Hematology

Question 1

What is the threshold of defining anemia?

Answer 1

HCT or HGB at or below the 2.5th percentile for age, race, and sex (typically somewhere below 11-11.5)

Question 2

What are causes of anemia of neonates to 3 months?

Answer 2

Hyperbilirubinemia
Chronic intrauterine blood loss
Thalassemia

Question 3

What are causes of anemia from 3 months to 6 months?

Answer 3

Sickle cell

Thalassemias

Question 4

What are causes of anemia from those greater than 6 months?

Answer 4

Nutritional deficiencies such as iron deficiency anemia

-consider menses in females

Question 5

In healthy term infants, what is the hemoglobin level?

Answer 5

HIGH

>14 g/dL

Question 6

When does the the hemoglobin in healthy term infants drop?

Answer 6

6-9 weeks (11g/dL)

Question 7

What do pencil poikilocytes, target cells, hemoglobin H or F indicate?

Answer 7

Thalassemia

Question 8

What do pencil poikilocytes, microcytic hypo chromic smear and low serum ferritin indicate?

Answer 8

Iron deficiency anemia

Question 9

What do bite cells, Heinz bodies, multiple nucleated RBCs, Red blood agglutination indicate?

Answer 9

Hemolytic anemia (G6PD)

Question 10

What do Howell-Jolly bodies indicate?

Answer 10

Splenic disorders

Question 11

What does basophilic stippling indicate?

Answer 11

Lead poisoning

Question 12

What is the most common inherited bleeding disorder?

Answer 12

Von Willebrands disease

Question 13

What is the function of vWF?

Answer 13

Aggregates PLTs and prolongs the half life of F. VII

Question 14

What is Type 1 vWB disease? And how does it present in patients?

Answer 14

The pt does not make sufficient VWF quantity; they present with mild to moderate PLT-type bleeding (skin and mucosal)

Question 15

What is Type 2 vWB disease? And how does it present in patients?

Answer 15

qualitative defect in vWF; moderate to severe bleeding that presents in childhood or adolescence

Question 16

What lab values diagnose vWB disease?

Answer 16

PLT normal or decreased 
PT, Fibrinogen is normal 
PTT normal or prolonged 
Bleeding time is prolonged 
*Depressed vWF activity

Question 17

What is the tx for vWF disease?

Answer 17

DDVAP
Antifibrinolytics
Cryoprecipitate if severe

Question 18

What is the most common childhood cancer?

Answer 18

Acute Leukemia

Question 19

A child is presenting with hepatosplenomegaly, anorexia, lymphadenopathy (nontender, firm, rubbery), gingival hyperplasia, abnormal bruising, and musculoskeletal pain. Lab results reveal pancytopenia. How do you confirm your suspected diagnosis?

Answer 19

Bone marrow biopsy and aspiration : >20% blasts

Question 20

A child is presenting with hepatosplenomegaly, anorexia, lymphadenopathy (nontender, firm, rubbery), gingival hyperplasia, abnormal bruising, and musculoskeletal pain. Lab results reveal pancytopenia. There are elevated serum phosphates as well. What is the best treatment?

Answer 20

Chemotherapy (Vincristine) + steroids

*Once in remission, a bone marrow transplant should be performed

Question 21

What is the most common cancer in the 15-19 year age group?

Answer 21

Hodgkin Lymphoma

Question 22

A 16 year old patient presents with painless cervial lymphadenopathy. She has a fever, has lost weight, and complains of drenching night sweats and pruritis. How would you diagnose this patient?

Answer 22

Lymph node biopsy = REED-STERNBERG CELLS

Question 23

What is stage III Lymphoma?

Answer 23

Lymph node region involved on both sides of the diaphram

Question 24

A 16 year old patient presents with painless cervial lymphadenopathy. She has a fever, has lost weight, and complains of drenching night sweats and pruritis. A biopsy reveals Reed-Sternberg cells. What is the treatment for this patient?

Answer 24

If caught early, in stage 1: radiation

If 2+ : radiation + Stanford 5 Chemo

Question 25

Which type of cancer typically presents as a high grade cancer, such as: tamponade, vena cava obstruction, intestinal obstruction, airway obstruction?

Answer 25

Non-Hodgkin lymphoma

Question 26

How do you diagnose Non-Hodgkin lymphoma?

Answer 26

Lymph node biopsy Elevated uric acid Elevated LDH

Question 27

What is the tx for Non-Hodgkin lymphoma?

Answer 27

Radiation, Standord 5 | Bone marrow transplant if remission is acheived

Question 28

What is the treatment for Hemophilia A?

Answer 28

``` F. VIII concentrates (Cryoparticipate) Recombinant VIII DDVAP Antifibrinolytic agents Cox 2 inhibitors ```

Question 29

What do you want to avoid in patients with Hemophilia A?

Answer 29

Aspirin--it prolongs bleeding

Question 30

What is Hemophilia A?

Answer 30

Congenital deficiency of F. VIII | X linked recessive inheritance

Question 31

If an infant experiences "spontaneous" bleeding into joints, soft tissues or other locations--what should you suspect?

Answer 31

Hemophilia A -as adults have significant joint disabilities

Question 32

What lab values are consistent with Hemophilia A?

Answer 32

``` Bleeding time normal PT normal Fibrinogen normal PTT prolonged* Low factor VIII Normal vWF ```

Question 33

If a patient with mild Hemophilia A/B is bleeding during a dental procedure, what is the best treatment?

Answer 33

Antifibrinolytics

Question 34

What is Hemophilia B?

Answer 34

Congenital deficiency of factor IX | X linked recessive

Question 35

What should you watch out for with Hemophilia B children?

Answer 35

They present the exact same way as a Hemophilia A patient

Question 36

What lab values diagnoses Hemophilia B?

Answer 36

``` Low factor IX, vWF normal PTT prolonged PT normal Bleeding time normal Fibrinogen normal ```

Question 37

What is the tx for Hemophilia B?

Answer 37

FFP (Pt receives all other clotting factors as 9 and can be more prone to clotting)

Question 38

If a patient with mild Hemophilia A/B has arthritis, what is the best treatment?

Answer 38

COX 2 inhibitor

Question 39

An Ashkenazi Jew has just has a lap chole and has had some mild prolonged bleeding. Based on this, what is the patients genetic condition?

Answer 39

Factor XI deficiency Hemophilia C Autosomal recessive

Question 40

If a patient has low XI, what can you expect their PTT to be? Normal or prolonged?

Answer 40

Prolonged

Question 41

What is the tx for Hemophilia C?

Answer 41

FFP and Amicar (fibrinolytics)

Question 42

What sxs are associated with lead poisoning?

Answer 42

Anemia Hearing loss Peripheral neuropathy *decreased IQ and cognitive deficiency

Question 43

At higher levels, what do you see with lead poisoning?

Answer 43

Seizures and encephalopathy

Question 44

When does lead screening take place?

Answer 44

12 months

Question 45

At what level on a finger stick is a formal blood draw required?

Answer 45

over 5

Question 46

At high levels, what is the tx for lead poisoning?

Answer 46

Chelation