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Peds Shelf > Heme Onc_UW > Flashcards

Heme Onc_UW Flashcards

1
Q

What confirms dx of leukemia?

A

bone marrow biopsy with greater than 25%

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2
Q

Most common pediatric renal malignancy?

A

Nephroblastoma

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3
Q

Wilms tumor is aka

A

Nephroblastoma

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4
Q

Burr cells are seen in?

A

Spiculated RBCs of similar size with regularly spaced projections. Seen in uremia or artifact of preparation

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5
Q

Pappenheimer bodies.

A

Seen in sideroblastic anemia. Results from phagosomes that engulf excessive amounts of iron.

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6
Q

Heinz bodies seen in

A

G6PD deficiency

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7
Q

Heinz bodies are

A

when Hb oxidizes in GPD deficiency, they precipitate into these heinz bodies.

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8
Q

Universal screening for iron deficiency anemia

A

complete CBC 9-12 months

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9
Q

Tx for iron deficiency anemia

A

empiric oral iron therapy. Recheck Hg in 4 weeks, if it has risen by 1g/dL, continue for 2-3 months.

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10
Q

Tx for ITP

A

If only skin manifestations: observe, regardless of platelet count. If bleeding, then regardless of platelet count give IVIG or glucocorticoids

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11
Q

S pneumo, H influenze and N. meningitidis have what in common?

A

Encapsulated bacteria

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12
Q

Common vaccinations of childhood that use live attentuated viruses?

A

Measles, mumps, rubella and chicken pox

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13
Q

What can prevent pneumococcal sepsis in sickle cell pateints

A

Pneumococcal vaccine and pencillin propylaxis (kids should get it till age 5)

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14
Q

Common vaccinations of childhood with bacterial toxoids

A

Tetanus and diptheria

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15
Q

Epistaxis, visible nasal mass, nasal obstruction

A

Juvenile angiofibroma

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16
Q

Side effects of hydroxyurea treatment

A

Suppresses the bone marrow. Can cause thrombocytopenia, leukopenia and anemia. but these effects are generally temporary and reversible but can predispose patient to infection.

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17
Q

MOA of hydroxyurea

A

Increases proportion of fetal hemoglobin so that the proportion of HgS is reduced and chances of sickling reduce

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18
Q

What bones are frequently affected in osteonecrosis due to sickle cell anemia?

A

Humeral and femural heads

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19
Q

What is anistocytosis

A

Blood cells of unequal size

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20
Q

Lab findings for iron deficiency anemia

A

Decreased MCV, decreased transferrin, anistocytosis => increased RDW, decreased MCH, decreased trasnferrin saturation, increased TIBC. Smear shows small hypochromic rbcs.

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21
Q

MCHC is elevated or depressed in hereditary spherocytosis? Why?

A

It’s elevated. Due to cellular dehydration and membrane loss, the MCHC is high.

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22
Q

What are the diagnostic tests for hereditary spherocytosis?

A

Osmotic fragility test with acidified glycerol lysis test and abnormal eosin-5-maleimide binding test (flow cytometry)

23
Q

What is the typical manifestation of hereditary spherocytosis

A

Coombs negative hemolytic anemia, jaundice and splenomegaly

24
Q

Genetic pattern of hereditary spherocytosis

A

75% A/D, 25% spontaneous

25
Q

What are lab findings/diagnostic tests for hereditary spherocytosis

A

Imp finding is elevated MCHC. Due to cell dehydration and lower cell volume, this is elevated. Spherocytes on peripheral blood smear, coombs negative. Diagnostically do the acidfied glycerol lysis test and the eosin 5 maleimide binding test.

26
Q

How is vitamin K related to factors 2,7,9,10 and factors C and S.

A

Vitamin K is a cofactor for the enzyme gamma glutamyl carboxylase that adds carboxyl groups to the glutamate residues of several coagulation factors such as 2, 7, 9, 10 and C and S. These extra carboxyl groups add the factors affinities for platelets

27
Q

Most common primary bone tumor in children

A

Osteosarcoma

28
Q

Osteosarcomas are found most commonly in

A

Metaphyses of long bones: proximal humerus, distal femur, proximal tibia.

29
Q

PE findings and X -ray findings for osteosarcoma

A

Large tender mass. X-ray finding: sun-burst periosteal pattern and “codman” triangle.

30
Q

Lymphoblast histology/staining

A

Lack peroxidase positive material, often contain +PAS staining material, 95% immunostain for TdT. (TdT is only present in pre B and T lymphocytes)

31
Q

Example of clotting disorders

A

Hemophilia A and B

32
Q

Activated PTT time in hemophilias

A

Increased activated PTT

33
Q

Examples of platelet defects

A

Von Willebrand and Bernard Soulier Syndrome

34
Q

Ewing’s sarcoma is often confused with

A

Osteomyelitis because of the intermittent fevers, leukocytosis, malaise, joint swelling

35
Q

Characteristic x-ray findings for Ewing’s sarcoma

A

Lytic, central bone lesion with periosteal reaction leading to onion skin appearance, followed by mottled appearance/moth eaten and extension into soft tissue

36
Q

Cobalamin deficiency presents as

A

Macrocytic anemia

37
Q

Most common inherited bleeding disorder

A

Impaired Von Willebrand factor

38
Q

Primary dose limiting side effect of hydroxyurea

A

Myelosuppression

39
Q

Brainstem is composed of

A

Midbrain, pons, medulla

40
Q

Pineal gland secretes

A

Melatonin

41
Q

What is parinaud syndrome

A

Downward gaze preference (sun setting gaze) with limited upward gaze, ptosis, upper eyelid retraction, pupillary abnormalities

42
Q

What are the clinical sx of pineal gland mass

A

Parinaud syndrome, obstructive hydrocephalus, precocious puberty (if the pineal gland mass is a germ cell tumor that secretes b-HCG)

43
Q

What are the clinical sx of obstructive hydrocephalus

A

papilledema, vomiting, headache, ataxias

44
Q

What kind of masses can arise from pineal gland?

A

variety but most common is the germ cell tumor

45
Q

Wht is the triad of hemolytic uremic sydnrome

A

renal failure, thrombocytopenia and microangiopathic hemolytic anemia

46
Q

clinical ppt of HUS

A

abdominal pain and diarrhea followed by bloody diarrhea. Within 5-7 days => anemia, thrombocytopenia and renal insufficiency.

47
Q

PE of HUS

A

Pallor, jaundice (hemolysis), edema, petechiae, HTN

48
Q

What is the characteristic peripheral smear of HUS

A

Schistocytes and large platelet

49
Q

Lead poisoning causes what kind of anemia

A

Microcytic anemia. Basophilic stippling on peripheral blood smear.

50
Q

Triad of Wiskott Aldrich Syndrome

A

Eczema, thrombocytopenia (from decreased platelet production), hypogammaglobulinemia leading to recurrent bacterial infections.

51
Q

How does polycythemia lead to cyanosis?

A

High red blood cell mass and hyperviscosity leads to cyanosis

52
Q

What is the definition of neonatal polycythemia

A

Hematocrit greater than 65%

53
Q

Wht are the risk factors for neonatal polycythemia

A

Increased erythropoiesis from Maternal diabetes, maternal hypertension, IUGR, smoking. Or erythrocyte transfusion from delayed cord clamping or twin twin transfusion

54
Q

Clinical presentation of neonatal polycythemia

A

Neurologic manifestations (irritability, jitteriness), respiratory distress and hypoglycemia.

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