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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
Difference between allopurinol and rasburicase
- Allopurinol prevents creation of uric acid | - Rasburicase makes uric acid more soluble (better agent if already making uric acid)
26
When to consider BMA for ITP
- features to suggest malignancy or BM failure (e.g., bicytopenia, LAD) - insufficient or no response to steroids and/or IVIG
27
3 buckets of symptoms for leukemia
- lack of normal BM output - infiltration by blasts - sanctuary site
28
x2 sanctuary site for leukemia
CNS and testicular
29
7 syndromes that predispose you to leukemia
- T21 - Neurofibromatosis type 1 - Noonan Syndrome - Bloom Syndrome - Ataxia telangectasia - Fanconi anemia - Li Fraumeni Syndrome
30
T21 specific malignancy concern + presentation
Transient myeloproliferative disorder - High WBC - Blasts - Pancytopenia
31
Non-marrow site for leukemia
CNS, testes, HSM, bone, skin, LN, mediastinum
32
What type of leukemia presents with DIC?
APML (acute promyelocytic leukemia)
33
To make diagnosis of leukemia you need….
>25% blasts in the bone marrow
34
Most common type of ALL
B cell (80%)
35
What makes a T cell ALL more likely (x3)?
Boys Large mediastinal mass High WBC count
36
Pathopneumonic sign for AML on smear
Auer rods
37
High risk features for B cell ALL (x5)
``` 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 ```
38
Induction chemo for ALL
Vincristine Steroids Peg-asparaginase
39
Methotrexate side effects (x3 system side effects)
- liver dysfunction - renal dysfunction - neurotoxicity
40
Cytarabine side effects
- flu like symptoms - neurotoxicity - corneal toxicity
41
Prophylaxis given in immunocompromised host (x2)
- PJP = Sentra | - fungal = fluconazole and caspofungin
42
What lymphoma is most common?
Non-Hodgkin lymphoma (60%)
43
Complications of mediastinal mass
- Airway compromise | - SVC syndrome
44
Late effects of Hodgkin lymphoma
- hypothyroidism - infertility - cardiomyopathy - pulmonary fibrosis - solid tumor (breast, thyroid, skin) - MDS/leukemia - avascular necrosis
45
Risk factors for non-hodgkin lymphoma
-immunodeficiency: WAS, ataxia telangiectasia, congenital hypogammaglobinemia, post-transplant, HIV
46
3 types of lymphoma
- Lymphoblastic lymphoma - Burkitts lymphoma - anaplastic large cell lymphoma or diffuse large B-cell lymphoma
47
What virus is related to Burkitt lymphoma?
EBV
48
What lymphoma should be on your ddx for an abdominal mass?
Burkitt lymphoma
49
Risk factors for TLS
- tumor type: burkitts, leukemia - timing: increased risk during first 72 hours of treatment - renal/cardio dysfunction: may not tolerate therapy
50
Where is the brain tumor if: A) head tilt B) behavioural change
A) posterior fossa | B) supratentorial
51
Types of tumours in posterior fossa
- pilocytic astrocytoma - medulloblastoma - ependymoma
52
Localizing signs for supratentorial
- hemiparesis - hemisensory loss - hyperreflexia - seizures - visual changes - behavioural - diencephalic syndrome of infants
53
Triad for infratentorial tumor
- long tract signs - ataxia - cranial neuropathies
54
Side effects of brain radiation
``` Cerebral edema Moya moya Skin toxicity Radiation necrosis Endo dysfunction Cognitive impairment Secondary brain tumor Somnolence syndrome ```
55
Two most common sympathetic chain locations for neuroblastoma
Adrenal glands | Paraspinal nerves
56
What scan is specific for neuroblastoma?
- MIBG scan
57
What age group has the best prognosis for neuroblastoma?
<18 months old
58
Genetic predispositions for wilm’s tumor
- WAGR - Beckwith Wiedemann - Denys-Drash
59
Most common renal tumor
Wilm’s
60
Most common liver tumor
Hepatoblastoma
61
Most helpful lab for hepatoblastoma
AFP
62
Common place for mets with sarcomas
Lungs
63
Most common soft tissue sarcoma
Rhabdomyosarcoma
64
Two most common bone sarcoma
Ewing and osteosarcoma
65
What bone sarcoma is typically in the center location in the transverse plane?
Ewing sarcoma
66
Classic XR findings for bone sarcoma
- osteosarcoma - sunburst | - ewing sarcoma - onion skin
67
How is retinoblastoma inherited?
Autosomal dominant (30% of cases)
68
Diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH)
5/8: - fever - cytopenias - splenomegaly - high ferritin - hypertriglyceridemia - hypofibrinogenemia - absent NK activity - increased soluble IL-2 receptor
69
CNS tumours - genetic predisposition
- NF1 and 2 | - tuberous sclerosis
70
Genetic predisposition with neuroblastoma
- PHOX2B mutation | - beckwith-wiedemann syndrome
71
Late effects of radiation
- secondary malignancy - pituitary dysfunction - hypothyroidism - pulmonary fibrosis - metabolic syndrome/stroke - poor growth - infertility - learning/psychosocial difficulties
72
Acute toxicity of radiation
- skin irritation - n/v - myelosuppression - mucositis - fatigue - esophagitis/dysphasia - alopecia
73
BMT complications
- 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)
74
Most common trigger for ITP and treatment options
Infection (~50% of cases) - Observation - Low plt precautions - IVIG - Oral CC's - Medication-refractory: splenectomy, rituximab
75
Further disorders to r/o if picture is no longer looking like ITP
- SLE and collagen vascular disease - Common variable immune deficiency - Myelodysplastic syndrome or BM failure - Viral infection
76
What cancer preventing vaccines are there?
- HPV | - Hep B (hepatocellular carcinoma)
77
Beta thalasemia - genotypes + clinical features
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
78
DVT risk factors
Immobilization, surgery, trauma, line/catheter, OCP, pregnancy, smoking, obesity, malignancy
79
Autoimmune hemolytic anemia - differences between warm and cold erythrocyte Ab
``` 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 ```
80
Hb barts
Tetramer of delta-globin chains
81
x2 ways to differentiate IDA vs thalasemia on CBC
Metzner index (>12 in IDA) + RDW (high in IDA)
82
What is the cause for hemolytic anemia in a patient with acute anemia, fatigue, jaundice, and dark urine following early spring swim?
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
83
Clinical presentation of Fanconi Anemia (system based)
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
84
Age of BM failure in Fanconi Anemia
5 years
85
Causes of acquired BM failure
ID: EBV, parvo, HIV Meds: AEDs, chemo, radiation Onc: Pre-leukemia Heme: Paroxysmal nocturnal hemoglobinuria Imm: Eosinophilic fasciitis, hypogammaglobinemia
86
x2 associated neurological disorders to iron deficiency anemia
ADHD and RLS
87
Risk factors and at risk populations for iron deficiency anemia
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
88
Most common cause of transient neutropenia + pathophysiology
Viral infection = influenza, adeno, CMV, EBV, VZV, measles, hep A+B, coxsackie - Patho: Marrow suppression, neut redistribution, sequestration in reticuloendothelial tissue
89
Most common cause of chronic childhood neutropenia
Autoimmune neonatal neutropenia (ANI) = resolves by 4 y/o, normal BM cellularity/arrest of late metamyelocytes, anti-neutrophil Ab's detected (mostly)
90
Classic triad for Wiskott-Aldrich Syndrome
Micro-thrombocytopenia, eczema, and immunodeficiency (usually combined type)
91
Etiology for thrombocytosis
- Reactive: anemia/hemolysis/blood loss, infection, inflammation, post-splenectomy - Primary: heme malignancies (polycythemia vera, CML, essential thrombocythemia), familial thrombocytosis
92
Clinical signs/symptoms for hemolytic anemia
Tachycardia, pallor, jaundice, organomegaly, discoloured urine (dark, brown, red) -Severe = hypovolemic shock or CHF
93
x2 findings on blood smear you could see with hemolytic anemia
Schistocytes, spherocytes
94
Complications of iron overload
- CVS: CHF, dysrhythmia, pericarditis - Liver: cirrhosis, fibrosis - Endo: growth/sexual development delays, hypoparathyroidism, hypothyroidism
95
Iron overload management
- Chelation therapy - Diet: drink tea - Splenectomy - Erythrocytapheresis/RBC exchange
96
Causes of prolonged INR
- Vitamin K deficiency - Warfarin - Liver disease - Factor VII deficiency
97
Causes of prolonged PTT
- Heparin - Hemophilia - vWD - Antiphospholipid antibodies
98
Causes of combined prolonged PT/PTT
- Heparin - Warfarin - Liver disease - DIC
99
Vitamin K dependent coagulation factors
II, VII, IX, X, protein C + S
100
Factors in common coagulation pathway
V, X, I, II
101
Common hereditary disorders that predispose to thrombosis (x6)
Factor V Leiden, protein C deficiency, protein S deficiency, antithrombin III deficiency, hyperhomocysteinemia, antiphospholipid Abs
102
Timing of anemia nadir for infants
- Term = 8-12 weeks | - Preterm = 6-8 weeks
103
x2 pathophysiologic mechanisms in sickle cell anemia
- Hemolysis: intra + extravascular | - Vaso-occlusion: acute exacerbations (pain crisis, stroke), chronic disease exacerbations (retinopathy, renal disease)
104
Most common cause of death in sickle cell
Infection - primarily pneumococcal with functional asplenia
105
Transfusion dosing for pRBC's, platelets, and FFP?
- pRBC's + FFP = 10-15 mL/kg | - platelets = 10 mL/kg
106
Coombs test - direct vs indirect
- 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
Cryoprecipitate - what is it and indications for use?
=plasma product of concentrated factors (VIII, XIII, vWF, fibrinogen) from FFP -Given when specific factors are NOT available, to reverse anticoagulation, or DIC
108
Howell-Jolly bodies - what are they? what conditions are they seen in?
= nuclear remnants of normal RBC maturation, usually removed by spleen -Found in patients with decreased/absent splenic function
109
Menorrhagia - what bleeding d/o to screen for?
vWD
110
vWD - what factor is impacted? what coag is affected?
PTT, decreased fVIII antigen + activity
111
What is von Willibrand factor?
- 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
alpha-thal clinical features + genotypes
``` 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
Typical age range for ITP
2-5 years
114
Three main options for management in ITP
- Observation - CC's - IVIG
115
Progression of labs in iron deficiency anemia
1. Ferritin 2. Transferrin saturation 3. MCV + Hb
116
What x3 bleeding disorders are on the ddx with prolonged PTT and normal INR?
- Hemophilia A and B - VWD - factor XI deficiency
117
TEC + Diamond Blackfan Anemia - similarities
- Disorder of RBC production during early childhood | - Low Hb and inappropriately low retics
118
TEC + Diamond Blackfan Anemia - differences
- 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
Presentation of hepatic sinusoidal obstructive syndrome - symptoms/signs, when typically presents, x1 complication
- Hepatomegaly, RUQ pain - Jaundice - Ascites/weight gain - Within first 3 weeks of BMT - Hepatorenal syndrome
120
x3 causes of pure red cell aplasia and what does this term mean?
=isolated anemia with VERY low retics - Aplastic anemia (from parvo) - Diamond Blackfan Anemia - TEC
121
Types of intrinsic hemolytic disorders
- Membranopathy: spherocytosis, elliptocytosis - Enzymopathy: G6PD deficiency, PK deficiency - Hemoglobinopathy: HbSS, B-thal
122
Types of extrinsic hemolytic disorders
- Autoimmune: Warm (IgG), cold (IgM), paroxysmal nocturnal hemoglobinuria - Hemolytic disease of the newborn - Non-immune HA: HUS, TTP, DIC
123
DDx for macrocytic anemia
- Megaloblastic: B12 or folate deficiency | - Non-megaloblastic: BM failure, hypothyroidism, T21, chronic liver disease, drugs
124
Iron supplementation
PO iron 3-6 mg/kg/day
125
How quickly will you notice a response from iron supplementation?
- Subjective improvement - Reticulocytosis within 3-7 days - Increased Hb 7-30 days - Repletion of iron stores by 3 months
126
What should you take to improve iron supplementation absorption?
-Orange juice (vitamin C)
127
What lab could you add on to iron studies in setting of potential inflammation?
Add CRP to ferritin
128
4 mechanisms of iron defiency
- Inadequate stores at birth - Insufficient iron in diet - Blood loss - Malabsorption
129
What is the leading cause death in SCD?
ACS
130
What is a side effect to monitor for with hydroxyurea?
Myelosuppression | Neutropenia
131
Classic facial findings in Beta Thalassemia Major
- Chipmunk facies - Frontal bossing - Maxillary expansion - Saddle nose - Depressed cranial vault
132
What would Hb analysis show in Beta Thalassemia Major?
- No HbA | - All HbA2 and HbF
133
Two main problems (+their associated disorder examples) in hemoglobinopathies?
- Problem with Hb quantities = thalassemia | - Problem with Hb quality = SCD
134
What is now used today instead of hemoglobin electrophoresis?
High performance liquid chromatography (HPLC)
135
What is the name of the hemoglobin that is gamma-4 (Y4) + what does it represent?
Hemoglobin barts | - suggests alpha thalassemia
136
What is the name of the hemoglobin that is beta-4 (B4) + what does it represent?
Hemoglobin H | - suggests alpha thalassemia
137
What is HbF made up of?
- 2 alpha chains | - 2 gamma chains
138
What is HbA2 made up of?
- 2 alpha chains | - 2 delta chains
139
What does (a) a little bit of HbA2 vs (b) a lot of HbA2 suggest?
(a) normal | (b) beta thal
140
Typical clinical presentation of Hereditary Spherocytosis?
- Mild-moderate anemia - Jaundice - Occasional hemolytic episodes - Neonatal jaundice
141
How to diagnose hereditary spherocytosis?
EMA testing by flow cytometry - osmotic fragility
142
Complications of hereditary spherocytosis (x3)?
- Hemolytic anemia (with intercurrent illness) - Aplastic crisis (with parvo especially) - Gallstones
143
x3 potential management strategies for hereditary spherocytosis?
- Transfusion for symptomatic anemia - Folate (for patients with high retics and high MCV) - Splenectomy
144
x4 common triggers for G6PD
- Moth balls - Fava beans - Sulfa drugs - Anti-malarials - Anti-pyretics
145
x4 indications for splenectomy in childhood
- 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
x2 main risks of splenectomy
- Infection (encapsulated bacteria) | - Thrombosis
147
Ddx for thrombocytopenia
- 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
Red flags for ITP
- <12 months of age - Family hx of "ITP" - Congenital anomalies - Consanguinity - Constitutional symptoms - Significant LAD and/or HSM - Unresponsive to therapy
149
When is ITP considered (a) persistent vs (b) chronic?
(a) 3-12 months | (b) >12 months
150
Essential/basic evaluation (hx, PEx, labs) in keeping with ITP?
- Hx: isolated bleeding sx - PEx: No HSM, no congenital abN, no LAD - Ix: Plts <100, anemia from bleeding, smear (normal to big plts)
151
First line treatment options for acute ITP (include options if severe bleeding)
- Steroids, IVIG, anti-RhD | - If severe bleeding = IV methylpred + IVIG + platelet transfusion +/- TXA
152
Steroids vs IVIG for ITP
- Steroids: slower (48-72h), could mask malignancy, can do as outpatient - IVIG: faster action (24-48h), requires inpatient
153
DDx for neonatal thrombocytopenia - based on (a) fetal/newborn, (b) <72 hours, (c) >72 hours, and (d) throughout neonatal period?
(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
Treatment for neonatal autoimmune thrombocytopenia?
- IVIG | - Transfusion if bleeding
155
If mother has an autoimmune condition, what kind of monitoring is recommended for baby?
Check platelets at birth and daily for a few days
156
Can NAIT occur during the first pregnancy?
Yes, 50%
157
What kind of Ab (immunoglobulin) of mother's passes to newborn via placenta?
IgG due to HPA mismatch
158
What is more severe and significant for newborns - NAIT vs neonatal autoimmune?
NAIT
159
x3 most common congenital thrombocytopenia syndromes?
- Wiskott-Aldrich Syndrome - TAR (thrombocytopenia absent radius) - CAMT (congenital amegakaryotic thrombocytopenia)
160
Tx for NAIT
- HPA/PLA-1 negative platelets - Maternal washed platelets - IVIG
161
What is the timeline for when neutropenia becomes chronic?
>3-6 months
162
DDx for acquired neutropenia
- 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
DDx for congenital neutropenia
- Severe congenital neutropenia - Cyclical neutropenia - Bone marrow failure syndromes - fanconi anemia, Schwachman-Diamond, Dyskeratosis congenita - Immunodeficiency: WAS, CD40 ligand deficiency, X-linked agammaglobulinemia
164
Red flag features for neutropenia
- 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
What treatment could be considered if neutropenia is persistent and severe?
GCS-F
166
DDx for pancytopenia
- Infections - Marrow infiltration: leukemia, lymphoma, neuroblastoma - Marrow aplasia: hypocellular, sick bone marrow - Megaloblastic anemia: severe B12/folate deficiency
167
Definition and criteria for aplastic anemia
= 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
What must you have to be pancytopenic?
At least 2 cell lines involved?
169
Etiology for aplastic anemia
- Acquired: viral suppression, drugs, toxins, idiopathic | - Congenital: Fanconi Anemia, Dsykeratosis congenita, Shwachman Diamond
170
What is the most common inherited bone marrow failure syndrome?
Fanconi Anemia
171
Important aspects to include in a newborn bleeding history for a child?
- Excessive bleeding post-circumcision, scalp/brain bleed, immunizations, after cord seperation - Vitamin K injection
172
Primary hemostasis vs secondary hemostasis symptoms
- 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
3 steps in hemostasis
- Vessel spasm - Primary = platelets adhere to injury site + aggregate to form plug - Secondary = formation of insoluble fibrin strands + coagulation
174
4 steps in primary hemostasis
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
Investigations for bleeding disorder
- CBC - Smear - PT/INR, fibrinogen - vWD studies - Blood group (for interpretation of vWD levels) - Factor VIII - Factor IX - LFTs - Renal function tests
176
What is the most common bleeding disorder?
Von Willebrand Disease
177
vWD subtypes
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
How does DDAVP work for vWD?
Induces release of von Willebrand antigen from cellular compartments
179
Management considerations for VWD (3 prong approach)
- Increase VWF = DDAVP, replacement of VWF concentrate - Inhibit fibrinolysis (TXA) - Hormonal therapy (for menorrhagia)
180
What factor deficiencies are (a) hemophilia A and (b) hemophilia B?
(a) fVIII deficiency | (b) fIX deficiency
181
What percentage of factor levels defines (a) mild, (b) moderate, and (c) severe hemophilia?
(a) >5% (b) 1-5% (c) <1%
182
Management for hemophilia
- 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
Early hemorrhagic disease of the newborn (timeline, RF's, bleeding sites, tx)
- 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
Classical hemorrhagic disease of the newborn (timeline, RF's, bleeding sites, tx)
- Within 2-7 days - RF's: none - Sites: ICH, GI, umbilical stump, bruising - Tx: vitamin K injection
185
Late hemorrhagic disease of the newborn (timeline, RF's, bleeding sites, tx)
- 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
What are triggers for DIC?
- Sepsis, severe infection - Vascular abnormality (Kasabach-Merritt Syndrome) - Trauma - Malignancy - Severe hemolysis - Liver disease - Severe protein C deficiency - Severe allergic or immunologic reactions
187
DIC - clinical presentation?
- Asymptomatic - Bleeding = bruising, petechiae, mucus membrane bleeding - Thrombosis = purpura fulminans, organ dysfunction - Hemolytic anemia
188
DIC - investigations + results
- Prolonged PT/INR - Decreased fibrinogen - Thrombocytopenia - Elevated D-dimer - Decreased protein C+S and anti-thrombin
189
Tx - DIC
- 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
Red flags of a lymph node for malignancy (x7)
- Firm, fixed, matted, non-mobile - Non-tender - >2cm - Supraclavicular - Rapidly growing - Presence of B symptoms - Persistence for >6 weeks
191
If concerning lymph node - what should you do?
-Refer to general surgery or ENT for EXCISIONAL biopsy
192
What is included in cryoprecipitate?
Factor VIII, fibrinogen, vWF
193
When to use cryoprecipitate?
- Bleeding in patient with fibrinogen <0.8-1.0 | - Bleeding with vWD or hemophilia ONLY if factor or DDAVP is unavailable
194
When to use FFP?
- If needing rapid reversal of warfarin | - For active bleeding or surgery or DIC if coags >1.5x normal
195
Platelet transfusion thresholds
- <10 - <20 = if coagulopathy - <50 = for surgery, epidurals, LPs - <100 = for neurosurgery, head trauma
196
What is the most common reason for a transient fever during a blood transfusion?
Sensitization to WBC antigens
197
Draw chemo man
Yep!
198
What GI complication is found in 50% of newborns with TAR?
CMPA - can make things difficult with ++bleeding from thrombocytopenia
199
Calculation for ANC
= WBC x %neutrophils (segmented+bands) x 10
200
What causes elevated AFP?
- Yolk sac tumors - Hepatocellular cancer - T21 - Ataxia telangiectasia - Hepatoblastoma
201
What organs are affected by leukocytosis?
- Brain - Lungs - Kidneys
202
What immediate steps do you need to take with a mediastinal mass?
- No sedation + do NOT lie flat - Biopsy --> once results back can then start steroids - Airway management - Start chemo
203
Complications of a mediastinal mass
- SVC syndrome - Pericarditis/pericardial effusion - Respiratory distress
204
Who is at highest risk for mediastinal mass?
- Male - Teenager - T-cell lymphoma - Burkitt lymphoma
205
How to treat hypoCa and hyperPO4 in TLS?
- Phosphate binders - Calcium supplement - Fluids
206
What will you find on smear for ITP?
Big baby platelets
207
What is the best initial test for pheo if (a) high suspicion vs (b) low suspicion?
(a) plasma metanephrines | (b) urine catecholamines
208
Most common endo abnormality in Langerhans?
Central DI
209
MEN2 A
- Medullary thyroid cancer - Pheo - Hyperparathyroidism
210
MEN2 B
- Medullary thyroid cancer - Pheo - Marfanoid features - Mucosal neuromas
211
Lynch syndrome = most common inherited colorectal cancer - Inheritance? - Amsterdam criteria? - When to start screening?
- Auto dominant - 3 family members, 2 generations involved, 1 family member <50 years old - Start colonoscopy at 20 years of age
212
Ataxia, nystagmus, and muscle jerks - dx?
Neuroblastoma
213
Example of an antracycline
Doxorubicin
214
What chemo agent causes SIADH
Vincristine
215
Symptoms/signs for neuroblastoma
- VIP: diarrhea - Headache - Flushing, sweating - HTN - Periorbital ecchymoses - Opsoclonus myoclonus - Abdo mass - Blueberry muffin rash
216
When can child with mono return to play?
3 weeks
217
HUS bugs (x4)
- E. coli 0157:H7 - Shigella - S. pneumo - HIV
218
What do you expect for (a) HbAC and (b) HbCC in terms of complications?
(a) asymptomatic | (b) mild anemia, splenomegaly, cholithiasis
219
Infratentorial structures
- Medulla - Cerebellum - Pons - Brainstem
220
Symptoms/signs of infratentorial tumor
- Ataxia - CN dysfunction - Diplopia - Papilledema - Nystagmus - Swallowing dysfunction - N/V - Headache
221
x2 confirmatory tests with spherocytes on smear
- Genetics | - Osmotic fragility test
222
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?
- Autoimmune hemolytic anemia - DAT - Steroids
223
What bone tumor to think about for a teenager who is active?
osteosarcoma

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