Hematology

Question 1

What is Idioapthic Thrombocytopenic Purpura (ITP)

Answer 1

Bleeding disorder that follows infection

IgM or igG antibodies cross-react against platelet surface protein, spleen destroys them, thrombocytopenia, see purpura

Question 2

Sx of ITP

Answer 2

Asymptomatic, mucocuteanous bleeds (purpura, bruises, petechia, epistaxis, gums)

Question 3

Dx of ITP

Answer 3

Isolated thrombocytopenia with normal coagulation studies

Question 4

Tx of ITP

Answer 4

Usually resove on own
Corticosteroids for non-life threatening bleeds (epistaxis, hematuria, hematochezia)
IVIG for severe acute bleeds
Platelet transfusion when extremely low platelet number

Question 5

What is Von Willebrand Disorder

Answer 5

Inherited bleeding disorder

Question 6

Sx of Von Willebrand Disorder

Answer 6

Fatigue, ecchymosis, epistaxis, menorrhagia, post-dental tonsillectomy bleeding

Question 7

Dx of Von Willebrand Disorder

Answer 7

Prolonged platelet function or mild prolongation of aPTT

PTT would correct with mixing study

Question 8

Tx of Von Willebrand Disorder

Answer 8

Desmopressin

OCP or IUD for menorrhagia

Question 9

What is Henoch-Schonlein Purpura (HSP)

Answer 9

A type of vasculitis

IgA immune complexes deposit in small vessels of kidney, skin, and intestinal walls

Question 10

Sx of HSP

Answer 10

Usually preceded by viral infection

Purpuric rash on dorsal surface, glomerulonephritis, hematuria, renal insufficiency, arthritis, abdominal pain

Question 11

Tx of HSP

Answer 11

Corticosteroids when intestinal involvement

Question 12

What are common features of AML

Answer 12

Adults 30-60
Auer Rods, >30% blasts
Tx: Cytarabine

Question 13

What are common features of CML

Answer 13

Adults 65yrs
Philadelphia Chromosome (good prognosis)
Tx: Imatinib

Question 14

What are common features of ALL

Answer 14

KIDS
Fatigue, pallor, easy bruising, hepatosplenomegaly, fever, bone pain, weight loss
Normochromatic/Normocytic anemia
Tx: Steroids, Vincristine, Methotrexate

Question 15

What are common features of CLL

Answer 15

Most common
Middle age and old
Lymphadenopathy, Hepatosplenomegaly
Well differentiated lymphocytes, High LDL
Tx: Chlorambucil

Question 16

What are common features of Hodgkin’s Disease

Answer 16

Peaks at 20 then again >50yrs
Associated with EBV
Painless lymphadenopathy, systemic B Sx
Reed Sternberg Cells (owl eyes)
Tx: Radiation, Chemo

Question 17

What factor is missing in Hemophilia A

Answer 17

Factor 8

Question 18

What pathway of clotting cascade is affected

Answer 18

Intrinsic

Question 19

Tx of Hemophilia A

Answer 19

Hemarthrosis, excessive hemorrhage, less commonly purpura/petechia

Question 20

Dx of Hemophilia A

Answer 20

Prolonged PTT, low factor 8, normal platelets, mixing study corrects PTT

Question 21

Tx of Hemophilia A

Answer 21

Desmopressin (transiently increases factor 8 and vWF)

Replace Factor 8

Question 22

What factor is missing in Hemophilia B

Answer 22

Factor 9

Question 23

Tx for Hemophilia B

Answer 23

Replace factor 9

NO DESMOPRSSIN - has nothing to do with factor 9

Question 24

Where do kids get Lead Poisoning

Answer 24

Flaking paint, artists paints, fruit tree sprays, pottery

Question 25

Sx of Lead Poisoning

Answer 25

Weakness, irritability, weight loss, vomiting, personality changes, ataxia, constipation, headache, developmnetal delays, convulsions

Question 26

Dx of Lead Poisoning

Answer 26

Screen all kids from 6 months-6yrs
Sx start at levels >80 mcg/dL
CBC: Normocytic, Hypochromic anemia with Basophilic stippling of RBC and reticulocytes

Question 27

What will CBC show with Lead Poisoning

Answer 27

Normocytic
Hypochromatic anemia
Basophilic Stippling of RBC and Reticulocytes

Question 28

What ages do you screen for Lead Poisoning

Answer 28

6months-6years

Question 29

Tx of Lead Poisoning

Answer 29

Succimer (chelator) - start when levels are >45

When encephalopathy is present need anticonvulsants, large dose of Vit.D