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Flashcards in Heme/Onc Deck (49):
1

What is the incidence of febrile transfusion reactions?

1% for pRBC and 10% for platelet transfusions

2

Diagnostic criteria of CVID:

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

3

Kostmass syndrome or Familial severe neutropenia

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

4

Schwachman-Diamond

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

5

diagnosis and treatment of LAD deficiency

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

6

Job syndrome

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

7

Chediak-Higashi

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

8

Evans syndrome

AIHA and ITP

9

TAR syndrome

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.

10

Bernard-Soulier Syndrome

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

11

Glanzmann Thrombasthenia

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

12

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

Fanconi syndrome

13

Diamond Blackfan anemia

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

14

How do you treat the anemia of diamond blackfan?

80% respond to steroids

15

What pathway does PT measure?

extrinsic

16

What pathway does PTT measure

intrinsic and common pathways

17

whats a normal bleeding time?

<10min

18

What would cause prolonged PT, but normal PTT

FVII def, liver disease, warfarin

19

What would cause prolonged PTT, but normal PT

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

20

Both PTT and PT are prolonged

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

21

Both PT and PTT are normal with increased bleeding

FXIII deficiency

22

Factor XII deficiency

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

23

What are the naturally occuring anticoagulants

Protein C, s, antithrombinq

24

Which conditions are associated with ALL?

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

25

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

25%

26

Which factors portend a worse prognosis in ALL

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

Side effects of 6-MP

myelosuppression and hepatotoxicity

28

side effects of L asparaginase

pancreatitis, increased glucose, thrombosis

29

side effect of etoposide

2ndary leukemias

30

cisplatin side effects

nephrotoxic, ototoxic, neurotoxic

31

WAGR syndrome

wilms tumor, aniridia, GU abnormalities, intellectual disability

32

Denys drash

wilms tumor, nephropathy, hemihermaphrodism

33

PArinaud syndrome

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

what is the youngest age typically do brain radiation?

3yo

35

Tumor markers of choriocarcinoma

(-) AFP, (+) BHcg

36

Tumor markers of germinoma (seminoma or dysgerminoma)

(-) AFP, (-) BHcg

37

Tumor markers of embryonal carcinoma

(+) AFP, (+) BHcg

38

Tumore markers of yolk sac tumor

(+) AFP, (-) BHcg

39

What do Leydig cell tumors produce?

androgens

40

What do Sertoli cell tumors produce

estrogens

41

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

hemangioendothelioma

42

what age is hepatoblastoma most likely in

<3

43

Can hepatocellular carcinoma cause increased AFP

yes. 50% do

44

hemangioendothelioma

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

In which conditions are astrocytomas associated?

neurofibromatosis type 1 or tuberous sclerosis

46

In which conditions is leukemia associated

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

47

In which conditions is neuroblastoma associated?

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

What does leukoreduction of blood do?

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

49

What does irradiation do?

decreased lymphocytes that can cause GVHD in immunocompromised patients.