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Pediatrics > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

What is the incidence of febrile transfusion reactions?

A

1% for pRBC and 10% for platelet transfusions

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2
Q

Diagnostic criteria of CVID:

A

1.) decrease of more than 2 standard deviations of one immunoglobulin below the mean, 2.) poor antibody response to protein and polysaccharide vaccines 3.) exclusion of other causes of hypogammaglobulin

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3
Q

Kostmass syndrome or Familial severe neutropenia

A

ANC is usually <200, associated moncytosis and eosinophilia. Children with early death and severe bacterial infections. Stem cell transplant is curative

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4
Q

Schwachman-Diamond

A

AR. neutropenia, exocrine pancreas failure, short stature, metaphyseal dysostoses.

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5
Q

diagnosis and treatment of LAD deficiency

A

diagnosis by flow cytometry. treatment with BMT. Lifespan of 2 years without it

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6
Q

Job syndrome

A

characteristic facies, hyperextensible joints, osteopenia, scoliosis, elevated IGe, cold abscesses - usually staph. pneumonia with pneumatocele. Txment - should be on bactrim prophy

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7
Q

Chediak-Higashi

A

AR, partial albinism, giant lysosomes in all granulocytes, NK cell dysfunction, neurologic decline. Get bad EBV infections

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8
Q

Evans syndrome

A

AIHA and ITP

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9
Q

TAR syndrome

A

presents in neonatal period with bleeding. Severe thrombocytopenia. No megakaryocytes in bone marrow. Newborns have no radii, but thumbs are normal. Most survive, and plt counts improve over time.

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10
Q

Bernard-Soulier Syndrome

A

mild thrombocytopenia and giant abnormal platelets that do not aggregate in response to ristocetin, but do aggregate in response to ADP, epi, collagen. Prolonged bleeding time. Deficiency of glycoprotein Ib in the platelet membrane - can’t aggregate. Severe mucocutaneous bleeding in infancy

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11
Q

Glanzmann Thrombasthenia

A

AR with normal platelet counts and poor platelet aggreg that does not respond to ADP, epi, collagen

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12
Q

short stature, abnormal radii and thumbs, microcephaly, cafe-au lait spots, renal anomalies. 15% risk of developing AML

A

Fanconi syndrome

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13
Q

Diamond Blackfan anemia

A

Macrocytic anemia and reticulocytopenia. Can have associated thumb abnormalities, short stature, glaucoma, renal anomalies, hypogonadism, short, webbed necks, CHD and intellectual disabililty.

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14
Q

How do you treat the anemia of diamond blackfan?

A

80% respond to steroids

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15
Q

What pathway does PT measure?

A

extrinsic

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16
Q

What pathway does PTT measure

A

intrinsic and common pathways

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17
Q

whats a normal bleeding time?

A

<10min

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18
Q

What would cause prolonged PT, but normal PTT

A

FVII def, liver disease, warfarin

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19
Q

What would cause prolonged PTT, but normal PT

A

heparin or problem with factors XII, XI, IX, VIII (hemophilia)

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20
Q

Both PTT and PT are prolonged

A

multiple factor deficiencies, problem in common pathway, liver disease, DIC

21
Q

Both PT and PTT are normal with increased bleeding

A

FXIII deficiency

22
Q

Factor XII deficiency

A

PTT very prolonged and PT normal, decreased FXII. Do not clinically have a bleeding disorder and can undergo surgery

23
Q

What are the naturally occuring anticoagulants

A

Protein C, s, antithrombinq

24
Q

Which conditions are associated with ALL?

A

down syndrome, Bloom syndrome, ataxia-telangestasia, fanconi anemia, neurofibromatosis

25
Q

What percent blasts need to be in BM for diagnosis of ALL?

A

25%

26
Q

Which factors portend a worse prognosis in ALL

A

age <1 or >10, presence of Phil Chr 9, 22 or {4,11}. WBC ct>50K on presentation, mature B cell or t cell, african american or hispanic ethnicity

27
Q

Side effects of 6-MP

A

myelosuppression and hepatotoxicity

28
Q

side effects of L asparaginase

A

pancreatitis, increased glucose, thrombosis

29
Q

side effect of etoposide

A

2ndary leukemias

30
Q

cisplatin side effects

A

nephrotoxic, ototoxic, neurotoxic

31
Q

WAGR syndrome

A

wilms tumor, aniridia, GU abnormalities, intellectual disability

32
Q

Denys drash

A

wilms tumor, nephropathy, hemihermaphrodism

33
Q

PArinaud syndrome

A

upward gaze impairment, dialted pupils with better reactivity to accomodation than to light, retraction or conversion nystagmus with lid retraction - seen with pineal tumors

34
Q

what is the youngest age typically do brain radiation?

A

3yo

35
Q

Tumor markers of choriocarcinoma

A

(-) AFP, (+) BHcg

36
Q

Tumor markers of germinoma (seminoma or dysgerminoma)

A

(-) AFP, (-) BHcg

37
Q

Tumor markers of embryonal carcinoma

A

(+) AFP, (+) BHcg

38
Q

Tumore markers of yolk sac tumor

A

(+) AFP, (-) BHcg

39
Q

What do Leydig cell tumors produce?

A

androgens

40
Q

What do Sertoli cell tumors produce

A

estrogens

41
Q

A child under the age of 6 months with multiple liver lesions and a normal AFP most likely has

A

hemangioendothelioma

42
Q

what age is hepatoblastoma most likely in

A

<3

43
Q

Can hepatocellular carcinoma cause increased AFP

A

yes. 50% do

44
Q

hemangioendothelioma

A

most common benign liver lesion of childhood. no biopsy necessary. solid lesions can be resected if necessary. If unresectable or consumption coagulopathy is present, treat with steroids

45
Q

In which conditions are astrocytomas associated?

A

neurofibromatosis type 1 or tuberous sclerosis

46
Q

In which conditions is leukemia associated

A

Fanconi anemia, ataxia-telangiectasia, Bloom syndrome, and trisomy 21.

47
Q

In which conditions is neuroblastoma associated?

A

Neuroblastoma can be seen in hereditary neuroblastoma because of ALK gene mutations, neurofibromatosis type 1, central congenital hypoventilation syndrome due to PHOX2b gene mutations, and Hirschsprung disease

48
Q

What does leukoreduction of blood do?

A

reduced the PMNs in blood (not lymphocytes). These usualyl cause cytokine release and decrease risk of febrile reaction

49
Q

What does irradiation do?

A

decreased lymphocytes that can cause GVHD in immunocompromised patients.

Pediatrics (25 decks)

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