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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
Q

Lab findings for hemolytic anemias

A 
Elevated retic count 
Low serum haptoglobin (bc it is used up transporting the released hemoglobin)
Elevated LDH 
Elevated indirect bili 
Hemoglobinuria not hematuria
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26
Q

Drug that most frequently causes neutropenia

A

Macrolides

27
Q

Presentation of cyclic neutropenia

A

Low WBC that lasts about 1 wk and reappears monthly

Oral lesions and enlarged lymph nodes

Child younger than 10 yrs

28
Q

Pts with cyclic neutropenia that is untreated die due to which organisms

A

Clostridia perfringens and gram negative organisms

29
Q

Tx for cyclic neutropenia

A

Daily rhG-CSF (recombinant human granulocyte colony stimulating factor)

30
Q

Presentation of kostmann agranulocytosis

A

Like cyclic neutropenia except ANC remains low

Autosomal recessive vs dominant

31
Q

Presentation of shwachman diamond syndrome

A

Pancreatic exocrine insufficiency (steatorrhea)

Pancytopenia - recurrent infections (especially upper respiratory and skin)

Skeletal abnormalities

32
Q

Which medications can lower platelet count

A

Sulfa, seizure meds and vancomycin

33
Q

What leads to worse prognosis in a pt with ITP

A

Kid older than 10 years

34
Q

Presentation of TAR syndrome

A

Thrombocytopenia and absent radius!

Presents within the first month of life

35
Q

Prolongued PT is associated with what

A

Vit K deficiency (factors 2,7,9,10)

36
Q

Prolongued PTT is associated with what

A

Hemophilia

May be Von Willebrand if female

37
Q

How is hemophilia inherited

A

X linked recessive

38
Q

Lab findings in DIC

A

Low platelets, low fibrinogen, elevated d dimer, prolongued thrombin time

39
Q

Presentation of osteoid osteoma

A

Tibia or femur pain, worse at night and relieved by ibuprofen

Central radiolucent surrounded by thick sclerotic bone

40
Q

Presentation of osteogenic sarcoma

A

Teenager usually going through a growth spurt who presents with pain

Common sites are the distal long bones (femur and humerus)

41
Q

How does Hodgkin’s lymphoma present

A

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
Q

What would a lymph node biopsy of Hodgkin lymphoma show

A

Reed sternberg cells

43
Q

How does non Hodgkin lymphoma present

A

Small child

Non tender mass in the abdomen

Airway compression

Rapid presentation

44
Q

What is the survival rate when neuroblastoma presents before 1 year of age

A

95 percent

45
Q

If a pt with neuroblastoma has hypertension what is the most likely reason

A

Renal artery compression

46
Q

How do you diagnose neuroblastoma

A

Biopsy of tumor

Elevated urine VMA and HMA (not catecholamines)

47
Q

What is the most important prognostic factor for neuroblastoma

A

Age

If <1 yr prognosis is excellent

48
Q

How does sporadic retinoblastoma present

A

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
Q

How does genetic retinoblastoma present

A

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
Q

What other malignancies in a child with retinoblastoma do you need to monitor for

A

Sarcomas and malignant melanomas

51
Q

What is the most common solid tumor of infancy

A

Neuroblastoma

52
Q

What is the most common pediatric abdominal malignancy

A

Wilms tumor

53
Q

If an abdominal XRAY shows calcification on an abdominal mass what should you think of

A

Neuroblastoma

Wilms tumor does not calcify

54
Q

What should you think of in a pt with hemihypertrophy

A

Wilms tumor

Beck with wiedemann syndrome

55
Q

How does superior vena cava syndrome present

A

Red face, facial swelling, upper extremity edema, distended neck veins

Due to extrinsic compression of the SVC by an anterior mediastinal tumor

56
Q

Side effect of cyclophosphamide

A

Hemorrhagic cystitis

57
Q

Side effect of cisplatin

A

Hearing loss and peripheral neuropathy

58
Q

Side effect of bleomycin

A

Pulmonary fibrosis

59
Q

Side effect of doxorubicin and daunomycin

A

Cardiac toxicity

60
Q

Side effect of vincristine/vinblastin

A

Neurotoxicity and SIADH

61
Q

Side effect of methotrexate

A

Oral and GI ulcers

Bone loss

62
Q

Would you intubate a pt with anterior mediastinal mass

A

No because the airway compression is below the vocal cords

63
Q

What can cause symptoms associated with anterior mediastinal masses

A

Thymoma
Teratoma
Thyroid carcinoma
Terrible lymphoma

laughing your way peds (26 decks)

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