14: Pediatric WBC And RBC Disorders

Question 1

What and when is the physiologic nadir in infants?***

Answer 1

Hb levels drop off steeply, looking like anemia, in infants at about 2 months of age

Question 2

Describe anemia’s effect on the Hb-O2 dissociation curve

Answer 2

2,3-DPG increases in RBCs -> O2 dissociation curve shifts right -> affinity of Hb for O2 is reduced in tissues

Question 3

What is important to ask about FHx in pt with anemia

Answer 3

FHx anemia, splenomegaly, jaundice, early onset gallstones

Question 4

Clinical sx of anemia in children

Answer 4

Sleepiness, irritability, decreased exercise tolerance, pallor, bloody stool/urine, LAD, fever, viral sx, pain, SOB, cough

Question 5

If anemia occurs along with other cell line issues, what is the likely culprit?

Answer 5

The bone marrow

Question 6

Acute vs chronic blood loss: cell type in anemia

Answer 6

Acute loss: normocytic RBCs

Chronic: microcytic hypochromic (bc Fe deficiency)

Question 7

ANC that represents mild neutropenia

Answer 7

1000-1500

Question 8

What immune deficiency occurs with DiGeorge syndrome?

Answer 8

T cell deficiency (bc absent thymus)

Question 9

1 and #2 causes of death for children due to ILLNESS in the US***

Answer 9

1. Brain cancer

2. Leukemias

Question 10

Overall #1 cause of death not related to illness in children in the US

Answer 10

Unintentional injuries / accidents

Question 11

Most common malignancy in pts 15-19 years old

Answer 11

Hodgkin Lymphoma

Question 12

Three viruses related to development of Hodgkin Lymphoma

Answer 12

1. HHV-6
2. Cytomegalovirus
3. EBV

Question 13

Three B signs of Hodgkin Lymphoma

Answer 13

1. Fever of 102.2 +
2. weight loss of >10% total body weight over 6 mo
3. Drenching night sweats

Question 14

Indications for a CXR in a pt with LAD

Answer 14

Pt with unexplained, persistent LAD + obvious underlying inflammatory process OR with LAD and respiratory sx-> CXR to r/o mediastinal mass

Question 15

Most common lymphoma in children and adolescents

Answer 15

non-Hodgkin lymphoma

Question 16

Classic triad of Wiscott-Aldrich syndrome

Answer 16

1. Recurrent sinus/pulmonary/ear infections
2. Severe atopic dermatitis
3. Bleeding bc significant thrombocytopenia

Question 17

What CA is linked to Wisconsin-Aldrich syndrome

Answer 17

Lymphoma, esp. B cell

Question 18

Discuss 3 types of Burkitt’s lympoma

Answer 18

1. Sporadic: abdominal dz, +/- EBV, most in Africa
2. Endemic: head and neck dz, assoc w EBV and malaria, most in Africa
3. Immunodeficiency-related: assoc with immunosuppressive drugs

Question 19

What % of all childhood leukemias are acute?

Answer 19

97%

Question 20

Trisomy 21 (Down Syndrome) is associated with which two leukemias?

Answer 20

ALL, AML

Question 21

Presentation of thrombocytopenia

Answer 21

Petechiae, purpura, gingival bleeding, epistaxis, menorrhagia, GI bleeding, hematocrit, CNS hemorrhage, ecchymoses in excess

Question 22

Three red flags in pts with thrombocytopenia

Answer 22

1. Evidence of pancytopenia
2. Raised LDH (high in CA)
3. Associated new renal impairment

Question 23

Condition to consider in a child who’s pale + big milk drinker

Answer 23

IDA

Question 24

Describe the Mentzer index

Answer 24

Measures MCV/RBC

1. <13 = Beta thalassemia
2. > 13 = IDA

Question 25

Other name for Kostmann syndrome

Answer 25

Severe congenital neutropenia

Question 26

Three CA that are increased risk in Franconia anemia

Answer 26

1. AML
2. Brain tumors
3. Wilms tumor

Question 27

Five causes of pancytopenia to consider in a differential

Answer 27

1. Primary malignancy
2. Bone marrow infiltration
3. Viral infection
4. Cytotoxic drugs
5. Radiation

Question 28

Treatment for ITP in children

Answer 28

Usually self-resolves with supportive care, may use prednisone when severe