Hematology

Question 0

Peripheral smear findings in HUS

Answer 0

Schistocytes and burr cells, minimal platelets

Question 1

Triad of hemolytic anemia, thrombocytopenia, and acute renal failure

Answer 1

HUS

Question 2

Fetal hemoglobin takes over at

Answer 2

12 weeks gestation (gone by 12 months)

Question 3

Red cell lifespan in newborn

Answer 3

45-60 days

Question 4

Hypersegmented PMNs

Answer 4

Megaloblastic anemia (B12, folate)

Question 5

Basophilic stippling

Answer 5

Lead poisoning

Question 6

Howell Jolly bodies

Answer 6

Functional asplenia

Question 7

Schistocytes

Answer 7

Microangiopathy

Question 8

Spherocytes

Answer 8

Spherocytosis, AIHA

Question 9

Target cells

Answer 9

Thalassemia, liver disease

Question 10

Teardrops

Answer 10

MDS

Question 11

Burr cells

Answer 11

Uremia vs. liver disease

Question 12

Mentzer index less than 12

Answer 12

Thalassemia

Question 13

Mentzer index above 12

Answer 13

Iron deficiency

Question 14

How to calculate Mentzer index

Answer 14

MCV/RBC

Question 15

Newborn screen only with hemoglobin F

Answer 15

Beta thalassemia major (tx with stem cell transplant)

Question 16

Goats milk is deficient in

Answer 16

Folate

Question 17

Vegans are deficient in

Answer 17

B12

Question 18

Mode of inheritance for spherocytosis

Answer 18

AD

Question 19

Normalcy tic anemia with reticulocytosis and increased MCHC

Answer 19

Spherocytosis

Question 20

Gold standard test to diagnose spherocytosis

Answer 20

Osmotic fragility

Question 21

Patients with spherocytosis s/p splenectomy still at risk for

Answer 21

Cholelithiasis

Question 22

Mode of inheritance of G6PD Deficiency

Answer 22

X linked AR

Question 23

Can cause G6PD problems

Answer 23

Fava beans, sulfonamides, antimalarials, moth balls

Question 24

Bite cells and blister cells

Answer 24

G6PD Deficiency

Question 25

Assay for CD59 or FLAER tests for

Answer 25

Paroxysmal Nocturnal Hemoglobinuria

Question 26

ELA2 gene, chronically low ANC, risk of AML/MDS, G-CSF is tx of choice, AR

Answer 26

Kostmann's Syndrome (severe congenital neutropenia)

Question 27

Severe neutropenia, GI symptoms, AR

Answer 27

Shwachman Diamond Syndrome

Question 28

Diagnose CGD with

Answer 28

NBT test

Question 29

AR, oculocutaneous albinism, neutropenia, prolonged bleeding, increased infections

Answer 29

Chediak Higashi

Question 30

IgE level over 1,000, cold skin abscesses 2/2 staph aureus, need bactrim ppx

Answer 30

Job Syndrome (hyper immunoglobulin E)

Question 31

Pentad of TTP

Answer 31

MAHA, neurologic symptoms, renal dysfunction, thrombocytopenia, fever

Question 32

Thrombocytopenia, absent radii, normal thumbs

Answer 32

TAR syndrome, AR

Question 33

X linked, thrombocytopenia, severe eczema, immunodeficiency, SMALL platelets,

Answer 33

Wiskott Aldrich. SCT is curative

Question 34

AR, thrombocytopenia with GIANT platelets

Answer 34

Bernard Soulier Syndrome

Question 35

Normal platelet count, severe bleeding and bruising

Answer 35

Glanzmann's Thrombasthenia. Tx with platelet transfusion

Question 36

Large hemangioma with DIC

Answer 36

Kasabach Merrit

Question 37

AR, no thumbs, short, anemia or thrombocytopenia, risk of AML

Answer 37

Fanconi Anemia. Defective DNA repair

Question 38

Normal red cell adenosine deaminase, HgB 6-8.

Answer 38

Transient Erythroblastopenia of Childhood. Self limited. May be 2/2 viral infection. Just monitor

Question 39

Short, webbed neck, heart disease, anemia, renal anomalies, hypotonia dims, glaucoma, MR. Abnormal red cell adenosine deaminase

Answer 39

Diamond Blackfan Anemia. SCT for steroid refractory pts

Question 40

Lyric punched out lesion on skull, external otitis that does not respond to antibiotics (draining ears). May have floating teeth or bad dermatitis

Answer 40

Langerhan's Cell Histiocytosis

Question 41

Hyperlipidemia, hyperferritinemia, fever, HSM, CNS symptoms

Answer 41

HLH (Hemophagocytic Lymphohistiocytosis). Tx with etoposide

Question 42

Mode of inheritance of Hemophilia A

Answer 42

X linked recessive