Non Neoplastic WBC Disorders and Leukemias and Lymphomas Flashcards
Order the WBCs in decreasing order of amount?
NLMEB
How do you calculate the absolute neutrophil count out of a CBC?
(%Neutrophils + %Bands) x (WBC)/100
How do we define neutropenia by value, and what is mild, moderate and severe neutropenia?
Less than 1500
Severe is less than 500
Moderate is between 500 and 1000
Mild is between 1000 and 1500
At what value do we say the risk of serious infection is high?
Less than 500 of ANC
What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Kostmann Syndrome?
Life threatening pyogenic infections
Maturational arrest of neutrophil precursors
Autosomal recessive
Bone marrow shows myeloid arrest, 60-80% have neutrophil ELA2 mutation
AML
What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Cyclic Neutropenia?
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
What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Shwachman Diamond Syndrome?
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
What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Leukocyte Adhesion Defieciency?
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
What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Hyper IgE syndrome/job syndrome?
Severe eczema, bacterial infections of lungs and skins, fungal infection
Neutrophil chemotaxis sucks
Sporadic and autosomal dominant
Good prognosis
What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Chediak Higashi Syndrome?
Oculocutaneous albinism, neuropathies, recurrent pyogenic infections
Can’t make granules and chemotaxis no good
Autosomal recessive
No good prognosis
What is the clinical manifestation, defect, inheritance pattern, method of diagnosis and risk of malignancy associated with Chronic Granulomatous Disease?
Recurrent purulent infections Can’t make superoxide to kill microbes Primarily x linked Diagnosed with nitroblue tetrazolium test Good prognosis
What is the family of drugs causing neutropenia?
Chemo
4 nutritional deficiencies causing neutropenia?
B12, folate, copper and starvation
What is the leading cause of death by disease past infancy among children?
Cancer
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?
Leukemias
Lymphomas
3 infectious agents implicated in the development of HL?
CMV, EBV, and HHV6
What is the pathognomonic feature of HL and how is it characterized?
RS cell
Large cell with multiple or mutilobulated nuclei
3 B systemic symptoms important for staging of HL?
Unexplained fever of 39
Two loss greater than 10% of body weight over 6 months
Drenching night sweats
Talk about diagnosing HL and the indications for a chest x ray?
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
What is the overall most common type of lymphoma in children and teens?
NHL at 60%
A small number of children have NHL secondary to specific etiologies, what are two inherited or acquired immune deficiencies?
SCID
Wiscott Aldrich Syndrome
What is the inheritance pattern of wiscott Aldrich syndrome and what are three clinical signs of it?
X linked
Recurrent sino-pulmonary and ear infections
Severe atopic dermatitis
Bleeding secondary to thrombocytopenia
4 major subtypes of childhood and teen NHL?
Burkitt
Lymphoblastic lymphoma
Diffuse large B cell lymphoma
Anaplastic large cell lymphoma
What are the two types of Burkitt?
Sporadic involving the abdominal
African or endemic involving head and neck, bone marrow and CNS
What is the condition responsible for the greatest percentage of childhood malignancies?
Leukemia
What is the genetic trisomy associated with both ALL and AML?
Trisomy 21
