Heme Onc Flashcards Preview

Laughing Pedi > Heme Onc > Flashcards

Flashcards in Heme Onc Deck (132)
Loading flashcards...
0

physiologic nadir of HCT/Hb in baby

2 mos

1

Iron def treatment (kids and adols)

iron elemental 3-6 mg/kg/day in kids
adolescents 325mg daily / BID

2

How to distinguish Thal trait from Fe def?

thal trait: all RBC are very small so very small MCV and RDW is normal. MArked discrepancy between Hgb and MCV.
Proportional MCV/Hgb in iron def

3

Normal MCV:

80

4

1-4 year old with very low Hgb and no retic, with normal MCV
Dx and Tx?

Transient Erythroblastenia of Childhood (TEC)
No treatment needed

5

progressive, normochromic, macrocytic anemia in infancy or early childhood with some congenital anomaly / growth failure?
Dx
Tx

Diamond Blackfan anemia - bone marrow has no erthroid precursors
Tx: steroids, pRBCs, stem cell trp

6

transmission of hereditary spherocytosis?
Exam finding?
Tx needed

autosomal dominant
Splenomegaly, may need splenectomy after 5y
need folate supp

7

chronic stable hemolysis w/ exacerbations, runs in family, MCHC is HIGH

Hereditary Spherocytosis

8

Periph smear showing RBC agglutination in what type of autoimmune hemolytic anemia?

Cold IgM autoimmune HA

9

Direct Coombs shows what?

presence of Ab on RBCs

10

Warm vs cold autoimmune anemia?

Warm: IgG: bad, occurs at body temp, splenomegaly
Cold: IgM: Liver, good.(usu from mycoplasma, HIV, or mono)

11

hypochromasia and microcytosis in mild anemia, maybe scattered target cells should lead you to consider what?

thalassemia (less hemoglobin per RBC because decreased hemoglobin produced)

12

Thalassemia and iron?

expect increased GI absorption of iron, and get iron overload even w/o transfusion in severe thal

13

Hb A2 made of what and when do you see it?

alpha 2 and Delta 2: No B2...

14

Hb A has what?

normal: two alpha and two beta

15

alpha 2 + gamma 2 is what type of hemoglobin?

Fetal.

16

cure for thal major

BMT

17

four gamma chains called what and what is the disorder? what do they get in older yrs?

in nweborn you get 4 gammas because no alpha: alpha thal (Barts), as they get older and beta chains mature, you get B4: Hb H

18

Hemoglobin H disease?
In who?
What is the defect?

This is alpha thal with Asians
variable phenotype with mild/moderate hypochromatic, microcytic anemia
Also liver / spleen issues

19

Hemoglobin C disease?

Sickle cell

20

Hb SS
Hb SC
Hb S beta thal + dz
Most severe?

Hb SS. No Hb A present. A2 and F are normal, no excess alpha chains
Shortest life expectancy: 40s

21

S beta thal null disease course?

severe, No Hb A, incr A2 and F, Looks very similar to SS

22

WHat is dactylitis and what may it be found in?

swelling of hands / feet, may be first manifestation of sickle cell

23

weird bug in sickle cell osteomyelitis?

salmonella

24

lusaback merritt

hemangioma and low plts (consumed by hemangioma)

25

bernard soulier

GP1b def causing congenital qualitative plt defect

26

Fibrinogen in DIC and tx?

low, give cryo

27

purpose of WinRHo (Anti-D) in ITP?

for Rh+ kids w/ ITP, trick spleen into causing mild HA and attacking RBC to leave plts alone

28

Evans vs ITP

Evans has autoimmune attack of MORE than one cell line. ITP ONLY plts

29

% of ITP w/ chronic disease, and who?

20%, usu females, infants, adol