Term
| pancytopenia, whats the next step? |
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Definition
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Term
| most common childhood cancer: |
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Definition
|
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Term
| B ALL has __ WBCs, while T ALL has __ WBCs. |
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Definition
|
|
Term
| high WBC and anterior mediastinal mass: |
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Definition
|
|
Term
| normal CBC and anterior mediastinal mass: |
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Definition
|
|
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 |
|
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Term
| If neuroblastoma arises in the chest, it is in the __ mediastinum. |
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Definition
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|
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 |
|
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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 |
|
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Term
| What is the most common cancer in men ages 15-35? |
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Definition
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|
Term
| carcinomas are of __ origin. |
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Definition
|
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Term
| Histiocytosis presentation: |
|
Definition
- rash
- eusinophilic granuloma
- diabetes insipidus |
|
|
Term
| liklihood of cure of childhood cancer: |
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Definition
|
|
Term
| overall leukemia survival rate: |
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Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| T-cell leukemia survival rate: |
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Definition
|
|
Term
| Infant leukemia survival rate: |
|
Definition
|
|
Term
|
Definition
- Brain tumor 50%
- Sarcomas 50%
- Neuroblastoma 60%
- Lymphomas 70%
- Hodgkins 80%
- Wilm's tumor 85% |
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Term
| Auer rods are pathognomic for ___. |
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Definition
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|
Term
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Definition
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|
Term
| clinical presentation of leukemia: |
|
Definition
- Bone marrow failure: anemia(low RBC), neutropenia, thrombocytopenia
- Tissue infiltration: blast cells in blood, organomegaly, mass, pain |
|
|
Term
|
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? |
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Definition
|
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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. |
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Definition
| - metaphysis of long bones |
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Term
| Ewing's sarcoma is MC in __ of long bones or in the __ __. |
|
Definition
- diaphysis
- axial skeleton |
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Term
| Osteosarcoma is MC in the __ of long bones, whereas Ewing's sarcoma is more common in the __ of long bones or the __ __. |
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Definition
- metaphysis
- diaphysis
- axial skeleton |
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Term
| Clincal features of BONE MARROW FAILURE: |
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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 |
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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: |
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Definition
| - lower cervical/supraclavicular nodes- rubbery, fixed |
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|
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: |
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Definition
|
|
Term
| CNS Red Flags that make you worry about cancer: |
|
Definition
- Diffuse enlargement of the pons
- Hydrocephalus with mass
- Mid-line shift |
|
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Term
| Pertussis is the only infection that will cause __, all other bacterial infections cause ___. |
|
Definition
- lymphocytosis (pertussis)
- neutrophilia |
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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 |
|
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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. |
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Definition
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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 |
|
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Term
| Pt has petechiae/purpura, is not sick, has no constitutional symptoms,no MS findings, and has no HSM. What labs do you order? |
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Definition
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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 |
|
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Term
| Really sick pt with petechiae and purpura, what should be in ddx? |
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Definition
- sepsis
- meningococcemia
Get pt urgently evaluated and treated |
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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 |
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Term
| which pts with petechiae and purpura need bone marrow exm? |
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Definition
- those presenting UNLIKE ITP
- sick
- pancytopenia
- HSM |
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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) |
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Definition
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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 |
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Term
| Other factors that raise concern with neutropenia: |
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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 |
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Term
| Antibiotics for Fever and Neutropenia: |
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Definition
| - Ceftazidine or Ceftipimine +/- Vancomycin +/- Aminoglycoside |
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|
Term
| which antibiotic do you NOT use for fever and neutropenia: |
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Definition
|
|
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 |
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Term
| Retinal detachment issues may indicate: |
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Definition
|
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Term
| The philadelphia chromosome is associated with __ __ __. |
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Definition
| chronic myelogenous leukemia (CML) |
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Term
| MC malignant brain tumor in children: |
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Definition
|
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Term
| only bacterial infection to cause lymphocytosis is ___ |
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Definition
|
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Term
| causes of an iron deficiency in infants less than 6 months: |
|
Definition
- Prematurity
- Small birth weight
- Neonatal anemia
- Perinatal blood loss
- Hemorrhage |
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|
Term
| Iron deficient children over the age of 24 months (2 years), need to be evaluated for __ __. |
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Definition
|
|
Term
| signs of sever iron deficiency anemia: |
|
Definition
- mild iron deficiency is usually asymptomatic
- severe> signs- pallor, fatigue, irritability, delayed motor development |
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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 |
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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 |
|
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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) |
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|
Term
| Ethnic groups more prone to sickle cell anemia: |
|
Definition
CAMMI
- Caribbean ancestry
- African
- Mediterranean
- Middle Eastern
- Indian |
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Term
| Symptoms of sickle cell, a hemolytic anemia, are caused by: |
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Definition
| tissue ischemia and organ dysfunction caused by vaso-occlusion |
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Term
| When do sickle cell symptoms appear and why? |
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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 |
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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 |
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Term
| Patient with sickle cell anemia are at greater risk for overwhelming infections with ___ ___, particularly __. |
|
Definition
- encapsulated bacteria
- pneumococci |
|
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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 |
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|
Term
| Sickle cell anemia lab findings: |
|
Definition
- Hemoglobin 7-10
- Retic count increased
- MCV normal to increased
- Sickle and target cell on smear |
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|
Term
| Results on neonatal screening indicative of sickle cell disease require promt confirmation with __ __. |
|
Definition
| hemoglobin electrophoresis |
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Term
| MC indication for transfusion in SCD: |
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Definition
|
|
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
|
|
Term
|
Definition
|
|
Term
|
Definition
Viruses: RIPMEV
- Rubella
- Varicella
- Measles
- Parvovirus
- Influenza
- EBV
most pts recover spontaneously within a few months |
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Term
| Chronic ITP lasts greater than: |
|
Definition
|
|
Term
|
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
|
Definition
- avoid ASA and other drugs that compromise platelet function
- bleeding precautions: restrict physical contact sports and use helmet) |
|
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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 |
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Definition
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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
|
|
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
|
|
Term
|
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
|
Definition
- aPTT sometimes prolonged
- PT normal
- PFA-100 or bleeding time prolonged |
|
|
Term
| Platelet number may be decreased with vWFD type __. |
|
Definition
|
|
Term
| Factor VIII and vWF antigen are decreaed in types __ and __ vWFD but may be normal in type __ vWFD. |
|
Definition
|
|
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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|
