Peds Hematology

Question 1

Acute Lymphocytic Leukemia (ALL) action?

Etiology?

Answer 1

immature lymph cells take over bone marrow, blood, other organs

unknown
higher in twins, tri 21, Klinefelter,
a/w Epstein-Barr, varicells

Question 2

ALL epidemiology?

Answer 2

most common childhood leuk (4-5yo)

2nd peak 50 yo

Question 3

ALL presentation?

Answer 3

B sxs, bruise/bleed
infections
Kids: joint pain
B-Cell: Abdominal Mass

Question 4

ALL diagnostics?

Answer 4

> =30% peripheral BLAST CELLS in marrow

immunophenotyping

Question 5

Hodgkin’s Lymphoma presentation?

Answer 5

Painless LAD (neck, supraclavicular and mediastinal)
B sxs
U 15 yo, 50 yo

Question 6

Hodgkin’s Lymphoma dx?

Answer 6

Reed-Sternberg cells (owl eyes) in LAD bx

(P) + Epstein-Barr virus

Question 7

Non-Hodgkin’s Lymphoma affects what cells?

Answer 7

B cells

Question 8

Non-Hodgkin’s Lymphoma presentation?

Answer 8

Painless LAD

Mets anywhere but U to head/neck, abd, lungs

Question 9

Hemophilia A effects what?

Answer 9

Factor VIII (deficiency)

Question 10

Hemophilia A dx?

Answer 10

Long PTT

PT, fibrinogen, platelet count/fxn = N

Question 11

Hemophilia A tx?

Answer 11

Factor VIII

Desmopressin (inc VIII)

Question 12

Hemophilia B effects what?

Who?

Answer 12

Factor IX (deficiency)

Males (x-linked)

Question 13

Hemophilia B dx?

Answer 13

Long PTT

PT, fibrinogen, platelet count/fxn = N

Question 14

Vit K Deficiency U caused by?

Answer 14

Diet/Malabsorb
Liver failure
Some ABX

Question 15

Vit K Deficiency dx?

Answer 15

Long PT, (P) PTT
Fibrinogen, platelet count = N
Vit K, II, VII, IX, X = low

Question 16

Thrombocytopenia is?

Answer 16

Low platelets

Question 17

Thrombocytopenia caused by?

Answer 17

ASA, NSAIDS
Decreased production: Low B12/Folate (U EtOH)
Destruction: Hepatosplenic, Autoimmune, Illness

Question 18

ITP caused by?

Answer 18

Platelet antibodies formed 2ndary to viral infection

U EBV or CMV

Question 19

ITP epidemiology?

Answer 19

Most C acquired bleeding dis in kids

Question 20

ITP presentation?

PE?

Answer 20

Healthy kid w/ easy bleeding (nose, gums) or bruising

Petechiae, muc memb bleed
No hepatospleomegaly or LAD

Question 21

ITP bad outcome?

Answer 21

Intracranial bleed

Question 22

ITP tx?

Answer 22

Self-limiting w/i 6 mo

Question 23

vWD is?

Answer 23

Most C congenital bleeding dis

Low vWD = unstable Factor VIII

Question 24

vWD dx?

Answer 24

Long PTT

PT = N

Question 25

vWD tx?

Answer 25

VIII | Desmopressin

Question 26

Henoch-Schonlein Purpura is?

Answer 26

IgA immune complex U post URI or strep

Question 27

Henoch-Schonlein Purpura presentation?

Answer 27

``` U 4-6yo Palpable purpura and petechiae U LE, Butt Arthralgia in knees/ankles P hematuria, kidney failure P abd pain before purpura ```

Question 28

Henoch-Schonlein Purpura tx?

Answer 28

Self-limiting

Question 29

Sickle Cell Anemia flairs result from?

Answer 29

``` Hypoxia Dehydration Extreme temps Acidosis Stress ```

Question 30

Sickle Cell Anemia presentation?

Answer 30

At 6 months (after Hgb F falls) ``` Painful Crisis (vascular occlusion) Aplastic or Hemolytic Anemia Hyposthenuria (unconcentrated urine) Leg ulcers Frequent infections (esp pneumo- and streptococcal) Hepatosplenomegaly Retinopathy ```

Question 31

Sickle Cell Anemia tx?

Answer 31

Pain control Hydroxyurea (chemo) Transfusions Stem cell transplant