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Peds: Hematology and Oncology
Peds: Hematology and Oncology Exam 2
106
Anatomy
Graduate
03/06/2012

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Term
pancytopenia, whats the next step?
Definition
bone marrow aspiration
Term
most common childhood cancer:
Definition
ALL (usually B lineage)
Term
B ALL has __ WBCs, while T ALL has __ WBCs.
Definition
- low
- high
Term
high WBC and anterior mediastinal mass:
Definition
T cell ALL
Term
normal CBC and anterior mediastinal mass:
Definition
Lymphoblastic Lymphoma
Term
Most common varieties of childhood cancers:
Definition
Left Brain MeN Women.
1. Leukemias (ALL and AML)
2. Brain tumors
3. Malignant Lymphomas
4. Neuroblastoma
5. Wilm's tumor
Term
If neuroblastoma arises in the chest, it is in the __ mediastinum.
Definition
- posterior mediastinum
Term
Difference b/w Wilm's tumor and Neuroblastoma:
Definition
- Wilm's tumor arises in the kidneys
- Neuroblastoma arises in the neural crest cells in adrenal medulla or in sympathetic ganglion along the spine
Term
2 most common intrabdominal tumors in kids:
Definition
- Wilm's tumor
- Neuroblastoma
Term
Less common childhood cancers:
Definition
BROGSHH
- Bone
- Retinoblastoma
- Other: nasopharyngeal, thyroid
- Germ cell tumors
- Soft tissue (rhabdo-)
- Hepatoma
- Histiocytosis
Term
2 most common bone tumors in children:
Definition
Osteosarcoma
Ewing's sarcoma
Term
What is the most common cancer in men ages 15-35?
Definition
testicular cancer
Term
carcinomas are of __ origin.
Definition
epithelial
Term
Histiocytosis presentation:
Definition
- rash
- eusinophilic granuloma
- diabetes insipidus
Term
liklihood of cure of childhood cancer:
Definition
70%
Term
overall leukemia survival rate:
Definition
70%
Term
ALL survival rate:
Definition
80%
Term
AML survival rate:
Definition
45%
Term
T-cell leukemia survival rate:
Definition
75%
Term
Infant leukemia survival rate:
Definition
40%
Term
survial rates:
Definition
- Brain tumor 50%
- Sarcomas 50%
- Neuroblastoma 60%
- Lymphomas 70%
- Hodgkins 80%
- Wilm's tumor 85%
Term
Auer rods are pathognomic for ___.
Definition
AML
Term
Treat AML with __ __.
Definition
retinoic acid
Term
clinical presentation of leukemia:
Definition
- Bone marrow failure: anemia(low RBC), neutropenia, thrombocytopenia
- Tissue infiltration: blast cells in blood, organomegaly, mass, pain
Term
peak incidence of ALL:
Definition
age 4 y/0

(no peak incidence with AML)
Term
Prognostic factors for ALL B type:
Definition
ACE Won Some.
- Age
- CNS
- Early response
- WBC
- Sex
Term
childhood solid tumors from MC to LC:
Definition
1. Neuroblastoma 0.9/100,00
2. Wilm's tumor 0.75/100,00
3. Bone tumors 0.5/100,000
4. Rhabdomyosarcoma 0.4/100,000
Term
Do neuroblastomas usually cross the midline?
Definition
NO
Term
Signs that make you consider CNS cord compression due to a spinal tumor:
Definition
- Morning vomiting
- Head tilt
- Midline back pain
Term
Osteosarcoma is MC in the __ of __ bones.
Definition
- metaphysis of long bones
Term
Ewing's sarcoma is MC in __ of long bones or in the __ __.
Definition
- diaphysis
- axial skeleton
Term
Osteosarcoma is MC in the __ of long bones, whereas Ewing's sarcoma is more common in the __ of long bones or the __ __.
Definition
- metaphysis
- diaphysis
- axial skeleton
Term
Clincal features of BONE MARROW FAILURE:
Definition
1. Anemia- NORMOcytic or MACROcytic
2. Thrombocytopenia
3. Leukopenia

2 or 3 of the above are RED FLAGS and HIGHLY SUGGESTIVE of bone marrow failure
Term
Abdominal masses with worrisome characteristics:
Definition
- firm and fixed

Need to distinguish enlarged spleen (smooth with notch) from masses which tend to be irregular
Term
characteristics of persistent lymphadenopathy that are concerning for cancer:
Definition
- lower cervical/supraclavicular nodes- rubbery, fixed
Term
PE findings when to worry about cancer:
Definition
- Bone marrow failure: anemia (normo or macro), thrombocytopenia, Leukopenia, Neutropenia
- Firm fixed abdominal mass
- Firm fixed lower cervical/supraclavicular nodes
- Specific neuro deficits, increased intracranial pressure
- Exopthalmos/proptosis
- White pupillary reflex
- Unilateral knee/shoulder pain/swelling
- Back pain
- Migratory Multifocal Skeletal Pain- could be sign of bone marrow failure
- Limp
- Refusal to walk
- Firm fixed mass in any location
- Vaginal bleeding/mass
- Firm scrotal mass
- Hematuria
- Nodular and/or purple skin lesions
Term
Imaging Red Flags when to worry about cancer:
Definition
- Mediastinal masses/widening (NEVER NORMAL)
- Persistent focal "pneumonia" (NEVER NORMAL)
- Diffuse demineralization w/ or w/o growth arrest lines
- Extra-skeletal calcification
- Loss of cortical bone integrity
- Mixed lytic/sclerotic lesions
- Soft tissue mass with or w/o bone lesions
- Pathologic fracture
- Vertebral body compressions (seen with leukemia and neuroblastoma)
Term
Vertebral body compression fractures may be seen with __ or __.
Definition
- leukemia
- neuroblastoma
Term
MC malignant brain tumor in children:
Definition
Medulloblastoma
Term
CNS Red Flags that make you worry about cancer:
Definition
- Diffuse enlargement of the pons
- Hydrocephalus with mass
- Mid-line shift
Term
Pertussis is the only infection that will cause __, all other bacterial infections cause ___.
Definition
- lymphocytosis (pertussis)
- neutrophilia
Term
Lab finding red flags that make you worry about cancer:
Definition
- BI- or pancytopenia
- Unexplained leukocytosis
- Marked neutrophilia without source
- Lymphocytosis
- Increased LDH
- Increased Uric acid
- Increased Ferritin
- Increased Calcium
Term
When NOT to worry about cancer:
Definition
- Microytic anemia (low MCV)
- Isolated severe thrombocytopenia
- Increased alkaline phosphate
Term
Because __ __ increases in kids when they are growing quickly, this is not an indicator of malignancy.
Definition
alkaline phosphate
Term
Important questions to ask to the child with petechiae or purpura:
Definition
Sick or not:
- Fever
- Fatigue
- Musckuloskeletal pain/swelling
- Abdominal pain/swelling
- Recent infection
Term
2 things you definitely need to palpate for in child with petechiae or purpura?
Definition
- adenopathy
- hepatosplenomegaly
Term
Pt has petechiae/purpura, is not sick, has no constitutional symptoms,no MS findings, and has no HSM. What labs do you order?
Definition
- CBC
- Blood group
- Rh
Term
If patient with petechiae and purpura is sick or has history and physical suggesting systemic illness, then check:
Definition
- CBC
- Blood bank specimen
- UA
- Chemistry panel- metabolic, renal, liver, LDH, and uric acid
Term
Really sick pt with petechiae and purpura, what should be in ddx?
Definition
- sepsis
- meningococcemia

Get pt urgently evaluated and treated
Term
Other tests to be selectively done on sick pts with petechiae and purpura:
Definition
- PT, PTT, Coagulation screen
- ANA and other autoimmune studies
- ESR
- Coomb's test
Term
which pts with petechiae and purpura need bone marrow exm?
Definition
- those presenting UNLIKE ITP
- sick
- pancytopenia
- HSM
Term
BEFORE YOU TREAT A PT WITH WHAT YOU THINK IS ITP WITH STEROIDS, REMEMBER THERE IS A LOT MORE POTENTIAL TO DO HARM THAN GOOD. (may mask cancer)
Definition
Term
Factors that make neutropenia urgent:
Definition
- Very young pt (age less than 3-6 months old)
- Severe ANC < 200
- Fever > 38.3 (100.94 F)
- Skin lesions suggetive hemtologenous process or favoring eccthyma
Term
Other factors that raise concern with neutropenia:
Definition
VOPARMC
- Vital sign changes (do NOT have to have fever to raise concern)
- Oxygenation changes
- Perfusion changes
- Abdominal pain/tenderness
- Rectal/perianal pain (warning sign of invasive infection)
- Malignancy known, esp if chemo in the past 2 weeks
- Central line present
Term
Antibiotics for Fever and Neutropenia:
Definition
- Ceftazidine or Ceftipimine +/- Vancomycin +/- Aminoglycoside
Term
which antibiotic do you NOT use for fever and neutropenia:
Definition
Ceftriaxone
Term
Chronic Benign Neutropenia:
Definition
- ANC < 200-500
- Presents ages 9-18 months
- Lasts months to years
- Upper Respiratory Infections, usually superficial
- Good prognosis, good treatment options
Term
Retinal detachment issues may indicate:
Definition
retinoblastoma
Term
The philadelphia chromosome is associated with __ __ __.
Definition
chronic myelogenous leukemia (CML)
Term
MC malignant brain tumor in children:
Definition
meduloblastoma
Term
only bacterial infection to cause lymphocytosis is ___
Definition
pertussis
Term
causes of an iron deficiency in infants less than 6 months:
Definition
- Prematurity
- Small birth weight
- Neonatal anemia
- Perinatal blood loss
- Hemorrhage
Term
Iron deficient children over the age of 24 months (2 years), need to be evaluated for __ __.
Definition
blood loss
Term
signs of sever iron deficiency anemia:
Definition
- mild iron deficiency is usually asymptomatic
- severe> signs- pallor, fatigue, irritability, delayed motor development
Term
Lab values expected with iron deficiency anemia:
Definition
Microcytic, Hypochromic, low Hemoglobin, low Hematocrit
- MCV, Abosolute retic count, ferritin, and serum iron are decreased
- TIBC and RDW are increased
Term
Thalassemia lab findings:
Definition
- Normal to increased ferritin
- Normal to increased serum iron
- Normal TIBC
- Increased erythrocytes
- MCV/RBC < 13
- Less likely to have increased RDW
Term
Recommended oral dose of elemental iron:
Definition
6 mg/kg/day in 3 divided daily doses

(mild iron deficiency can be treated with 2 mg/kg/day once daily before breakfast)
Term
Ethnic groups more prone to sickle cell anemia:
Definition
CAMMI
- Caribbean ancestry
- African
- Mediterranean
- Middle Eastern
- Indian
Term
Symptoms of sickle cell, a hemolytic anemia, are caused by:
Definition
tissue ischemia and organ dysfunction caused by vaso-occlusion
Term
When do sickle cell symptoms appear and why?
Definition
- symptoms are unusual before ages 3-4 months because there are high levels of fetal hemoglobin that inhibit sickling
- moderately sever hemolytic anemia may present around 1 year of age
Term
MC initial symptom of sickle cell anemia:
Definition
Dactylitis aka hand and foot syndrome
occurs in up to 50% of children before age of 3
Term
Patient with sickle cell anemia are at greater risk for overwhelming infections with ___ ___, particularly __.
Definition
- encapsulated bacteria
- pneumococci
Term
signs and symptoms of sickle cell:
Definition
- Pallor
- Fatigue
- Jaundice
- Gallstones
- Splenomegaly (may have functional asplenia as early as 3 months> increased risk for infections, particularly pneumococci)
- Splenic sequestration
- Dactylitis
- Strokes
- Acute Chest Syndrome
- Tissue damage from vaso-occlusion
Term
Acute splenic sequestration with sickle cell anemia is characterized by:
Definition
- Sudden spleen enlargement
- Pooling of red cells
- Acute exacerbation of anemia
- In some cases, shock and death
Term
Acute Chest Syndrome associated with sickle cell anemia is characterized by __, __ __ __, and __ __ __ with __. This condition is caused by pulmonary __, __, or __ __ from __ __ __.
Definition
- Fever
- Pleuritic chest pain
- Acute pulmonary infiltrates with hypoxemia
- infection
- infarction
- fat embolism from ischemic bone marrow
Term
Sickle cell anemia lab findings:
Definition
- Hemoglobin 7-10
- Retic count increased
- MCV normal to increased
- Sickle and target cell on smear
Term
Results on neonatal screening indicative of sickle cell disease require promt confirmation with __ __.
Definition
hemoglobin electrophoresis
Term
MC indication for transfusion in SCD:
Definition
stroke
Term
when is RBC transfusion indicated for SCD:
Definition
- for acute exacerbations of anemia (aplastic crisis or splenic sequestration)
- NOT indicated for chronic steady state anemia
Term
Most common bleeding disorder of childhood:
Definition
Acute ITP
Term
ITP ages:
Definition
2-5 years old
Term
Common causes of ITP:
Definition
Viruses: RIPMEV
- Rubella
- Varicella
- Measles
- Parvovirus
- Influenza
- EBV

most pts recover spontaneously within a few months
Term
Chronic ITP lasts greater than:
Definition
6 months
Term
Presentation of ITP:
Definition
- acute onset
- multiple petechiae and ecchymosis
- epistaxis
- no other physical findings are usually present
Term
Expected CBC findings with ITP:
Definition
- Markedly reduced platelet count (<50,000 and often < 10,000)
- Platelets are of larger size suggesting accelerated production of new platelets
- Normal WBC and diff and hemoglobin
Term
Typical ITP treatment:
Definition
- avoid ASA and other drugs that compromise platelet function
- bleeding precautions: restrict physical contact sports and use helmet)
Term
ITP with life-threatening bleed tmt:
Definition
- Platelet transfusion
- Emergent splenectomy
- Corticosteroids
- IVIG
Term
platelet transfusion should be avoided for ITP unless there is lifethreatening bleed. then transfuse platelets, emergent splenectomy, cs and IVIG
Definition
Term
Hemophilia A is a deficiency of factor __. It is more common in __ and is __ __ __. 1/3 of cases are due to a __ __.
Definition
- factor 8
- males
- x-linked recessive
- new mutation
Term
distinguish b/w mild, moderate, and severe hemophilia A:
Definition
- mild (5-40% plasma factor VIII): bleeding only at times of trauma and surgery
- severe (<1% plasma factor VIII activity): frequent spontaneous bleeding involving skin, mucous membranes, joints, muscles, and viscera
Term
male newborn with fhx of hemophilia A, what should be done?
Definition
- cord blood sampling for factor VIII
Term
MCC of disease related death with Hemophilia A:
Definition
Intracranial hemorrhage (occur spontaneously with moderate to sesvere deficiency)
Term
MC complications of Hemophilia A:
Definition
- Hemarthroses, recurrent can cause joint destruction
- Large intramuscular hematomas> compartment syndrome, muscle and nerve death
- Intracranial hemorrhage
Term
Tmt of non-life/non-limb threatening hemorrhage associated with Hemophilia A:
Definition
- 20-30 U/kg Factor VIII to acheive a rise in plasma factor VIII activity to 40-60%
Term
mild hemophilia A may respond to :
Definition
Desmopressin> endothelial release of factor VIII and vWF

but most require factor VIII transfusion
Term
What is the most common INHERITED bleeding disorder among Caucasians with a prevalence of 1%?
Definition
Von Willebrand Disease
Term
70-80% of pts with vWD have CLASSIC TYPE 1 VON WILLEBRAND DISEASE, which is caused by a __ __ __ of von Willebrand factor.
Definition
- partial quantitative deficiency
Term
Type 2 vWD is caused by a __ __ of vWF.
Definition
qualitative deficiency (dysfunctional)
Term
Type 3 vWD is caused by a nearly _ __ of vWF.
Definition
complete deficiency
Term
presentation of vWD:
Definition
- increased bruising
- increased epistaxis
- bleeding prolonged with trauma/surgery
- menorrhagia is often presenting sign in females
Term
with hemophilia A, __ is prolonged and __ is normal.
Definition
- aPTT is prolonged
- PT is normal
Term
vWFD findings:
Definition
- aPTT sometimes prolonged
- PT normal
- PFA-100 or bleeding time prolonged
Term
Platelet number may be decreased with vWFD type __.
Definition
2
Term
Factor VIII and vWF antigen are decreaed in types __ and __ vWFD but may be normal in type __ vWFD.
Definition
- 1 and 3
- 2
Term
vWF is important for platelet adherence to the endotheliu
Definition
Term
Treatment for type 1 and 2 vWFD:
Definition
- Desmopressin acetate 0.3mcg/kg diluted in 20-30 mL saline, given over 20-30 minutes
- this will increase plasma vWF by 3-5 fold and halt bleeding
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