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Studying > Heme Onc > Flashcards

Heme Onc Flashcards

1
Q

Microcytic anemia

MCV<80

A
  • iron deficiency
  • thalassemia
  • anemia of chronic disease
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2
Q

Normocytic anemia

MCV 80-100

A 
retics high (>2%): hemorrhagic, hemolytic anemias
retics <2%: leukemias, aplastic anemia, red cell aplasia, bone marrow failure syndromes
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3
Q

Macrocytic anemia

MCV>100

A

if megaloblasts and segmented neutrophils = megaloblastic: vitamin B12 def, folate deficiency, drug-induced
Non-megaloblastic: alcohol abuse, myelodysplastic, liver disease, congenital bone marrow failure syndromes

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4
Q

Mentzer index

A

MCV/RBC
>13 = iron deficiency
< 13 = thalassemia

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5
Q

Labs for hemolysis

A

increased retics, LDH, unconjugated bili,

decreased haptoglobin

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6
Q

Warm AIHA

vs. cold

A 
Warm = IgG
Cold = IgM
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7
Q

Pure red cell aplasia

  • definition
  • causes
A

= isolated anemia with very low reticulocytes

  • post-viral: parvo B19,
  • transient erythroblastopenia of childhood (TEC) - recovery in 1-2 months
  • congenital pure red cell aplasia = Diamond Blackfan Anemia
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8
Q

Normal hemoglobins on electrophoresis

A

Hgb A: 95-98%
Hgb A2: 2-3%
Hgb F: 0.8-2%

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9
Q

HPLC for hemoglobin SS

A
  • mostly Hgb S
  • no hemoglobin A
  • increased HgF
  • increased Hg A2
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10
Q

Beta thalassemia major

diagnosis

A

hemoglobin analysis (No A)
- all A2 and F)
Features: skeletal findings, face, hepatosplenomegaly, para-aortic masses, Asian, African, Mediterranean

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11
Q

Complications of sickle cell

A

Infants: sepsis, dactylitis, sequestration

Older kids: sepsis, pain crisis, chest crisis, stroke, priapism

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12
Q

Management sickle cell disease and fever

A
  • admit
  • blood culture and then antibiotics e.g. CTX
  • hydration (2x maintenance)
  • incentive spirometry, O2 as needed
  • simple transfusion only if indicated
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13
Q

Acute chest syndrome in sickle cell

  • definition
  • management
A

definition: fever, resp sx, new infiltrate on CXR
Mgmt:
- IV CTX, oral macrolide, supplemental O2
- simple transfusion if hemoglobin is >10 below baseline
- urgent exchange transfusion If rapid progression of ACS , decline in Hgb despite simple transfusion, progressing respiraotyr symptoms

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14
Q

Sickle cell disease surveillance

A
  • pen prophylaxis to 5 yo
  • vaccination (asplenic)
  • folic acid
  • transcranial dopplers (monitor for stroke risk)
  • echo, PFTs, sleep studies
  • silent strokes and neurocognitive issues
  • pulmoanry HTN
  • gallstones
  • sleep apnea
  • iron overload
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15
Q

Hydroxyurea in sickle cell disease

A
  • decrease frequency of pain episodes/acute complication
  • prevents life-threatening neurologic events in those at risk of stroke

watch for myelosuppression/ neutropenia

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16
Q

Hereditary spherocytosis

diagnosis

A

EMA testing by flow cytometry (osmotic fragility)

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17
Q

Complications of HS (3)

A
  • hemolytic anemia
  • aplastic crisis e.g. parvo
  • increased gall stones
    Tx: symptomatic transfusion, folate for some pts, splenectomy for some
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18
Q

General indications for a splenectomy (4+)

A
  • traumatic splenic rupture
  • congenital hemolytic anemia if require ongoing transfusions
  • sickle cell anemia with recurrent splenic sequestration
  • severe, symptomatic chronic ITP with failure to respond to medical management (last resort)
  • more rare: splenic vein thrombosis, echinocccal cyst, splenic abscess, select leukemias, lymphomas, myeloproliferative d/os (very rare)
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19
Q

ITP red flags (7)

A
  • age < 12 months
  • family history of “ITP”
  • congenital anomalies
  • consanguinity
  • constitutional symptoms
  • significant lympahdenopathy+/- hepatosplenomegaly
  • lack of response to first line tx
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20
Q

When to treat ITP

A
  • if bleeding/high risk
    1st line: IVIG, steroids, Anti-D
    IVIG = response in 24-48hr (steroids 48-72hr), no worry about masking leukemia
    inpts vs. outpatient
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21
Q

NAIT

- management

A
  • transfuse if bleeding and/or platelets < 30
  • random platelets = fastest
  • then HPA-1 negative/matched platelets or washed mat platelets
  • IVIG = increased response to transfusion, decreased duration of thrombocytopenia
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22
Q

Neonatal autoimmune thrombocytopenia

management

A
  • generally less severe

- treatment: IVIG, transfusion if bleeding

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23
Q

Transfusing neonates platelets

A
  • stable pts, plts < 20
  • unstable, plts <30
  • infant bleeding or invasive procedure, plts < 50
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24
Q

Red flags in neutropenia

A
  • infections
  • growth issues/ FTT
  • mouth ulcers esp if cyclic
  • other congenital anomalies
  • consanguinity
  • family hx of neutropenia/MDS/cancer
  • other cytopenia
  • no neutrophil response in time of fever/infection
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25
Q

First level investigations for neutropenia

A
  • liver and renal function
  • electrolytes
  • CRP
  • DAT
  • immunoglobulins
  • ANA
  • Hgb analysis
    (rpt CBC monthly to see if persists)
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26
Q

Aplastic anemia definition

A
  • pancytopenia with a hypocellular bone marrow in absence of abN infiltrate or marrow fibrosis
    at least 2 of the following: hgb<100, plt<50, ANC <1.5
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27
Q

Fanconi anemia

  • screening test
  • features
A
  • chromosome fragility test

Features: congenital abN, short stature, digits, facies, cardiac/renal etc. cancer risk

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28
Q

Von Willebrand disease

  • genetics
  • screening test
A
  • mostly AD
  • screen: VWAg, Ristocetin cofactor (VWF activity), factor VIII

platelets should be normal in VWD

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29
Q

VWD types and treatment

A

Type 1 = most common, mild quantitative defect often treat with DDAVP

Type 2= rare d/o of VWF function; DDAVP with caution (sometimes make it worse)

Type 3 = severe, AR, very little/no VWF
- needs VWF replacement

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30
Q

Coag cascade

- Intrinsic pathway

A

Intrinsic pathway affects PTT

- 12, 11, 9, 8

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31
Q

Coag cascade

  • extrinsic pathway
  • what does it affect?
A

Extrinsic pathway affects PT/INR

- 7

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32
Q

Coag cascade

- common pathway

A

Affects PTT and PT/INR
10, 5, 2 (prothrombin), 1 (fibrinogen)
- think DIC, vitamin K deficiency

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33
Q

Hemophilia

  • genetics
  • factors
A

X-linked
Hemophilia A: Vactor VIII
Hemophilia B: Factor IX
Treatment: factor replacement e.g. prophylaxis or on demand bleeds

sx: circ bleeding, delayed surgical bleeds, muscle bleeds, jt bleeds

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34
Q

Classic hemorrhagic disease of newborn

A

from 24hrs to week

- up to 2% of infants who have not received Vit K prophylaxis

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35
Q

Diagnosis of DIC

A

Coagulopathy = increased INR, PT, PTT

  • hypofibrinogen
  • thrombocytopenia
  • elevation in D-dimers/ fibrinogen degradation products
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36
Q

Transfusions

  • frozen plasma
  • cryoprecipitate
A

FP: for emergency reversal of warfarin, for active bleeding or surgery and coags >1.5x normal
Cryopreciptiate (factor 8, fibrinogen, VWF)
- for bleeding in pt with fibrinogen <1
- for bleeding with VWD or hemophilia ONLY if factor or DDAVP unavailable

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37
Q

pulmonary embolism investigations

A

CT angio is preferable to V/Q scan

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38
Q

Red flags for malignancy in lymphadenopathy

A
  • firm, fixed, matted, non-mobile
  • non-tender
  • rapidly growing
  • persistent > 6 weeks
  • presence of constitutional sx or pruritis
  • size > 2cm
  • supraclavicular
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39
Q

osteosarcoma vs. Ewing

A

Osteo: metaphyses of long bones, sclerotic destruction, sunburst pattern, mets to lungs and bones
ES: diaphyses of long bones, flat bones, lytic, periosteal reaction, onion-skinning, mets to lungs and bones

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40
Q

Infratentorial tumors

- triad of sx

A

headache, nausea + emesis, papilledema

Other sx: disorders of equilibrium, gait, coordination, torticollis, blurred vision, diplopia, nystagmus

41
Q

Supratentorial tumors

- presentation

A

Lateralized defects e.g. focal motor weakness, focal sensory changes, language disorders, focal seizures, reflex asymmetry, language disorders,
premature hand preference

42
Q

3 acute chemo side effects

A
  1. myelosuppression
  2. nausea/vomiting/mucositis
  3. alopecia
43
Q

Acute and late effects:

- asparaginase

A

Asparaginase: acute: pancreatitis, anaphylaxis, coagulopathy, hyperglycemia;

44
Q

Acute and late effects:

- cisplatin

A

Acute: nausea, low Mg, renal, tinnitus,
Late: SNHL

45
Q

Acute and late effects

- cytarabine

A

acute: fever, rashes, conjunctivities, nausea

NO late

46
Q

Acute and late effects

- cyclophosphamide

A

Acute: hemorrhagic cystitis, SIADH, nausea
Late: infertility, secondary malignancies

47
Q

Acute and late effects

- doxorubicin

A

Acute: red secretions, acute arrhtyhmia/heart failure, mucositis

Late: decreased cardiac function/heart failure

48
Q

Acute and late effects

- etoposide

A

Acute: hypotension, allergy/anaphylaxis

Late: 2nd malignancy

49
Q

Acute and late effects

- vincristine

A

Acute: constipation, foot drop, sensory neuropathy, vocal cord dsfxn, ptosis, jaw pain, SIADH
Late: incomplete recovery from severe neuropathy

50
Q

Acute and late effects

- radiation therapy

A

Acute: skin redness, irritation, burn, mucositis, N+V, myelosuppression, tiredness, alopecia

Late: 2nd malignancies, pit dysfunction, low thryoid, pulmonary fibrosis, metabolic syndrome, poor growth, infertility, learning difficulties

51
Q

Bleomycin and busulfan late side effect

A

pulmonary fibrosis

52
Q

Cancer predisosition:

  • Beckwith wiedemann
  • NF-1
A

BW: Wilms tumor, hepatoblastoma, neuroblastoma

NF1: optic glioma, GIST, JMML, MPNST, pheo,

53
Q

Wilms tumor

- syndromes predisposed

A
  • WAGR
  • Denys-Drash
  • Beckwith-Wiedemann
  • Sotos
  • NF 1
    Other: Simpson-Golabi-Behmel, Perlman
54
Q

Leukemia

- syndromes predisposed

A
  • T21, NF-1, Noonan, Li-fraumeni, Bloom syndrome
  • Bone marrow failure syndromes
  • Immunodeficiencies: WAS, X-linked agammaglobulinemia
55
Q

CNS tumors

- syndromes predisposed

A

NF1, NF2, Tuberous sclerosis, von hippel-Lindau, Gorlin and turcot syndromes

56
Q

G6PD blood smear

A
  • RBC inclusions
  • Heinz bodies
  • bite taken from RBCs
  • polychromasia representing reticulocytosis
57
Q

Hemophilia lab findings

A
  • PT is normal

- PTT is abnormal 2-3x ULN if severe

58
Q

Iron deficiency anemia

treatment

A

3-6 mg/kg/day elemental iron

- limiting milk intake

59
Q

Response to iron therapy

  • 12-24hrs
  • 48-72hrs
  • 4-30 days
  • 1-3 months
A

12-24hrs: clinical sx improve
48-72hrs: reticulocytosis peaking at 5-7 days
4-30 days: increase in hemoglobin level
1-3 months: repletion of stores

60
Q

Indications for IRRADIATED RBCs

A 
- reduces risk of post-transfusion GVHD
use it in: 
- intrauterine or exchange transfusion, 
- SCID and DiGeorge requiring heart surgery
- HSCT 
controversial: 
- prem infants
- T-cell antibody therapy
- organ allografts
- immunosuppressive drugs
-HIV
61
Q

Fanconi anemia

  • defining features
  • diagnosis
A
  • thumb, radius abnormalities, CALMs, squamous cell cancer, short stature
  • chromosomal breakage test and gene sequencing
62
Q

Schwackman diamond

  • defining features
  • diagnosis
A
  • pancreatic insufficiency, short stature, abnormal thorax

- gene testing, evidence of pancreatic insufficiency, imaging showing fatty infilatration of pancreas

63
Q

Dyskeratosis congenita

  • defining features
  • diagnosis
A
  • nail findings, mucosal findings, hyperpigmented skin, squamous cell cancer
  • quantitative analysis of telomere length (flow FISH), gene sequencing
64
Q

Diamond Blackfan Anemia

A

Elevated adenosine deaminase levels! (differentiates it from TEC)

  • > 90% in first year of life
  • anemia - normochromic and macrocystic and reticulocytopenia
  • absent RBC precursors
  • Autosomal dominant
  • other anomalies: craniofacial, upper limb and hand abnomrlaities (thumb), GU, cardiac, ophtho, MSK, short stature is common
65
Q

High risk factors in ALL

A
  • Age < 1 or > 10 years
  • WBC > 50,000
  • Present CNS disease
  • DNA index <1.16
  • cytogenetics (t4:11), t(9;22)
  • slow response to therapy
66
Q

Auer rods

A

AML

67
Q

Reed-Sternberg cells

A

Hodgkin Lymphoma

68
Q

Lymph node biopsy to be considered if

A
  • persistent or unexplained fever
  • weight loss
  • night sweats
  • supraclavicular location
  • mediastinal mass
  • hard nodes
  • fixation to surrounding tissue
  • increase in size over baseline in 2 weeks, no decrease in size in 4-6 weeks and no regression to normal in 8-12 weeks or if new signs and sx develop
69
Q

Treatment neonatal alloimmune thrombocytopenia

A
  • transfusion of compatible platelets (maternal washed, HPA1a- negative)
  • IVIG

if known pre-natal maternal IVIG

70
Q

Treatment neonatal autoimmune thrombocytopenia

A
  • platelets if emergency
  • IVIG to infant (sometimes steroids to infant after delivery)
    If known, prenatal maternal steroids
71
Q

Neuroblastoma tumor markers

A

urine homovanillic acid and vanillylmandelic acid
(catecholamine metabolites)
- elevated in 95%

72
Q

Neuroblastoma

N-Myc amplifcation

A

N-Myc amplification is bad

73
Q

Neuroblastoma stage 4s features

A

infants < 12 months with localized disease (aka can have skin, liver and small amount of BM involvement)

  • spontaneously regresses in most cases
  • chemo if unfavourable histology or sx from mass effect
74
Q

Opsoclonus myoclonus (ataxia) syndrome

A
  • rapid, dancing eyes
  • myoclonus and ataxia (dancing feet)
    DDX: acute cerebellar ataxia, neuroblastoma, para-infectious
75
Q

Tumour lysis syndrome lab findings

A
  • hyperkalemia
  • hyperphosphatemia
  • secondary hypocalcemia
  • hyperuricemia
  • AKI
76
Q

Risk factors for TLS

A
  • first 72 hrs after chemo/rads
  • larger tumor burden, masses and higher WBC (blast counts)
  • tumor type (Burkitt, TALL, AML, other NHL)
  • pts with renal or cardiac dysfunction
77
Q

Prevention of TLS

A
  • IV hydration e.g. 2x maintenance
  • use of hypouricemic agents e.g. allopurinol and rasburicase (not for G6PD)
  • manage and monitor electrolyte disturbances
78
Q

Presentation of pheochromocytoma

A
  • paroxysmal hypertension (attacks eventually give way to continuous HTN), HA palpitations, AP, dizziness, V, sweating
  • hypermetabolic so become cachectic
79
Q

Preop management pheochromocytoma

A
  1. alpha blockage
  2. beta blockage
    - fluid loading
80
Q

Reversal agent for:

  • warfarin
  • heparin
A
  • Warfarin: vitamin K plus either prothrombin complex concentrate or FFP
  • Heparin: protamine
81
Q

Treatment for polycythermia/ hyperviscosity

A
  • partial exchange transfusion with normal saline

- consider if Hct > 70-75% or lower if signs of hyperviscosity are present

82
Q

Transient myeloproliferative disorder

A
  • high leukocyte counts, blast cells, associated anemia, thrombocytopenia, hepatosplenomegaly
  • usually resolve within first 3 months of life
  • require follow up because 20-30% will develop typical leukemia by 3 yrs
83
Q

Sickle cell infectious organisms

A
  • strep pneumonia
  • haemophilus influenzae
  • neisseria meningitidis
  • salmonella
84
Q

Hydroxyurea benefits

A
  • decrease painful episodes, dactylitis
  • decrease rate of acute chest syndrome
  • decrease blood transfusions
  • some evidence for prevention of recurrent priapism
85
Q

Management sickle cell acute focal neuro deficit

A
  • O2 to keep sats >96%
  • blood transfusion within 1hr of presentation
  • consider exchange transfusion (associated with decreased risk of second stroke vs. simple transfusion)
  • neuroimaging
86
Q

Prevention of stroke in Sickle Cell

A
  • chronic blood transfusion therapy to keep maximum HbS concentration <30%
87
Q

Sickle cell acute chest syndrome features

A

New density on CXR PLUS 2+ of:

  • fever
  • resp distress
  • cough
  • chest pain
88
Q

Management acute chest syndrome in sickle

A
  • 3rd gen cephalosporin and macrolide
  • +/- asthma management
  • oxygen
  • pain control
  • blood transfusion = only method to abort ACS; given if 1 of: decreasing O2 sat, increasing work of breathing, rapid change in resp effort, drop in Hgb 2g/dL below baseline, hx of ACS requiring ICU admission
89
Q

Splenic sequestration

features and tx

A
  • most often 6mo-2 yrs,
  • rapid spleen enlargement and a decline in Hgb by 2g/dL from baseline
    Tx: blood transfusion (generally 5mL/kg because spleen may release blood)
    prevention = splenectomy
90
Q

Hereditary spherocytosis

genetics/ epi

A 
Autosomal dominant (mostly)
Northern European
91
Q

Hereditary spherocytosis

Diagnosis

A
  • flow cytometry EMA binding test
  • can be diagnosed with positive family history, and clinical/lab features e.g. splenomegaly, spherocytes, reticulocytosis, elevated mean corpuscular hemoglobin concentration
92
Q

Hereditary spherocytosis indications for splenectomy

A
  • Pts with severe HS (e.g. Hgb <60-80, reticulocytes>10%, regular transfusions)
  • pts with moderate HS with frequent hypoplastic or aplastic crises, poor growth, cardiomegaly
93
Q

Beta-thal major

  • presentation
  • treatment
A
  • homozygous Beta gene defect
  • progressive hemolytic anemia with profound weakness and cardiac decompensation
  • Tx with transfusions for anemia but also growth failure, bone deformities, hepatosplenomegaly
94
Q

Beta-thal major on Hb analysis

A
  • increased HbF and HbA2,
95
Q

TEC features

A
  • children between 6 mo and 3 yrs (most >12mo)
  • reticulocytopenia and mod-severe normocytic anemia (+/- mild neutropenia)
  • NORMAL RBC adenosine deaminase levels
    recover within 1-2 months
96
Q

Monitoring anticoagulants

  • LMWH
  • warfarin
  • UFH
  • rTPA
A
  • LMWH: anti-Xa level
  • warfarin: INR
  • UFH: PTT
  • rTPA: fibrinogen/D-dimer
97
Q

Absolute contraindications to tPA (thrombolytic therapy)

A
  • major surgery within 7 days
  • hx of significant bleeding - intracranial, pulmonary, GI
  • peripartum asphyxia with brain damage
  • uncontrolled HTN
  • severe thrombocytopenia
    if serious bleeding occurs, stop thrombolysis, start cryoprecipitate to replace fibrinogen
98
Q

What is in cryoprecipitate?

A
  • fibrinogen
  • factor VIII
  • Factor XIII
  • von willebrand factor

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