Red cell/White cell disorders, Leukemia/Lymphoma (Peds)

Question 1

The physiologic nadir for hemoglobin in infants occurs at approximately?

Answer 1

2 months of age

Question 2

What effect does anemia have on the hemoglobin-oxygen-dissociation curve?

This causes the affinity of hemoglobin for oxygen to become reduced in?

Answer 2

1) Concentration of 2,3-DPG increases within the RBC leading to the curve to shift to the right
2) Tissues needing to be oxygenated

Question 3

At what rate of development for anemia does the body better compensate it?

What rate usually results in more dramatic symptoms?

Answer 3

1) Slowly developing anemia

2) Rapidly developing anemia

Question 4

What does the MCV (mean corpuscular volume) tell you?

Answer 4

The mean volume of individual RBCs in a sample

Question 5

What does the MCHC (mean corpuscular hemoglobin concentration) tell you?

Answer 5

Grams of HGB per 100 mL of RBCs

Question 6

What does the RDW (Red blood cell distribution width) tell you?

Answer 6

Variability of RBC sizes in a sample

Question 7

What does an H/H (hemoglobin/hematocrit) lab test tell you?

Answer 7

1) The amount of hemoglobin in the blood

2) The volume percentage of red blood cells in blood

Question 8

What tells you if the RBCs are microcytic, normocytic, or macrocytic?

What tells you if the RBCs are hypochromic, normochromic, or hyperchromic?

Answer 8

1) MCV

2) MCHC

Question 9

What are general causes of anemia?

Answer 9

1) Decreased RBC production (in bone marrow)

2) Increased RBC destruction

Question 10

Hemoglobinuria, hemoglobinemia, and hemosiderinuria are due to what type of hemolysis?

Answer 10

Intravascular

Question 11

Splenomegaly and hepatomegaly are due to what type of hemolysis?

Answer 11

Extravascular

Question 12

Chronic blood loss will lead to what type of anemia?

Answer 12

Microcytic hypochromic anemia

Question 13

A low number of reticulocytes in a patient with anemia is indicative of?

Answer 13

Inadequate bone marrow response

Question 14

What is a normal bone marrow response to anemia?

Answer 14

Increased number of reticulocytes

Question 15

What is a congenital pure red blood cell aplasia that almost always presents in infancy?

Answer 15

Diamond-Blackfan anemia

Question 16

What does Diamond-Blackfan anemia result in?

Answer 16

Macrocytic anemia with low reticulocyte count

Question 17

The most common inherited form of aplastic anemia is?

Answer 17

Fanconi anemia

Question 18

What can fanconi anemia progress to?

We may not see symptoms until?

Answer 18

1) Pancytopenia

2) Around 10 years-old

Question 19

Microcytic, hypochromic anemia in infants and toddlers is most often due to?

This is most likely caused by?

Answer 19

1) Iron deficiency

2) Dietary lack of iron

Question 20

How could a peds patient present with iron deficiency anemia?

Answer 20

Pale and diet mostly made up of cow’s milk

Question 21

Mentzer index is most helpful in distinguishing mild IDA from?

Answer 21

Beta thalassemia trait

Question 22

If the Mentzer index is less than 13 it means?

If it’s greater than 13?

Answer 22

1) IDA

2) Beta thalassemia trait

Question 23

How is absolute neutrophil count calculated?

Answer 23

ANC = ((%neutrophils + %bands) X (WBC)) / 100

Question 24

Neutropenia occurs when a decrease in the absolute neutrophil count is less than?

Mild neutropenia: ANC = ?

Moderate neutropenia: ANC = ?

Severe neutropenia: ANC < ?

Answer 24

1) 1500/µL
2) 1000 - 1500
3) 500 – 1000
4) 500

Question 25

What would lead poisoning show on a peripheral smear?

Answer 25

Basophilic stippling

Question 26

If the ANC is < 500, the risk of what is high?

Answer 26

Serious infection

Question 27

Impaired myeloid differentiation caused by maturational arrest of neutrophil precursors causes what condition?

Answer 27

Kostmann syndrome (Severe Congenital Neutropenia)

Question 28

What are the clinical manifestations of Kostmann syndrome? What is the inheritance of Kostmann syndrome? Kostmann syndrome increases the risk of?

Answer 28

1) Life threatening pyogenic infections early in life 2) Autosomal recessive 3) AML

Question 29

Cyclic fever, oral ulcers, gingivitis, periodontal disease, and recurrent bacterial infections are clinical manifestation of what condition?

Answer 29

Cyclic neutropenia

Question 30

What is defected in cyclic neutropenia? What is the inheritance of cyclic neutropenia? What is the ANC for cyclic neutropenia?

Answer 30

1) Stem cell regulatory defect 2) Sporadic or autosomal dominant 3) ANC <200 for 3-7 days every 15-35 days

Question 31

Shwachman-Diamond syndrome is due to a triad of what?

Answer 31

1) Neutropenia 2) Exocrine pancreatic insufficiency 3) Skeletal abnormalities

Question 32

What is the inheritance of Shwachman-Diamond syndrome? Shwachman-Diamond syndrome increases the risk for? What is defected in Shwachman-Diamond syndrome?

Answer 32

1) Autosomal recessive 2) Myelodysplastic syndrome or leukemia 3) Neutrophil mobility

Question 33

Bone marrow failure syndrome with all cell lines affected results in?

Answer 33

Fanconi anemia

Question 34

What is the inheritance of Fanconi anemia? Fanconi anemia increases the risk for?

Answer 34

1) Autosomal recessive | 2) AML, brain tumors, and Wilms tumor

Question 35

What does Leukocyte adhesion deficiency result in?

Answer 35

1. Delayed separation of umbilical cord stump (>3 weeks) 2. Recurrent and severe bacterial and fungal infections without pus accumulation 3. Poor wound healing

Question 36

What is the inheritance of Leukocyte adhesion deficiency? What is the cause of Leukocyte adhesion deficiency?

Answer 36

1) Autosomal recessive | 2) Neutrophils have diminished adhesion to surfaces so they can't migrate out of blood vessels

Question 37

Hyperimmunoglobulin E syndrome (Job syndrome) causes a triad of?

Answer 37

1. Severe eczema 2. Recurrent bacterial infections (usually staph) of skin 3. Recurrent pulmonary infections (bacterial, fungal)

Question 38

What is the inheritance of Hyperimmunoglobulin E syndrome (Job syndrome)?

Answer 38

Sporadic or autosomal dominant

Question 39

What is a very rare autosomal recessive disease that causes partial oculocutaneous albinism, neuropathies, and recurrent pyogenic infections?

Answer 39

Chediak-Higashi syndrome

Question 40

Chronic granulomatous disease is due to recurrent purulent infections with? Chronic granulomatous disease is due to absent? What is the inheritance of Chronic granulomatous disease?

Answer 40

1) Fungal or bacterial catalase-positive organisms 2) Superoxide 3) X-linked recessive

Question 41

What virus causes neutropenia and causes a slapped cheek appearance?

Answer 41

Parvovirus B19

Question 42

DiGeorge syndrome is associated with an absent? This results in what type of immunodeficiency?

Answer 42

1) Thymus | 2) T-cell immunodeficiency

Question 43

The #1 cause of death of children due to illness is?

Answer 43

Brain Cancer

Question 44

The most common malignancy between age 15-19 years-of-age is?

Answer 44

Hodgkin Lymphoma (HL)

Question 45

What infectious agent may be involved with Hodgkin lymphoma?

Answer 45

EBV

Question 46

What are considered a pathognomonic feature of HL?

Answer 46

Reed-Sternberg cells

Question 47

What are the B-signs used in the staging of Hodgkin lymphoma?

Answer 47

1) Unexplained fever 2) Weight loss 3) Night sweats

Question 48

Any patient with persistent, unexplained LAD unassociated with an obvious underlying inflammatory or infectious process should undergo? Especially if?

Answer 48

1) CXR to rule out a mediastinal mass | 2) They display respiratory symptoms

Question 49

What is the inheritance of Wiskott-Aldrich syndrome? Wiskott-Aldrich syndrome predisposes a patient to?

Answer 49

1) X-linked recessive | 2) Non-Hodgkin lymphoma

Question 50

What is the classic triad of Wiskott-Aldrich syndrome?

Answer 50

1) Recurrent sinopulmonary and ear infections 2) Severe atopic dermatitis 3) Bleeding secondary to significant thrombocytopenia

Question 51

What condition commonly manifests as abdominal (sporadic type) or head and neck disease (endemic type) with involvement of the bone marrow or CNS?

Answer 51

Burkitt Lymphoma

Question 52

What account for the greatest percentage childhood malignancies?

Answer 52

Leukemias

Question 53

Trisomy 21 (Down Syndrome) is associated with what leukemias?

Answer 53

ALL and AML

Question 54

At what platelet count is clinically significant bleeding possible? When is life threatening hemorrhage possible?

Answer 54

1) Less than 20,000 μl | 2) Less than 10,000 μl

Question 55

The decreased platelet counts seen in Immune thrombocytopenic purpura is due to? The platelets that are present have what distinct feature? What precipitates ITP by about 1-2 weeks? What do you treat it with?

Answer 55

1) Anti-platelet antibodies 2) Large 3) Viral illness 4) Prednisone

Question 56

What is Kasabach-Merritt phenomenon due to? What is it associated with?

Answer 56

1) Thrombocytopenia and hypofibrinogenemia | 2) Giant hemangioma and intravascular coagulation

Question 57

What are the signs and symptoms that are “red flags” in patients with thrombocytopenia?

Answer 57

1) Pancytopenia 2) Raised LDH (elevated in cancer) 3) Associated new renal impairment

Question 58

Hemolytic Uremic syndrome presents as classic triad of?

Answer 58

1) Microangiopathic hemolytic anemia 2) Thrombocytopenia 3) Renal damage