Non Neoplastic WBC Disorders and Leukemias and Lymphomas

Question 1

Order the WBCs in decreasing order of amount?

Answer 1

NLMEB

Question 2

How do you calculate the absolute neutrophil count out of a CBC?

Answer 2

(%Neutrophils + %Bands) x (WBC)/100

Question 3

How do we define neutropenia by value, and what is mild, moderate and severe neutropenia?

Answer 3

Less than 1500
Severe is less than 500
Moderate is between 500 and 1000
Mild is between 1000 and 1500

Question 4

At what value do we say the risk of serious infection is high?

Answer 4

Less than 500 of ANC

Question 5

What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Kostmann Syndrome?

Answer 5

Life threatening pyogenic infections
Maturational arrest of neutrophil precursors
Autosomal recessive
Bone marrow shows myeloid arrest, 60-80% have neutrophil ELA2 mutation
AML

Question 6

What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Cyclic Neutropenia?

Answer 6

Cyclic fever, mouth problems like ulcers gingivitis, recurrent bacterial infections
Stem cell regulatory defect in maturation
Sporadic or autosomal dominant
CBC 2-3 times weekly for 6-8 weeks to document cycles. ELA2 mutation
No increased risk

Question 7

What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Shwachman Diamond Syndrome?

Answer 7

Triad of neutropenia, exocrine pancreas insufficiency, and skeletal problems
Defects in neutrophil migration and movement
Autosomal recessive
Neutropenia, low trypsinogen, bone problems, SBDS mutation
MDS or leukemia

Question 8

What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Leukocyte Adhesion Defieciency?

Answer 8

Delayed separation of umbilical cord, recurrent and severe bacterial infection and fungal infections without pus, and poor wound healing
Neutrophils have diminished adhesion and cannot migrate out of vessels
Autosomal recessive

Question 9

What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Hyper IgE syndrome/job syndrome?

Answer 9

Severe eczema, bacterial infections of lungs and skins, fungal infection
Neutrophil chemotaxis sucks
Sporadic and autosomal dominant
Good prognosis

Question 10

What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Chediak Higashi Syndrome?

Answer 10

Oculocutaneous albinism, neuropathies, recurrent pyogenic infections
Can’t make granules and chemotaxis no good
Autosomal recessive
No good prognosis

Question 11

What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Chronic Granulomatous Disease?

Answer 11

Recurrent purulent infections
Can’t make superoxide to kill microbes
Primarily x linked
Diagnosed with nitroblue tetrazolium test
Good prognosis

Question 12

What is the family of drugs causing neutropenia?

Answer 12

Chemo

Question 13

4 nutritional deficiencies causing neutropenia?

Answer 13

B12, folate, copper and starvation

Question 14

What is the leading cause of death by disease past infancy among children?

Answer 14

Cancer

Question 15

What is the most common type of cancer diagnosed in children 0-14 and what is the most common type of cancer diagnosed in 15-19 year olds?

Answer 15

Leukemias

Lymphomas

Question 16

3 infectious agents implicated in the development of HL?

Answer 16

CMV, EBV, and HHV6

Question 17

What is the pathognomonic feature of HL and how is it characterized?

Answer 17

RS cell

Large cell with multiple or mutilobulated nuclei

Question 18

3 B systemic symptoms important for staging of HL?

Answer 18

Unexplained fever of 39
Two loss greater than 10% of body weight over 6 months
Drenching night sweats

Question 19

Talk about diagnosing HL and the indications for a chest x ray?

Answer 19

Any patient with persistent, unexplained LAD that is not associated with a known underlying inflammatory or infectious process should undergo chest x ray to rule out mediastinal mass before doing LN biopsy

Question 20

What is the overall most common type of lymphoma in children and teens?

Answer 20

NHL at 60%

Question 21

A small number of children have NHL secondary to specific etiologies, what are two inherited or acquired immune deficiencies?

Answer 21

SCID

Wiscott Aldrich Syndrome

Question 22

What is the inheritance pattern of wiscott Aldrich syndrome and what are three clinical signs of it?

Answer 22

X linked
Recurrent sino-pulmonary and ear infections
Severe atopic dermatitis
Bleeding secondary to thrombocytopenia

Question 23

4 major subtypes of childhood and teen NHL?

Answer 23

Burkitt
Lymphoblastic lymphoma
Diffuse large B cell lymphoma
Anaplastic large cell lymphoma

Question 24

What are the two types of Burkitt?

Answer 24

Sporadic involving the abdominal

African or endemic involving head and neck, bone marrow and CNS

Question 25

What is the condition responsible for the greatest percentage of childhood malignancies?

Answer 25

Leukemia

Question 26

What is the genetic trisomy associated with both ALL and AML?

Answer 26

Trisomy 21