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Flashcards in heme/onc Deck (88):
1

low reticulocyte count indicates what?

bone marrow failure or diminished hematopoiesis

2

clinical features of iron deficiency anemia in adolescents

spoon shaped nails and diminished attention and ability to learn

3

low serum ferritin results in INCREASED/DECREASED transferrin?

increased transferrin and decreased transferrin saturation

4

what should iron be ingested with?

vitamin C to enhance intestinal iron absorption

5

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

thalassemia

6

type of thalasemia prevalent in south east asians?

alpha thalassemia

7

two alpha glob in genes are deleted and have mild anemia

a thalassemia minor

8

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

Hemoglobin H disease

9

what is hemoglobin Barts ?

seen in three alpha glob in genes deletion

binds oxygen very strongly doesn't release it to tissue

10

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

fetal hydrops

11

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

b thalassemia major

12

what population has b thalassemia major?

mediterranean background

13

what is the treatment of beta thalassemia major?

lifelong transfusions and splenectomy

14

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

hemochromatosis
deferoxamine

15

Beta thallasemia minor is commonly misdiagnosed as what?

iron deficiency anemia

16

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

beta thalassemia minor

17

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

sideroblastic anemia

18

macrocytic megaloblastic anemias are characterized with MCV > ?

95

19

what are two major causes of macrocytic megaloblastic anemia?

folic acid and vitamin B 12 deficiency

20

vitamin b 12 must first combine with what?

instrinsic factor secreted by gastric parietal cells

21

where is vitamin b 12 absorbed?

terminal ileum

22

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

smooth red tongue and neurology manifestations

23

what is the treatment of vitamin b 12 def?

monthly IM vitamin b 12 injections

24

spectrin defect causing RBC membrane instability autosomal dominant?

hereditary spherocytosis

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