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MCQ by topic > Hematology > Flashcards

Hematology Flashcards

1
Q

What is the mentzer index and how do you use it to determine if microcytic anemia is due to iron deficiency or thalassemia?

A

Mentzer index = MCV/RBC
<13 suggests thalassemia
>13 suggests iron deficiency

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2
Q

What globin chains make up:
HgbA
HgbA2
HgbF
HgbBarts
HgbH
HgbS
HgbSS

A

A: a2b2. (95-98%)
A2: a2o2 (2-3%)
F: a2y2 (0.8-2%)
Barts: y4 (hydrops fettles, 4 mutations)
H: b4 (3 mutations)
S: b2S2
SS: S4

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3
Q

What do you see on bloodwork in kids with AIHA? what is the tx?

A

hemolysis with DAT+
increased retics
increased unconjugated bilirubin
low haptoglobin
increased LDH
urine dip shows free hemoglobin

Tx= steroids 2mg/kg/day and wean over 4-6 weeks

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4
Q

What are 2 causes of pure red cell aplasia?

A

Diamond Blackman anemia
Transient erythroblastopenia of childhood (TEC)

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5
Q

definition of pure red cell aplasia

A

isolated anemia with VERY LOW RETICS

often post viral (ex. Parvo)

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6
Q

presentation of TEC

A

well child b/w 6months-3 yrs, acute reticulopenic anemia, recovery 1-2 months

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7
Q

presentation of Diamond blackfan anemia

A

+/- congenital abnormalities
presents less than age 2
ELEVATED ADA
transfusion dependent
risk of malignancy
low retics

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8
Q

Presentation of beta thalassemia major

A

Skeletal findings/face
HSM
para-aortic masses
Asian, African, mediterranean
Dx: hemoglobin analysis (No A, have A2 and F)
Tx: transfusion, iron chelation

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9
Q

what test do they use to diagnoses hemoglobinopathies?

A

high performance liquid chromatography

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10
Q

2 congenital causes of thrombocytopenia

A

wiskott Aldrich (small platelets, eczema, immunodeficiency)
thrombocytopenia/absent radii (TAR)

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11
Q

Red flag for ITP (aka something else going on) (7)

A

age <12 months
family history of ITP
congenital anomalies
consanguinity
constitutional symptoms
sig lymphadenopathy +/- HSM
lack of response to first line therapy

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12
Q

Definition of ITP

A

platelets <100
newly dignosed <3 months
persistent 3-12 months
chronic >12 months

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13
Q

What type of platelets do you transfuse in NAIT?

A

random pooled platelets (fastest)

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14
Q

in which infants should you automatically check platelets at birth and daily for a few days?

A

all infants to mothers with known autoimmune disease

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15
Q

3 types of von willebrand disease

A

Type1: most common, AD, milder quantitative defect, can treat with DDAVP

Type2: Rare, disorder of VWF function, DDAVP with caution, can make it worse

Type3: Severe, AR, very little/no VWF, act like hemophilia patients (joint and muscle bleeds), no use for DDAVP, needs VWF replacement

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16
Q

Intrinsic pathway factors (4)

A

12, 11, 9, 8

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17
Q

Extrinsic pathway factor (1)

A

7

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18
Q

Common pathway factors (4)

A

10, 5, 2 (prothrombin), 1(fibrinogen)

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19
Q

what factor affected in hemophilia A and hemophilia B?

A

8 in A
9 in B

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20
Q

cause of early hemorrhagic disease of the newborn

A

early = in first 24 hours
maternal anticonvulsants or antiTB meds
prevented by giving bit K to mother in late pregnancy

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21
Q

tx for type III vWF disease

A

factor VIII/von willebrand’s concentrate

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22
Q

vitamin K affects which factors

A

2,7,9,10 therefore intrinsic, extrinsic and common pathways

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23
Q

What globin chains make up:
HgbA

A

A: a2b2. (95-98%)

24
Q

hemoglobin A2 made up of what chains

A

A2o2 (2-3%)

25
Q

HgbF

A

a2gamma2

26
Q

HgbBarts

A

y4 (hydrops fetalis)

27
Q

Hgb H

A

Beta4 (3 mutations)

28
Q

HgbS

A

B2S2

29
Q

HgbSS

A

S4

30
Q

lab abnormalities in TEC

A

mod-severe anemia
MVC normal
low retics
may see neutropenia
platelets may be normal or increased

31
Q

red flag feature of TEC

A

if >1 RBC transfusion, think of alternative diagnosis

32
Q

common oxidants causing G6PD exacerbations

A

sulfas
nitrofurantoin
dapsone
naphthalene (moth balls)
anti-malarials
rasburicase
fava beans
aspirin
infections
DKA
hepatitis

33
Q

true or false: splenectomy is treatment for G6PD

A

false –not useful. Tx is supportive care and avoidance of triggers

34
Q

HgbA > HbS = ?

A

sickle cell trait

35
Q

HgbS&raquo_space; HgbF and no hgbA = ?

A

sickle cell disease –either SCD-SS or SCD-SBo

36
Q

HgbS&raquo_space; Hgb A and Hgb F =?

A

most likely SCD-SB+

37
Q

HgbS and HgbC = ?

A

SCD-SC

38
Q

what are risk factors for stroke in SCD?

A

SS genotype
low baseline Hb
previous TIA
high blood pressure
rate of and recent episode of acute chest crisis

39
Q

What is the treatment of stroke prevention in SCD?

A

chronic blood transfusion to maintain HgbS <30%

40
Q

treatment of gallstones in SCD?

A

elective cholecystectomy

41
Q

3 indications for exchange transfusion in sickle cell disease

A

stroke
severe ACS
pre-op for major surgeries

42
Q

indicators of poor prognosis is SCD

A

dactylics before age 1
hgb concentration <7g/dL
leukocytosis without infection

43
Q

2 electrolyte abnormalities caused by massive blood transfusion

A

hyperkalemia
hypocalcemia (citrate in blood product chelates calcium ions)

44
Q

Most common infection from blood transfusion

A

HepB (1 in 1.1-1.7 million)

45
Q

causes of elevated PT/INR AND PTT (4)

A

DIC
vit K deficiency
liver disease
heparin

46
Q

how to differentiate PT/PTT prolongation from vit K deficiency vs DIC

A

low platelets in DIC

47
Q

Does hemophilia A affected PT PTT or both?

A

PTT, factor 8 deficiency, extrinsic pathway

48
Q

somebody who doesn’t respond to heparin might have what disease?

A

antithrombin deficiency

49
Q

_________ is valuable and the diagnostic test of choice to detect a PE

A

Helical or spiral CT with an intravenous contrast agent is valuable and the diagnostic test of choice to detect a PE

50
Q

What 3 lab abnormalities might you see in Kasabach-Merritt Sydrome

A

severe thrombocytopenia, hypofibrinogenemia, elevated D dimer (caused by large hemangioma causing intravascular coagulation with thrombocytopenia and hypofibrinogenemia)

51
Q

Red flags suggesting alternate diagnosis in ITP (ie. need bone marrow biopsy)

A

B symptoms
bone pain
recurrent thrombocytopenia
poor treatment response
lymphadenopathy
HSM
child is “unwell”
signs of chronic disease
low hb (unless mildly low and explained by bleeding)
high MCV
abnormal WBC/neutrophil
Abnormal cellular morphology

52
Q

classification of severity of ITP and management

A
53
Q

Left supraclavicular lymph node due to what?

A

metastatic malignancy from the abdomen

54
Q

Right supraclavicular lymph node due to what?

A

malignancy from the mediastinum

55
Q

what does presence of megakaryoblasts on peripheral smear indicate?

A

transient myelproliferative disorder

56
Q

7 syndromes the predispose to leukemia

A

Trisomy 21
NF1
Ataxia Telangiectasia
Fanconi Anemia
Li-Fraumeni Syndrome
Bloom syndrome
Noonan syndrome

57
Q

4 syndromes associated with wilm’s tumor

A

WAGR, Denys-Drash, NF1, Beckwith-Wiedemann

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