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Royal Super 4th Year Brain > Oncology/Hematology > Flashcards

Oncology/Hematology Flashcards

1
Q

What contraindication must you check for prior to starting a DOAC?

A

Antiphospholipid syndrome

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2
Q

What inherited thrombophilia can cause heparin resistance? What is the inheritance pattern? What chemotherapeutic can cause this?

A

Antithrombin III deficiency

  • autosomal dominant
  • asparginase
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3
Q

Most common risk factors for VTE in children

A
  • Most common = central-access vascular catheter
  • Localized or systemic infections + inflammatory disorders
  • Inherited thrombophilias
  • Malignancy
  • Estrogen containing contraceptives
  • Trauma
  • Immobility
  • Recent surgery
  • Obesity
  • Congenital heart disease
  • Nephrotic syndrome
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4
Q

Worrisome clinical features of peripheral LAD (multiple - nodes, history, investigations, etc.)

A
  • Systemic symptoms
  • Supraclavicular node
  • Generalized LAD
  • Fixed, non-tender, matted nods
  • LN >1cm in neonatal period
  • LN >2cm that increase in size + do not respond to antimicrobials
  • Abnormal CXR
  • Abnormal CBC
  • Elevated LDH
  • Absence of symptoms in the ear/nose/throat
  • Persistently elevated ESR/CRP despite antimicrobial therapy
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5
Q

TLS labs

A

Hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia

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6
Q

Major differential for TEC + differentiating features

A

Diamond-Blackfan Syndrome
- Age (in infancy), no preceding viral illness, non-heme abnormalities (craniofacial abnormalities, growth failure, developmental delays), progressive macrocytic anemia

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7
Q

Langerhans cell histiocytosis - endocrinopathy

A

Pituitary involvement - central DI

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8
Q

Best way to investigate for pheochromocytoma

A

Plasma metanephrines

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9
Q

Pheochromocytoma - where is tumour found?

A

Adrenal medulla

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10
Q

MEN1/Type 1

A
  • Three P’s: hyperparathyroidism, pituitary adenoma, pancreatic islet cell tumor
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11
Q

Most common inherited colorectal cancer

A

Lynch Syndrome

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12
Q

Adverse effect from bleomycin

A

Pulmonary fibrosis

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13
Q

Adverse effect from etoposide

A

Secondary leukemia

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14
Q

Adverse effect cyclophosphamide

A

Hemorrhagic cystitis

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15
Q

Major non-malignant long-term complication of Hodgkin Lymphoma treatment

A

Cardiovascular complications

- cardiomyopathy

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16
Q

Malignancy that can cause Horner Syndrome

A

Neuroblastoma

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17
Q

What kind of blood do you give to DiGeorge?

A

Irradiated

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18
Q

What is the best preventative treatment for stroke in HbSS?

A

Transfusions

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19
Q

Goal for HbF for HbSS

A

Less than 30%

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20
Q

Blood smear for iron deficiency anemia

A

Hypochromic cells

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21
Q

What platelets to give to baby with NAIT?

A

Washed maternal platelets
Or PLA1 negative platelets
+/- IVIG to prolong platelet survival

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22
Q

Wiskott Aldrick Syndrome - triad

A

Immunodeficiency
Micro thrombocytopenia
Eczema

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23
Q

Ulcerated tonsils/pharynx - what malignancy?

A

Leukemia

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24
Q

Diagnosis of neonatal thrombocytopenia when maternal platelet count is normal

A

Neonatal alloimmune thrombocytopenia

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25
Q

Difference between allopurinol and rasburicase

A
  • Allopurinol prevents creation of uric acid

- Rasburicase makes uric acid more soluble (better agent if already making uric acid)

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26
Q

When to consider BMA for ITP

A
  • features to suggest malignancy or BM failure (e.g., bicytopenia, LAD)
  • insufficient or no response to steroids and/or IVIG
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27
Q

3 buckets of symptoms for leukemia

A
  • lack of normal BM output
  • infiltration by blasts
  • sanctuary site
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28
Q

x2 sanctuary site for leukemia

A

CNS and testicular

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29
Q

7 syndromes that predispose you to leukemia

A
  • T21
  • Neurofibromatosis type 1
  • Noonan Syndrome
  • Bloom Syndrome
  • Ataxia telangectasia
  • Fanconi anemia
  • Li Fraumeni Syndrome
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30
Q

T21 specific malignancy concern + presentation

A

Transient myeloproliferative disorder

  • High WBC
  • Blasts
  • Pancytopenia
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31
Q

Non-marrow site for leukemia

A

CNS, testes, HSM, bone, skin, LN, mediastinum

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32
Q

What type of leukemia presents with DIC?

A

APML (acute promyelocytic leukemia)

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33
Q

To make diagnosis of leukemia you need….

A

> 25% blasts in the bone marrow

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34
Q

Most common type of ALL

A

B cell (80%)

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35
Q

What makes a T cell ALL more likely (x3)?

A

Boys
Large mediastinal mass
High WBC count

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36
Q

Pathopneumonic sign for AML on smear

A

Auer rods

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37
Q

High risk features for B cell ALL (x5)

A 
Age <1 year and > 10 years
Initial WBC >50x10^9
CNS and testicular disease
Cytogenetic abnormalities: Philadelphia chromosome, MLL rearrangement, hypodipoloidy
Poor response to induction chemotherapy
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38
Q

Induction chemo for ALL

A

Vincristine
Steroids
Peg-asparaginase

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39
Q

Methotrexate side effects (x3 system side effects)

A
  • liver dysfunction
  • renal dysfunction
  • neurotoxicity
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40
Q

Cytarabine side effects

A
  • flu like symptoms
  • neurotoxicity
  • corneal toxicity
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41
Q

Prophylaxis given in immunocompromised host (x2)

A
  • PJP = Sentra

- fungal = fluconazole and caspofungin

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42
Q

What lymphoma is most common?

A

Non-Hodgkin lymphoma (60%)

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43
Q

Complications of mediastinal mass

A
  • Airway compromise

- SVC syndrome

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44
Q

Late effects of Hodgkin lymphoma

A
  • hypothyroidism
  • infertility
  • cardiomyopathy
  • pulmonary fibrosis
  • solid tumor (breast, thyroid, skin)
  • MDS/leukemia
  • avascular necrosis
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45
Q

Risk factors for non-hodgkin lymphoma

A

-immunodeficiency: WAS, ataxia telangiectasia, congenital hypogammaglobinemia, post-transplant, HIV

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46
Q

3 types of lymphoma

A
  • Lymphoblastic lymphoma
  • Burkitts lymphoma
  • anaplastic large cell lymphoma or diffuse large B-cell lymphoma
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47
Q

What virus is related to Burkitt lymphoma?

A

EBV

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48
Q

What lymphoma should be on your ddx for an abdominal mass?

A

Burkitt lymphoma

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49
Q

Risk factors for TLS

A
  • tumor type: burkitts, leukemia
  • timing: increased risk during first 72 hours of treatment
  • renal/cardio dysfunction: may not tolerate therapy
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50
Q

Where is the brain tumor if:
A) head tilt
B) behavioural change

A

A) posterior fossa

B) supratentorial

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51
Q

Types of tumours in posterior fossa

A
  • pilocytic astrocytoma
  • medulloblastoma
  • ependymoma
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52
Q

Localizing signs for supratentorial

A
  • hemiparesis
  • hemisensory loss
  • hyperreflexia
  • seizures
  • visual changes
  • behavioural
  • diencephalic syndrome of infants
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53
Q

Triad for infratentorial tumor

A
  • long tract signs
  • ataxia
  • cranial neuropathies
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54
Q

Side effects of brain radiation

A 
Cerebral edema
Moya moya
Skin toxicity
Radiation necrosis
Endo dysfunction
Cognitive impairment
Secondary brain tumor
Somnolence syndrome
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55
Q

Two most common sympathetic chain locations for neuroblastoma

A

Adrenal glands

Paraspinal nerves

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56
Q

What scan is specific for neuroblastoma?

A
  • MIBG scan
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57
Q

What age group has the best prognosis for neuroblastoma?

A

<18 months old

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58
Q

Genetic predispositions for wilm’s tumor

A
  • WAGR
  • Beckwith Wiedemann
  • Denys-Drash
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59
Q

Most common renal tumor

A

Wilm’s

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60
Q

Most common liver tumor

A

Hepatoblastoma

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61
Q

Most helpful lab for hepatoblastoma

A

AFP

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62
Q

Common place for mets with sarcomas

A

Lungs

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63
Q

Most common soft tissue sarcoma

A

Rhabdomyosarcoma

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64
Q

Two most common bone sarcoma

A

Ewing and osteosarcoma

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65
Q

What bone sarcoma is typically in the center location in the transverse plane?

A

Ewing sarcoma

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66
Q

Classic XR findings for bone sarcoma

A
  • osteosarcoma - sunburst

- ewing sarcoma - onion skin

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67
Q

How is retinoblastoma inherited?

A

Autosomal dominant (30% of cases)

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68
Q

Diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH)

A

5/8:

  • fever
  • cytopenias
  • splenomegaly
  • high ferritin
  • hypertriglyceridemia
  • hypofibrinogenemia
  • absent NK activity
  • increased soluble IL-2 receptor
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69
Q

CNS tumours - genetic predisposition

A
  • NF1 and 2

- tuberous sclerosis

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70
Q

Genetic predisposition with neuroblastoma

A
  • PHOX2B mutation

- beckwith-wiedemann syndrome

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71
Q

Late effects of radiation

A
  • secondary malignancy
  • pituitary dysfunction
  • hypothyroidism
  • pulmonary fibrosis
  • metabolic syndrome/stroke
  • poor growth
  • infertility
  • learning/psychosocial difficulties
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72
Q

Acute toxicity of radiation

A
  • skin irritation
  • n/v
  • myelosuppression
  • mucositis
  • fatigue
  • esophagitis/dysphasia
  • alopecia
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73
Q

BMT complications

A
  • infection
  • acute GVHD (skin, gut, liver)
  • veno occlusive disease of the liver or sinusoidal obstructive syndrome
  • graft failure
  • post transplant lymphoproliferative disorder (PTLD; usually EBV related)
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74
Q

Most common trigger for ITP and treatment options

A

Infection (~50% of cases)

  • Observation
  • Low plt precautions
  • IVIG
  • Oral CC’s
  • Medication-refractory: splenectomy, rituximab
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75
Q

Further disorders to r/o if picture is no longer looking like ITP

A
  • SLE and collagen vascular disease
  • Common variable immune deficiency
  • Myelodysplastic syndrome or BM failure
  • Viral infection
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76
Q

What cancer preventing vaccines are there?

A
  • HPV

- Hep B (hepatocellular carcinoma)

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77
Q

Beta thalasemia - genotypes + clinical features

A

B/B = normal
B+/B or B0/B = beta minor = mild to no anemia
B+/B+ or B0/B+ = beta intermedia = micro anemia, HSM, growth failure, extra-medullary hematopoiesis
B0/B0 = thal major = severe anemia, tx dependent

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78
Q

DVT risk factors

A

Immobilization, surgery, trauma, line/catheter, OCP, pregnancy, smoking, obesity, malignancy

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79
Q

Autoimmune hemolytic anemia - differences between warm and cold erythrocyte Ab

A 
Warm = IgG, against Rh Ab, extravascular, associated with systemic disease (e.g., SLE), tx with immunosuppression/splenectomy
Cold = IgM, against I or i Ab, mostly intravascular (+complement), associated with infection (e.g., EBV), tx with warmth
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80
Q

Hb barts

A

Tetramer of delta-globin chains

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81
Q

x2 ways to differentiate IDA vs thalasemia on CBC

A

Metzner index (>12 in IDA) + RDW (high in IDA)

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82
Q

What is the cause for hemolytic anemia in a patient with acute anemia, fatigue, jaundice, and dark urine following early spring swim?

A

Paroxysmal cold hemoglobinuria = transient autoimmune hemolysis secondary to Donath-Landsteiner Ab (attaches at cold temperatures –> complement cascade –> detaches once warm + lysis continues), DAT usually negative

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83
Q

Clinical presentation of Fanconi Anemia (system based)

A

CNS: Dev delays, cognitive deficits
HEENT: Hearing loss, strabismus
CVS: CHD
Resp: TEF
GU: Structural abnormalities
MSK: Short stature, absent/bifid thumbs, vertebral defects
Heme: BM failure, cancer predisposition
Endo: Hypothyroidism, GH deficiency, osteopenia, hyperglycemia, dyslipidemia
Derm: Pigmentation differences, cafe au lait

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84
Q

Age of BM failure in Fanconi Anemia

A

5 years

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85
Q

Causes of acquired BM failure

A

ID: EBV, parvo, HIV
Meds: AEDs, chemo, radiation
Onc: Pre-leukemia
Heme: Paroxysmal nocturnal hemoglobinuria
Imm: Eosinophilic fasciitis, hypogammaglobinemia

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86
Q

x2 associated neurological disorders to iron deficiency anemia

A

ADHD and RLS

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87
Q

Risk factors and at risk populations for iron deficiency anemia

A

Low BW, prematurity, no iron rich foods after 6 months, early introduction to cow’s milk, ++ milk, lead exposure, low SES, poor nutrition, picky eating, menorrhagia, athletes, chronic disease, alternative diets, obesity

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88
Q

Most common cause of transient neutropenia + pathophysiology

A

Viral infection = influenza, adeno, CMV, EBV, VZV, measles, hep A+B, coxsackie
- Patho: Marrow suppression, neut redistribution, sequestration in reticuloendothelial tissue

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89
Q

Most common cause of chronic childhood neutropenia

A

Autoimmune neonatal neutropenia (ANI) = resolves by 4 y/o, normal BM cellularity/arrest of late metamyelocytes, anti-neutrophil Ab’s detected (mostly)

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90
Q

Classic triad for Wiskott-Aldrich Syndrome

A

Micro-thrombocytopenia, eczema, and immunodeficiency (usually combined type)

91
Q

Etiology for thrombocytosis

A
  • Reactive: anemia/hemolysis/blood loss, infection, inflammation, post-splenectomy
  • Primary: heme malignancies (polycythemia vera, CML, essential thrombocythemia), familial thrombocytosis
92
Q

Clinical signs/symptoms for hemolytic anemia

A

Tachycardia, pallor, jaundice, organomegaly, discoloured urine (dark, brown, red)
-Severe = hypovolemic shock or CHF

93
Q

x2 findings on blood smear you could see with hemolytic anemia

A

Schistocytes, spherocytes

94
Q

Complications of iron overload

A
  • CVS: CHF, dysrhythmia, pericarditis
  • Liver: cirrhosis, fibrosis
  • Endo: growth/sexual development delays, hypoparathyroidism, hypothyroidism
95
Q

Iron overload management

A
  • Chelation therapy
  • Diet: drink tea
  • Splenectomy
  • Erythrocytapheresis/RBC exchange
96
Q

Causes of prolonged INR

A
  • Vitamin K deficiency
  • Warfarin
  • Liver disease
  • Factor VII deficiency
97
Q

Causes of prolonged PTT

A
  • Heparin
  • Hemophilia
  • vWD
  • Antiphospholipid antibodies
98
Q

Causes of combined prolonged PT/PTT

A
  • Heparin
  • Warfarin
  • Liver disease
  • DIC
99
Q

Vitamin K dependent coagulation factors

A

II, VII, IX, X, protein C + S

100
Q

Factors in common coagulation pathway

A

V, X, I, II

101
Q

Common hereditary disorders that predispose to thrombosis (x6)

A

Factor V Leiden, protein C deficiency, protein S deficiency, antithrombin III deficiency, hyperhomocysteinemia, antiphospholipid Abs

102
Q

Timing of anemia nadir for infants

A
  • Term = 8-12 weeks

- Preterm = 6-8 weeks

103
Q

x2 pathophysiologic mechanisms in sickle cell anemia

A
  • Hemolysis: intra + extravascular

- Vaso-occlusion: acute exacerbations (pain crisis, stroke), chronic disease exacerbations (retinopathy, renal disease)

104
Q

Most common cause of death in sickle cell

A

Infection - primarily pneumococcal with functional asplenia

105
Q

Transfusion dosing for pRBC’s, platelets, and FFP?

A
  • pRBC’s + FFP = 10-15 mL/kg

- platelets = 10 mL/kg

106
Q

Coombs test - direct vs indirect

A
  • Direct: pt’s washed RBC’s + Coombs serum (antihuman globulin) = agglutination if pt’s RBC’s have been coated in vivo by an Ab
  • Indirect: pt’s serum + RBC’s of known type + Coombs serum = agglutination if Abs in the serum are binding to Ag’s on RBC’s
107
Q

Cryoprecipitate - what is it and indications for use?

A

=plasma product of concentrated factors (VIII, XIII, vWF, fibrinogen) from FFP
-Given when specific factors are NOT available, to reverse anticoagulation, or DIC

108
Q

Howell-Jolly bodies - what are they? what conditions are they seen in?

A

= nuclear remnants of normal RBC maturation, usually removed by spleen
-Found in patients with decreased/absent splenic function

109
Q

Menorrhagia - what bleeding d/o to screen for?

110
Q

vWD - what factor is impacted? what coag is affected?

A

PTT, decreased fVIII antigen + activity

111
Q

What is von Willibrand factor?

A
  • Synthesized in megakaryocytes + endothelial cells
  • Large multimeric protein –> binds to collagen at endothelial injury = bridge between injury + adherence of platelets
  • Carrier protein for fVIII
112
Q

alpha-thal clinical features + genotypes

A 
aa/aa = normal
a-/aa = silent carrier, lower normal MCV
a-/a- = a-thal trait, mild microcytic anemia
a-/-- = HbH disease, moderate microcytic anemia, splenomegaly, jaundice
--/-- = a-thal major, severe intrauterine anemia, hydrops fetalis
113
Q

Typical age range for ITP

114
Q

Three main options for management in ITP

A
  • Observation
  • CC’s
  • IVIG
115
Q

Progression of labs in iron deficiency anemia

A
  1. Ferritin
  2. Transferrin saturation
  3. MCV + Hb
116
Q

What x3 bleeding disorders are on the ddx with prolonged PTT and normal INR?

A
  • Hemophilia A and B
  • VWD
  • factor XI deficiency
117
Q

TEC + Diamond Blackfan Anemia - similarities

A
  • Disorder of RBC production during early childhood

- Low Hb and inappropriately low retics

118
Q

TEC + Diamond Blackfan Anemia - differences

A
  • TEC = self-limited, presents >1 year of age
  • DBA = requires life-long treatment, presents during first 6 months, elevated adenosine deaminase, ribosomal protein mutation
119
Q

Presentation of hepatic sinusoidal obstructive syndrome - symptoms/signs, when typically presents, x1 complication

A
  • Hepatomegaly, RUQ pain
  • Jaundice
  • Ascites/weight gain
  • Within first 3 weeks of BMT
  • Hepatorenal syndrome
120
Q

x3 causes of pure red cell aplasia and what does this term mean?

A

=isolated anemia with VERY low retics

  • Aplastic anemia (from parvo)
  • Diamond Blackfan Anemia
  • TEC
121
Q

Types of intrinsic hemolytic disorders

A
  • Membranopathy: spherocytosis, elliptocytosis
  • Enzymopathy: G6PD deficiency, PK deficiency
  • Hemoglobinopathy: HbSS, B-thal
122
Q

Types of extrinsic hemolytic disorders

A
  • Autoimmune: Warm (IgG), cold (IgM), paroxysmal nocturnal hemoglobinuria
  • Hemolytic disease of the newborn
  • Non-immune HA: HUS, TTP, DIC
123
Q

DDx for macrocytic anemia

A
  • Megaloblastic: B12 or folate deficiency

- Non-megaloblastic: BM failure, hypothyroidism, T21, chronic liver disease, drugs

124
Q

Iron supplementation

A

PO iron 3-6 mg/kg/day

125
Q

How quickly will you notice a response from iron supplementation?

A
  • Subjective improvement
  • Reticulocytosis within 3-7 days
  • Increased Hb 7-30 days
  • Repletion of iron stores by 3 months
126
Q

What should you take to improve iron supplementation absorption?

A

-Orange juice (vitamin C)

127
Q

What lab could you add on to iron studies in setting of potential inflammation?

A

Add CRP to ferritin

128
Q

4 mechanisms of iron defiency

A
  • Inadequate stores at birth
  • Insufficient iron in diet
  • Blood loss
  • Malabsorption
129
Q

What is the leading cause death in SCD?

130
Q

What is a side effect to monitor for with hydroxyurea?

A

Myelosuppression

Neutropenia

131
Q

Classic facial findings in Beta Thalassemia Major

A
  • Chipmunk facies
  • Frontal bossing
  • Maxillary expansion
  • Saddle nose
  • Depressed cranial vault
132
Q

What would Hb analysis show in Beta Thalassemia Major?

A
  • No HbA

- All HbA2 and HbF

133
Q

Two main problems (+their associated disorder examples) in hemoglobinopathies?

A
  • Problem with Hb quantities = thalassemia

- Problem with Hb quality = SCD

134
Q

What is now used today instead of hemoglobin electrophoresis?

A

High performance liquid chromatography (HPLC)

135
Q

What is the name of the hemoglobin that is gamma-4 (Y4) + what does it represent?

A

Hemoglobin barts

- suggests alpha thalassemia

136
Q

What is the name of the hemoglobin that is beta-4 (B4) + what does it represent?

A

Hemoglobin H

- suggests alpha thalassemia

137
Q

What is HbF made up of?

A
  • 2 alpha chains

- 2 gamma chains

138
Q

What is HbA2 made up of?

A
  • 2 alpha chains

- 2 delta chains

139
Q

What does (a) a little bit of HbA2 vs (b) a lot of HbA2 suggest?

A

(a) normal

(b) beta thal

140
Q

Typical clinical presentation of Hereditary Spherocytosis?

A
  • Mild-moderate anemia
  • Jaundice
  • Occasional hemolytic episodes
  • Neonatal jaundice
141
Q

How to diagnose hereditary spherocytosis?

A

EMA testing by flow cytometry - osmotic fragility

142
Q

Complications of hereditary spherocytosis (x3)?

A
  • Hemolytic anemia (with intercurrent illness)
  • Aplastic crisis (with parvo especially)
  • Gallstones
143
Q

x3 potential management strategies for hereditary spherocytosis?

A
  • Transfusion for symptomatic anemia
  • Folate (for patients with high retics and high MCV)
  • Splenectomy
144
Q

x4 common triggers for G6PD

A
  • Moth balls
  • Fava beans
  • Sulfa drugs
  • Anti-malarials
  • Anti-pyretics
145
Q

x4 indications for splenectomy in childhood

A
  • Traumatic splenic rupture
  • Congenital hemolytic anemias requiring ongoing transfusion support
  • Sickle cell anemia with recurrent splenic sequestration
  • Severe, symptomatic chronic ITP with failure to respond to medical management
146
Q

x2 main risks of splenectomy

A
  • Infection (encapsulated bacteria)

- Thrombosis

147
Q

Ddx for thrombocytopenia

A
  • Production problem: congenital (WAS, TAR, CAMT, BM failure syndromes), acquired (marrow infiltration, aplastic anemia, chemo)
  • Destruction problem: immune (ITP, NAIT, SLE, heparin/drug), non-immunw (DIC, HUS, TTP, infection, Kasabach-Merritt, artificial heart valve)
  • Loss: massive transfusion, surgery
  • Consumption: splenic sequestration
148
Q

Red flags for ITP

A
  • <12 months of age
  • Family hx of “ITP”
  • Congenital anomalies
  • Consanguinity
  • Constitutional symptoms
  • Significant LAD and/or HSM
  • Unresponsive to therapy
149
Q

When is ITP considered (a) persistent vs (b) chronic?

A

(a) 3-12 months

(b) >12 months

150
Q

Essential/basic evaluation (hx, PEx, labs) in keeping with ITP?

A
  • Hx: isolated bleeding sx
  • PEx: No HSM, no congenital abN, no LAD
  • Ix: Plts <100, anemia from bleeding, smear (normal to big plts)
151
Q

First line treatment options for acute ITP (include options if severe bleeding)

A
  • Steroids, IVIG, anti-RhD

- If severe bleeding = IV methylpred + IVIG + platelet transfusion +/- TXA

152
Q

Steroids vs IVIG for ITP

A
  • Steroids: slower (48-72h), could mask malignancy, can do as outpatient
  • IVIG: faster action (24-48h), requires inpatient
153
Q

DDx for neonatal thrombocytopenia - based on (a) fetal/newborn, (b) <72 hours, (c) >72 hours, and (d) throughout neonatal period?

A

(a) Alloimmune, TORCH, chromosomal abnormality, severe Rh disease
(b) Chronic fetal hypoxia, perinatal asphyxia, perinatal infection (E.coli, GBS) > thrombosis, Kasabach-Merritt, metabolic disease
(c) Late-onset sepsis, NEC
(d) Congenital (WAS, CAMT, TAR), neonatal autoimmune

154
Q

Treatment for neonatal autoimmune thrombocytopenia?

A
  • IVIG

- Transfusion if bleeding

155
Q

If mother has an autoimmune condition, what kind of monitoring is recommended for baby?

A

Check platelets at birth and daily for a few days

156
Q

Can NAIT occur during the first pregnancy?

157
Q

What kind of Ab (immunoglobulin) of mother’s passes to newborn via placenta?

A

IgG due to HPA mismatch

158
Q

What is more severe and significant for newborns - NAIT vs neonatal autoimmune?

159
Q

x3 most common congenital thrombocytopenia syndromes?

A
  • Wiskott-Aldrich Syndrome
  • TAR (thrombocytopenia absent radius)
  • CAMT (congenital amegakaryotic thrombocytopenia)
160
Q

Tx for NAIT

A
  • HPA/PLA-1 negative platelets
  • Maternal washed platelets
  • IVIG
161
Q

What is the timeline for when neutropenia becomes chronic?

A

> 3-6 months

162
Q

DDx for acquired neutropenia

A
  • Infection: post-infection, viral, bacterial
  • Immune mediated: neonatal alloimmune neutropenia, primary autoimmune neutropenia, secondary autoimmune conditions, aplastic anemia
  • Drug induced
  • Storage disorders
  • Malignancy
  • Chronic idiopathic
  • Nutritional: B12/folate deficiency
163
Q

DDx for congenital neutropenia

A
  • Severe congenital neutropenia
  • Cyclical neutropenia
  • Bone marrow failure syndromes - fanconi anemia, Schwachman-Diamond, Dyskeratosis congenita
  • Immunodeficiency: WAS, CD40 ligand deficiency, X-linked agammaglobulinemia
164
Q

Red flag features for neutropenia

A
  • Infections
  • Growth concerns, FTT
  • Mouth ulcers (especially if cyclic pattern)
  • Other congenital anomalies
  • Consanguinity
  • Family hx of neutropenia, MDS, cancer
  • Other cytopenias
  • No neutrophil response in times of fever/infection
165
Q

What treatment could be considered if neutropenia is persistent and severe?

166
Q

DDx for pancytopenia

A
  • Infections
  • Marrow infiltration: leukemia, lymphoma, neuroblastoma
  • Marrow aplasia: hypocellular, sick bone marrow
  • Megaloblastic anemia: severe B12/folate deficiency
167
Q

Definition and criteria for aplastic anemia

A

= pancytopenia with hypocellular BM in the absence of an abnormal infiltrate or marrow fibrosis
-Criteria - at least two of the following: Hb<100, plt<50, and neut<1.5

168
Q

What must you have to be pancytopenic?

A

At least 2 cell lines involved?

169
Q

Etiology for aplastic anemia

A
  • Acquired: viral suppression, drugs, toxins, idiopathic

- Congenital: Fanconi Anemia, Dsykeratosis congenita, Shwachman Diamond

170
Q

What is the most common inherited bone marrow failure syndrome?

A

Fanconi Anemia

171
Q

Important aspects to include in a newborn bleeding history for a child?

A
  • Excessive bleeding post-circumcision, scalp/brain bleed, immunizations, after cord seperation
  • Vitamin K injection
172
Q

Primary hemostasis vs secondary hemostasis symptoms

A
  • Primary: epistaxis, easy bruising, menorrhagic, mucocutaneous bleeds, trauma, petechiae/purpura
  • Secondary: delayed bleeding after deep lacerations, surgery, blunt trauma, hemorrhage into subcutaneous tissues/joints/muscles/abdo organs
173
Q

3 steps in hemostasis

A
  • Vessel spasm
  • Primary = platelets adhere to injury site + aggregate to form plug
  • Secondary = formation of insoluble fibrin strands + coagulation
174
Q

4 steps in primary hemostasis

A
  1. Injury: endothelium is damaged –> subendothelial collagen exposure
  2. Adhesion: vWF floats -> uncoils when sees subendothelial collagen -> platelets bind to GP1b/9
  3. Activate: plts activate when exposed to GP2B/3A allowing for aggregation
  4. Aggregation: plt plug
175
Q

Investigations for bleeding disorder

A
  • CBC
  • Smear
  • PT/INR, fibrinogen
  • vWD studies
  • Blood group (for interpretation of vWD levels)
  • Factor VIII
  • Factor IX
  • LFTs
  • Renal function tests
176
Q

What is the most common bleeding disorder?

A

Von Willebrand Disease

177
Q

vWD subtypes

A
  1. Most common, vWF works but less of it, AD, DDAVP
  2. Rare, abnormal structure/function of vWF, use DDAVP with caution
  3. Very severe deficiency, AR, similar to hemophilia, don’t use DDAVP
178
Q

How does DDAVP work for vWD?

A

Induces release of von Willebrand antigen from cellular compartments

179
Q

Management considerations for VWD (3 prong approach)

A
  • Increase VWF = DDAVP, replacement of VWF concentrate
  • Inhibit fibrinolysis (TXA)
  • Hormonal therapy (for menorrhagia)
180
Q

What factor deficiencies are (a) hemophilia A and (b) hemophilia B?

A

(a) fVIII deficiency

(b) fIX deficiency

181
Q

What percentage of factor levels defines (a) mild, (b) moderate, and (c) severe hemophilia?

A

(a) >5%
(b) 1-5%
(c) <1%

182
Q

Management for hemophilia

A
  • Prophylaxis of factor = regular tx for patients with severe disease
  • On-demand = mild/moderate patients, give for bleeds
  • Routine vaccines = smallest gauge needle, SC if possible, pressure
  • Follow-up at hemophilia center
183
Q

Early hemorrhagic disease of the newborn (timeline, RF’s, bleeding sites, tx)

A
  • Within first 24 hours
  • RF’s: maternal use of drugs (AEDs, warfarin, TB drugs)
  • Sites: ICH, GI, umbilical stump, bruising
  • Tx: mother vit K late pregnancy
184
Q

Classical hemorrhagic disease of the newborn (timeline, RF’s, bleeding sites, tx)

A
  • Within 2-7 days
  • RF’s: none
  • Sites: ICH, GI, umbilical stump, bruising
  • Tx: vitamin K injection
185
Q

Late hemorrhagic disease of the newborn (timeline, RF’s, bleeding sites, tx)

A
  • Within 1-6 months (even later)
  • RF’s: no newborn vit K, disorders that interfere with vitamin K intake (liver dz, CF, GI, chronic Abx)
  • Sites: ICH, GI, mucocut
  • Tx: parenteral vitamin K
186
Q

What are triggers for DIC?

A
  • Sepsis, severe infection
  • Vascular abnormality (Kasabach-Merritt Syndrome)
  • Trauma
  • Malignancy
  • Severe hemolysis
  • Liver disease
  • Severe protein C deficiency
  • Severe allergic or immunologic reactions
187
Q

DIC - clinical presentation?

A
  • Asymptomatic
  • Bleeding = bruising, petechiae, mucus membrane bleeding
  • Thrombosis = purpura fulminans, organ dysfunction
  • Hemolytic anemia
188
Q

DIC - investigations + results

A
  • Prolonged PT/INR
  • Decreased fibrinogen
  • Thrombocytopenia
  • Elevated D-dimer
  • Decreased protein C+S and anti-thrombin
189
Q

Tx - DIC

A
  • Tx underlying cause
  • Correction of perfusion, oxygenation, and acid-base imbalance
  • Transfuse based on bleeding - not to fix labs (FFP if PTT >2x normal, cryo if fib >1g/L, plt >30 but caution as can increase thrombosis)
  • Anti-coagulation
  • Activated FVIIa
190
Q

Red flags of a lymph node for malignancy (x7)

A
  • Firm, fixed, matted, non-mobile
  • Non-tender
  • > 2cm
  • Supraclavicular
  • Rapidly growing
  • Presence of B symptoms
  • Persistence for >6 weeks
191
Q

If concerning lymph node - what should you do?

A

-Refer to general surgery or ENT for EXCISIONAL biopsy

192
Q

What is included in cryoprecipitate?

A

Factor VIII, fibrinogen, vWF

193
Q

When to use cryoprecipitate?

A
  • Bleeding in patient with fibrinogen <0.8-1.0

- Bleeding with vWD or hemophilia ONLY if factor or DDAVP is unavailable

194
Q

When to use FFP?

A
  • If needing rapid reversal of warfarin

- For active bleeding or surgery or DIC if coags >1.5x normal

195
Q

Platelet transfusion thresholds

A
  • <10
  • <20 = if coagulopathy
  • <50 = for surgery, epidurals, LPs
  • <100 = for neurosurgery, head trauma
196
Q

What is the most common reason for a transient fever during a blood transfusion?

A

Sensitization to WBC antigens

197
Q

Draw chemo man

198
Q

What GI complication is found in 50% of newborns with TAR?

A

CMPA - can make things difficult with ++bleeding from thrombocytopenia

199
Q

Calculation for ANC

A

= WBC x %neutrophils (segmented+bands) x 10

200
Q

What causes elevated AFP?

A
  • Yolk sac tumors
  • Hepatocellular cancer
  • T21
  • Ataxia telangiectasia
  • Hepatoblastoma
201
Q

What organs are affected by leukocytosis?

A
  • Brain
  • Lungs
  • Kidneys
202
Q

What immediate steps do you need to take with a mediastinal mass?

A
  • No sedation + do NOT lie flat
  • Biopsy –> once results back can then start steroids
  • Airway management
  • Start chemo
203
Q

Complications of a mediastinal mass

A
  • SVC syndrome
  • Pericarditis/pericardial effusion
  • Respiratory distress
204
Q

Who is at highest risk for mediastinal mass?

A
  • Male
  • Teenager
  • T-cell lymphoma
  • Burkitt lymphoma
205
Q

How to treat hypoCa and hyperPO4 in TLS?

A
  • Phosphate binders
  • Calcium supplement
  • Fluids
206
Q

What will you find on smear for ITP?

A

Big baby platelets

207
Q

What is the best initial test for pheo if (a) high suspicion vs (b) low suspicion?

A

(a) plasma metanephrines

(b) urine catecholamines

208
Q

Most common endo abnormality in Langerhans?

A

Central DI

209
Q

MEN2 A

A
  • Medullary thyroid cancer
  • Pheo
  • Hyperparathyroidism
210
Q

MEN2 B

A
  • Medullary thyroid cancer
  • Pheo
  • Marfanoid features
  • Mucosal neuromas
211
Q

Lynch syndrome = most common inherited colorectal cancer

  • Inheritance?
  • Amsterdam criteria?
  • When to start screening?
A
  • Auto dominant
  • 3 family members, 2 generations involved, 1 family member <50 years old
  • Start colonoscopy at 20 years of age
212
Q

Ataxia, nystagmus, and muscle jerks - dx?

A

Neuroblastoma

213
Q

Example of an antracycline

A

Doxorubicin

214
Q

What chemo agent causes SIADH

A

Vincristine

215
Q

Symptoms/signs for neuroblastoma

A
  • VIP: diarrhea
  • Headache
  • Flushing, sweating
  • HTN
  • Periorbital ecchymoses
  • Opsoclonus myoclonus
  • Abdo mass
  • Blueberry muffin rash
216
Q

When can child with mono return to play?

217
Q

HUS bugs (x4)

A
  • E. coli 0157:H7
  • Shigella
  • S. pneumo
  • HIV
218
Q

What do you expect for (a) HbAC and (b) HbCC in terms of complications?

A

(a) asymptomatic

(b) mild anemia, splenomegaly, cholithiasis

219
Q

Infratentorial structures

A
  • Medulla
  • Cerebellum
  • Pons
  • Brainstem
220
Q

Symptoms/signs of infratentorial tumor

A
  • Ataxia
  • CN dysfunction
  • Diplopia
  • Papilledema
  • Nystagmus
  • Swallowing dysfunction
  • N/V
  • Headache
221
Q

x2 confirmatory tests with spherocytes on smear

A
  • Genetics

- Osmotic fragility test

222
Q

7 yo F with pallor and decreased energy. Viral illness 1-2 weeks ago. CBC: Normal WBC/plts, retics 24%, Hb 40. Scleral icterus on exam. What is dx, next test, and tx?

A
  • Autoimmune hemolytic anemia
  • DAT
  • Steroids
223
Q

What bone tumor to think about for a teenager who is active?

A

osteosarcoma

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