, alloimunization
lab and cong anomalies ass’d with fanconi anemia
PANcytopenia, (AR, DNA repair imparied), absent or abnl thumbs, short staturs, abnl radii, micocephaly, cafe-au-lait, renal anomalies
fanconi anemia at risk for other malignancies
AML>ALL, hepatic, squamous cell carcinoma
3 MC types of red cell aplasias and differentiation
Parvo - school aged, rash, fever, arthropathy
TEC (trans erythroblastopenia of childhood) - 1-4 yo, normocytic RBCs
DBA (diamond blackfan) - <1 yo, macrocytic RBCs, congenital anomalies (SS, glaucoma, webbed neck, CHD, DD, renal abn, hypogonadism)
