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heme/onc Flashcards

1
Q

low reticulocyte count indicates what?

A

bone marrow failure or diminished hematopoiesis

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2
Q

clinical features of iron deficiency anemia in adolescents

A

spoon shaped nails and diminished attention and ability to learn

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3
Q

low serum ferritin results in INCREASED/DECREASED transferrin?

A

increased transferrin and decreased transferrin saturation

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4
Q

what should iron be ingested with?

A

vitamin C to enhance intestinal iron absorption

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5
Q

anemia characterized by defective synthesis of one of the Hgb Chains?

A

thalassemia

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6
Q

type of thalasemia prevalent in south east asians?

A

alpha thalassemia

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7
Q

two alpha glob in genes are deleted and have mild anemia

A

a thalassemia minor

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8
Q

three alpha glob in genes are deleted - patients have severe anemia at birth .

A

Hemoglobin H disease

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9
Q

what is hemoglobin Barts ?

A

seen in three alpha glob in genes deletion

binds oxygen very strongly doesn’t release it to tissue

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10
Q

four alpha glob in genes deleted resulting in only hgb harts formation

A

fetal hydrops

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11
Q

hepatosplenomegaly
bone marrow hyperplasia
maxillary hyperplasia w/ prominent cheekbones and skull deformities

A

b thalassemia major

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12
Q

what population has b thalassemia major?

A

mediterranean background

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13
Q

what is the treatment of beta thalassemia major?

A

lifelong transfusions and splenectomy

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14
Q

what is a complication of beta thalassemia major? what drug can help it?

A

hemochromatosis

deferoxamine

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15
Q

Beta thallasemia minor is commonly misdiagnosed as what?

A

iron deficiency anemia

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16
Q

whmild asymptomatatic anemia w/ hemoglobin level 2-3 below age appropriate norms and target cells and anisocytosis?

A

beta thalassemia minor

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17
Q

characterized by prussian blue staining resulting from accumulation of iron in mitochondria of RBC precursors in bone marrow ?

A

sideroblastic anemia

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18
Q

macrocytic megaloblastic anemias are characterized with MCV > ?

A

95

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19
Q

what are two major causes of macrocytic megaloblastic anemia?

A

folic acid and vitamin B 12 deficiency

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20
Q

vitamin b 12 must first combine with what?

A

instrinsic factor secreted by gastric parietal cells

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21
Q

where is vitamin b 12 absorbed?

A

terminal ileum

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22
Q

what is up w/ tongues in vitamin b 12 deficiency?

A

smooth red tongue and neurology manifestations

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23
Q

what is the treatment of vitamin b 12 def?

A

monthly IM vitamin b 12 injections

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24
Q

spectrin defect causing RBC membrane instability autosomal dominant?

A

hereditary spherocytosis

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25
splenomegaly, pigmentary gallstones, aplastic crises and high retic count ?
hereditary spherocytosis
26
what studies are used for hereditary spherocytosis ?
osmotic fragility studies
27
tx of hereditary spherocytosis
transfusions and splenectomy after 5 years of age
28
what are 2 enzyme deficiencies of RBS?
pyruvate kinase and G6PD
29
what does blood smear show in G6PD deficiency?
bite cells and hemighosts/ heinz bodies
30
what are some triggers of hemolysis in G6PD
fava beans, infection, drugs
31
positive direect coombs test indicates?
autoimuno hemolytic anemia
32
tx for autoimmune hemolytic anemia
corticosteroids and transufision
33
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
34
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
35
what is management for alloimmune hemolytic anemia?
phototherapy and exchange transfusion
36
cause SS
single amino acid substitution of valine for glutamic acid on number 6 position of B glob in chain of Hgb
37
what leads to distorted RBC shape in SS?
polymerization of HgB
38
2 genes for Hgb S?
SS disease
39
SS trait
1 gene for Hgb S
40
what is the hemoglobin makeup for those w/ SS trait?
Hgb A, Hg S and some HgF
41
diagnosis of SS disease?
Hg electrophoresis
42
what should you always suspect in a person presenting w/ priapism?
sickle cell
43
parvovirus B 19 can cause?
aplastic crisis ( SLAPPED CHEEKS)
44
leading cause of death from SS disease?
infection (sepsis or meningitis)
45
red blood life span SS
10-50 days
46
hemoglobin in SS
6-9 g/dL
47
hematocrit in SS
18-27
48
reticulocyte count in SS
5-15 %
49
abc count in SS
12,000-20,000
50
platelet count in SS
>500,000
51
bili in ss
increased
52
blood smear in SS
sickled cells, target cells, howell jolly bodies
53
bone marrow in SS
erythroid hyperplasia
54
treatment in SS?
hydroxyurea, oral penicillin, daily folic acid, routine immunizations, serial transcranial doppler ultrasound
55
other name for fanconi anemia?
congenital aplastic anemia
56
inheritance for fanconi anemia
autosomal recessive
57
what are the skeletal abnormalities of falcon anemia?
absence or hypoplasia of the thumb and radius
58
what are lab findings in pancytopenia?
decreased RBCs, leukocytes and platelets
59
sulfonamides, anticonvulstants, chloramphenical, HIV, EBV and CMV chemicals and radiation may cause ?
acquired aplastic anemia
60
increase in RBC's relative to total blood volume
polycythemia
61
apparent increase in RBC mass caused by a decrease in plasma volume
relative polycythemia
62
what is the most common cause of relative polycythemia
dehydration
63
prolonged PTT and normal PT w/ hemarthroses?
hemophilia A ( factor 8 deficiency)
64
management of hemophilia A
DDAVP
65
no hemarthrosis, mucosal bleeding, menorrhagia
vvon wile brand disease
66
diagnosis of vwf ?
ristocetin cofactor assay
67
management of vwf?
ddavp
68
factor 9 deficiency
hemophilia B
69
what is vitamin K responsible for?
factors II, VII, IX and X proteins C and S
70
what is PTT and PT in vitamin K deficiency?
prolonged in BOTH
71
tx for vitamin K def?
IM vitamin K prevents hemorrhagic disease
72
accelerated fibrinogenesis and fibrinolysis
DIC
73
thromboycytopenia, prolongation of PT and PTT, reduction in clotting factors and elevated fibrin (D - DIMER)
DIC
74
thrombocytopenia, unusually small platelets, excezma and defects in T and B cell immunity
wiscott aldrich syndrome
75
how can you distinguish between tar and fanconi?
TAR has a thumb !
76
what is the treatment for ITP?
IVIG
77
what causes ITP?
viral infection
78
what is the treatment for chronic ITP
splenectomy
79
when mom has thrombocytopenia
neonatal immune mediated thrombocytopenia
80
when mom doesn't have thrombocytopenia but still passes antibodies against fetuses platelets
isoimmune thrombocytopenia
81
kasabach merrit syndrome
when large hemangiomas sequester and destroy platelets
82
diminished ability of platelets to aggregate and forma clot bc of deficient adhesive glycoprotein IIB/IIIa
glanzmann's thrombasthenia
83
absent platelet membrane glycoproteins and vwf?
bernard soulier syndrome
84
noncyclic neutropenia with mild infections w/ low ANC
chronic benign neutropenia of childhood
85
kostmann syndrome ( severe congenital agranulocytosis
86
recurrent fever, oral ulcers, stomatitis diagnosis?
cyclical neutropenia diagnosed by serial neutrophil counts
87
short stature, immunodeficiency fine hair and neuropenia
cartilage hair hypoplasia syndrome
88
exocrine pancreatic insufficiency w/ short stature caused by metaphyseal chondrodysplasia and neutro penia w/ constant otitis media?
schwachman diamond syndrome

PEDIATRIC BRS (9 decks)

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