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laughing your way peds > Heme-onc > Flashcards

Heme-onc Flashcards

1
Q

Microcytic anemia with high rdw

A

Iron deficiency and lead toxicity

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2
Q

When do you see ringed sideroblast

A

Lead poisoning

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3
Q

Drinking goat milk lead to what deficiency

A

Folate

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4
Q

A hemolytic anemia without an elevated retic count would make you think of what

A

Parvovirus

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5
Q

Coombs test positive is what type of anemia

A

Autoimmune hemolytic

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6
Q

How does G6PD deficiency present

A

Hemolytic anemia in AA or Mediterranean boy (x linked)

Sudden onset of parlor and anemia, dark Uribe and jaundice

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7
Q

Agents that cause oxidative stress and exacerbate G6PD

A

Sulfa containing antibiotic, nitrofurantoin, moth balls, antimalarial meds (chloroquine and primaquine) fava beans, rasburicase

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8
Q

When should testing for G6PD be done

A

Several weeks after the episode

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9
Q

Heinz bodies are found when

A

G6PD deficiency

*bite and blister cells

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10
Q

Presentation of hereditary spherocytosis

A

Mild or moderate anemia, splenomegaly, intermittent jaundice, gallstones

Autosomal dominant

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11
Q

How do you diagnose hereditary sperocytosis

A

Osmotic fragility testing

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12
Q

Fragile red cells with increased MCHC are found in which condition

A

Hereditary spherocytosis

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13
Q

What is the etiology of hereditary spherocytosis

A

Spectrin deficiency or mutation

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14
Q

What is the etiology of sickle cell

A

Valine is substituted for a glutamic acid at amino acid 6 of the beta globin chain

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15
Q

Until what age is antibiotic prophylaxis for sickle cell disease indicated

A

Through age 5

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16
Q

What finding will you find in blood smear for a pt with sickle cell

A

Howell jolly bodies

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17
Q

What should you think of if you have a kid older than 5 with sickle cell in which you can palpate a spleen

A

By age 5 kids with sickle cell have functional asplenia and you can’t feel a spleen. Thus you want to think of a variation.

Hemoglobin SC or sickle trait and thalassemia together

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18
Q

Children with sickle cell and thalassemia together are resistant to what

A

Malaria

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19
Q

How is acute chest syndrome define and what would be the next study to obtain

A

Chest pain, infiltrate in chest X-ray, hypoxia

You want an ABG to confirm hypoxia

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20
Q

How does fanconi’s anemia present

A

After age 3

Macrocytic anemia with elevated fetal hemoglobin

Abnormal skin pigmentation

Growth retardation (short stature)

Renal abnormalities

Absent thumb

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21
Q

How is fanconi anemia inherited

A

Recessive

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22
Q

What are patients with fanconi anemia at risk for

A

Transformation to AML or myelodysplastic syndrome

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23
Q

How does diamond Blackfan anemia present (DBA)

A

DBA =

Dysmosphic (craniofacial problems, thumb abnormalities, urogenital defects)

Babies (infants 2-3 months)

Anemia (macrocytic, severe and chronic)

*require transfusions and steroids are helpful

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24
Q

How does transient erythroblastopenia of childhood present (TEC)

A

TEC =

Transient
Erythroid production suppression (anemia)
Children (18-26 months)

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25
Lab findings for hemolytic anemias
``` Elevated retic count Low serum haptoglobin (bc it is used up transporting the released hemoglobin) Elevated LDH Elevated indirect bili Hemoglobinuria not hematuria ```
26
Drug that most frequently causes neutropenia
Macrolides
27
Presentation of cyclic neutropenia
Low WBC that lasts about 1 wk and reappears monthly Oral lesions and enlarged lymph nodes Child younger than 10 yrs
28
Pts with cyclic neutropenia that is untreated die due to which organisms
Clostridia perfringens and gram negative organisms
29
Tx for cyclic neutropenia
Daily rhG-CSF (recombinant human granulocyte colony stimulating factor)
30
Presentation of kostmann agranulocytosis
Like cyclic neutropenia except ANC remains low Autosomal recessive vs dominant
31
Presentation of shwachman diamond syndrome
Pancreatic exocrine insufficiency (steatorrhea) Pancytopenia - recurrent infections (especially upper respiratory and skin) Skeletal abnormalities
32
Which medications can lower platelet count
Sulfa, seizure meds and vancomycin
33
What leads to worse prognosis in a pt with ITP
Kid older than 10 years
34
Presentation of TAR syndrome
Thrombocytopenia and absent radius! Presents within the first month of life
35
Prolongued PT is associated with what
Vit K deficiency (factors 2,7,9,10)
36
Prolongued PTT is associated with what
Hemophilia May be Von Willebrand if female
37
How is hemophilia inherited
X linked recessive
38
Lab findings in DIC
Low platelets, low fibrinogen, elevated d dimer, prolongued thrombin time
39
Presentation of osteoid osteoma
Tibia or femur pain, worse at night and relieved by ibuprofen Central radiolucent surrounded by thick sclerotic bone
40
Presentation of osteogenic sarcoma
Teenager usually going through a growth spurt who presents with pain Common sites are the distal long bones (femur and humerus)
41
How does Hodgkin's lymphoma present
Typically in teens Non tender enlarged cervical or supraclavicular lymph nodes Weight loss, fever, night sweats High WBC with a relatively low lymphocyte count
42
What would a lymph node biopsy of Hodgkin lymphoma show
Reed sternberg cells
43
How does non Hodgkin lymphoma present
Small child Non tender mass in the abdomen Airway compression Rapid presentation
44
What is the survival rate when neuroblastoma presents before 1 year of age
95 percent
45
If a pt with neuroblastoma has hypertension what is the most likely reason
Renal artery compression
46
How do you diagnose neuroblastoma
Biopsy of tumor Elevated urine VMA and HMA (not catecholamines)
47
What is the most important prognostic factor for neuroblastoma
Age If <1 yr prognosis is excellent
48
How does sporadic retinoblastoma present
Usually unilateral Only 5% will have a family Hx. If one parent had only 1 eye affected then there is only a 1/20 (5%) chance of a child having it
49
How does genetic retinoblastoma present
Autosomal dominant with incomplete penetrance. Gene is on the long arm of chromosome 13. If a parent had both eyes affected there is 1/2(50%) chance of any child having it
50
What other malignancies in a child with retinoblastoma do you need to monitor for
Sarcomas and malignant melanomas
51
What is the most common solid tumor of infancy
Neuroblastoma
52
What is the most common pediatric abdominal malignancy
Wilms tumor
53
If an abdominal XRAY shows calcification on an abdominal mass what should you think of
Neuroblastoma Wilms tumor does not calcify
54
What should you think of in a pt with hemihypertrophy
Wilms tumor Beck with wiedemann syndrome
55
How does superior vena cava syndrome present
Red face, facial swelling, upper extremity edema, distended neck veins Due to extrinsic compression of the SVC by an anterior mediastinal tumor
56
Side effect of cyclophosphamide
Hemorrhagic cystitis
57
Side effect of cisplatin
Hearing loss and peripheral neuropathy
58
Side effect of bleomycin
Pulmonary fibrosis
59
Side effect of doxorubicin and daunomycin
Cardiac toxicity
60
Side effect of vincristine/vinblastin
Neurotoxicity and SIADH
61
Side effect of methotrexate
Oral and GI ulcers | Bone loss
62
Would you intubate a pt with anterior mediastinal mass
No because the airway compression is below the vocal cords
63
What can cause symptoms associated with anterior mediastinal masses
Thymoma Teratoma Thyroid carcinoma Terrible lymphoma

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