Hémato-onco Flashcards
Dans l’anémie falciforme, nommer les organes et dommages associés
- CNS
- AVC, ICT - Yeux
-hémorragie rétinienne
- rétinopathie - Coeur ; IC congestive
- Poumons
-EP
- HTP, cor pulmonare
- infarctus pulmonaire
- infection - Vasculaire ; phénomènes occlusifs
- Foie
- infactus
- hépatite
- séquestration hépatique
- cholestase intrahépatique - VB ; augmentation incidence calculs de bilirubine
- Rate ; séquestration aigüe, infarctus
- Urinaire
- hyposthénurie (dim de capacité à concentrer l’urine, perte H2O)
- hématurie
- IRC ad terminale - OGE ; dim fertilité, impotence, priapisme
- Osseux ; infarctus moelle, ostéomyélite, nécrose avasculaire
- Grossesse : insuffisance placentaire, mort foetale
13 : Immunodéficience relative
- Hémolyse chronique
12
Dans le syndrome aortique aigü (acute chest syndrome);
1) nommer les sx principaux
2) nommes les 2 bactéries généralements associées
3) énoncer le tx et pour les ATB, les doses
3)
1) SX PRINCIPAUX
fièvre, toux, hypoxie, toux, douleur thoracique, dyspnée, nouveaux infiltrats sur le RXP
2) ESPÈCES EN CAUSE
Mycoplasma sp
Chlamydia sp
3) TRAITEMENT ACUTE CHEST SYNDROME
Hydratation
Analgésie
Oxygénation adéquate et ventilation
ATB empiriques: cephalo + macrolide
- Ceftriaxone 1-2 g IV (50 mg/kg) die
- azithromycine 500 mg IV (10 mg/kg) die
Exchange blood transfusion si multi-lobes
Dans l’anémie falciforme;
a) quelle est la forme Hb?
b) pour un hétérozygote, quel est le % d’Hb anormale
c) pour un homozygote, quel est le % d’Hb anormale
d) contre quelle maladie protège cette forme d’Hb?
e) quelles sont les populations touchées?
f) quelles phénomènes physiologiques sont engendrés par l’anémie falciforme?g) Quels sont les facteurs précipitants d’une crise vaso-occlusive?
a) Mutation autosomale récessive a/n gène B-globine produisant l’HbS. Le GR est plus rigide, moins déformable, plus facile à lyser. Augmente la viscosité du sang.
b) Hb hétérozygote, 40% Hb anormale, maladie généralement bénigne/Asx
c) Hb homozygote HbSS, 85% Hb anormale, maladie sévère
d) Malaria
e) Afrique, Moyen-Orien, Méditérannée, Inde
f) hémolyse + crise vaso-occlusive
g) crises vaso-occlusives précipitées par une infection, l’exposition au froid, un stresseur (trauma)
Quel est le rôle de l’hydroxyurée dans le tx de l’anémie falciforme?
Induction d’Hb foetale et légère myélosupression. Réduit l’incidence des crises vaso-occlusives, l’atteinte organique et induit une meilleure longévité.
Doses 20-25 mg/kg/j PO au long cours
a) Nommer les critères DX de la POLYCYTHEMIA VERA / ERYTHROCYTOSE
b) Quel est le tx?
a) CRITÈRES MAJEURS
1 - Hb > 165 homme| > 160 femme OU Ht > 49% homme | > 48% femme
2 - prolifération a/n moëlle ou myélodysplasie
3 - JAK2 mutation
CRITÈRE MINEUR
Niveau érythropoïétine subnormal
b) TRAITEMENTS
phlébotomie pour obtenir un Ht < 45 %
ex : retirer 500 cc sang par 500 NS
en cas d’urgence, ad 1,5 L sur 24h
Hydroxyurée pour son effet myélosuppresseur
Nommer les causes de polycythémie vera / érythrocytose PRIMAIRE
Shunt droite-gauche
Maladie pulmonaire
Carboxyhémoglobinémie
Altitudes élevées
Hémoglobines à haute affinité
SAOS/SAHS
Maladie rénale
Tumeurs : hépatome, adrénergisme, méningiome, pheochromocytome, hémangioblastome
Discuter des types d’anémie selon le VGM, du DDX, du type de cellules trouvées sur le frottis
voir figure ci-dessous
- Which of the following statements best describes the use of the WBC count in emergency medicine decision making?
a. The absence of leukocytosis excludes the presence of significant disease.
b. The absolute neutrophil count, presence of bandemia, and immature granulocyte count may be more helpful than the total WBC count in identifying bacterial infection.
c. The total WBC count is both specific and sensitive for serious bacterial infection.
d. The WBC count is highly discerning in screening for illness in the acute care setting.
B. The WBC count and accompanying differential count are among the most common laboratory tests ordered in the ED. The WBC count has not proved to be a highly sensitive or specific test, and the
absence of leukocytosis does not exclude the presence of significant disease. The WBC count has limited screening value in the acute care setting. The absolute neutrophil count or an elevated band count may be more helpful than the total WBC count in identifying bacterial infection. The immature granulocyte count can be used to help identify
patients with or without sepsis.
- Which of the following can affect the “normal” WBC count?
a. Age and ethnicity
b. Exercise
c. Gender
d. Tobacco use
e. All of the above
Answer: E. All of the statements are true. One unique problem in WBC disorders is the wide variability in normal values and the multiple factors influencing them. Normal values for the WBC count are listed in Table 110.1. The “normal” WBC count range is age-dependent and may
be shifted upward by exercise, female gender, smoking, or pregnancy.
Decreases in the total WBC count range by 1000 to 1200 cells/mm3 have been noted in the Black population.
In evaluating a patient with severe neutropenia, which of the following
is false?
a. If the patient is febrile, basic isolation techniques and specific therapies should be initiated after cultures are completed.
b. If the patient is febrile, urine and blood cultures should be obtained.
c. The clinician should ask about medications, history of neutropenia,
family history, and also review information regarding recent infections.
d. The classic physical findings associated with infection will be obvious owing to the inflammatory response.
e. The review of systems focuses on bleeding problems, generalized
symptoms such as fatigue, sweats, and weight loss, or autoimmune symptoms.
Answer: D. The classic physical signs of infection may be blunted in severe neutropenia, because the inflammatory or purulent response may be hampered by the limited numbers of neutrophils.
A patient with a known history of chronic myeloid leukemia (CML) presents with shortness of breath. The WBC count is 25,000 cells/mm³.
Which of the following conditions is LEAST likely to be the cause of the patient’s dyspnea:
a. Angina
b. Heart failure
c. Hyperleukocytosis resulting in pulmonary ventilation-perfusion abnormalities
d. Renal failure and fluid overload
e. Severe anemia
Answer: C. Hyperleukocytosis can occur in CML, though the comparatively more mature, “less sticky” cells found in CML rarely cause problems unless the count exceeds 500,000 cells/mm3. A higher cell count may cause leukostasis and result in deafness, visual impairment, pulmonary
ventilation-perfusion abnormalities, or priapism. Treatment involves hydration, leukapheresis, transfusion as necessary, allopurinol to prevent severe hyperuricemia, and specific chemotherapy. The need for urgent therapy in CML is usually related to hyperuricemia and renal injury, or severe anemia and subsequent angina or heart failure.
When applied to lymphocytic neoplasms, the terms acute and chronic describe all of the following except:
a. Aggressiveness of therapy required in treatment
b. Cell maturity
c. Patient survival time
d. Rapidity of onset
Answer: C. Historically, acute and chronic were descriptive terms applied to lymphocytic neoplasms with respect to patient survival time. The terms acute and chronic are currently utilized to describe cell maturity, rapidity of onset, and aggressiveness of therapy required in
treatment. Chronic lymphocytic leukemia (CLL) is primarily a B-cell disorder and is the most common form of leukemia in patients 50 years or older. Acute lymphocytic leukemia (ALL) is most commonly diagnosed in children younger than 10 years and is the most frequent
malignant neoplasm in children younger than 15 years of age.
Anemia with an elevated mean corpuscular volume (MCV) is not
typically seen with which of the following conditions?
a. Folate deficiency
b. Hypothyroidism
c. Iron deficiency
d. Vitamin B12 deficiency
Answer: c. Iron deficiency anemia typically presents as a microcytic anemia. Liver disease may present with either a macrocytic anemia or a normocytic anemia reflecting an anemia caused by multiple mechanisms.
- An elevated mean corpuscular hemoglobin concentration (MCHC)
is expected in which of the following conditions?
a. Anemia of chronic disease
b. Iron-deficiency
anemia
c. Sideroblastic anemia
d. Spherocytosis
Answer: d.
The MCHC index is a measure of the concentration of hemoglobin. Low values represent hypochromia, whereas high values are noted only in patients with decreased cell membrane area relative
to volume, such as spherocytosis.
A 21-year-oldvman presents with easy fatigue and lack of energy. During a recent clinic visit, he was found to be anemic with a hemoglobin of 8 g/dL, mean corpuscular hemoglobin concentration (MCHC) of 25%, mean corpuscular volume (MCV) of 61 fL,
and a peripheral smear remarkable for target cells and basophilic stippling. Serum iron levels were normal. What is the most likely explanation for his anemia?
a. Iron deficiency
b. Porphyria
c. Sideroblastic anemia
d. Thalassemia
Answer: d. Hypochromic, microcytic anemias with normal serum iron
imply thalassemia (a defect in globin chain production). Both alpha-and
beta-thalassemia manifest as a hypochromic microcytic anemia
with target cells and basophilic stippling. The microcytosis is generally more severe than with iron-deficiency anemia. Sideroblastic anemia generally presents with elevated serum iron.
A 73-year-old woman presents with progressive fatigue. Her only past medical history is rheumatoid arthritis for which she takes methotrexate. She has no cardiopulmonary history and does not smoke. Physical examination is remarkable only for bilateral metacarpophalangeal, chronic swelling, and mild splenomegaly. Recent
blood tests from the clinic are remarkable for a high normal serum
iron, hemoglobin of 10 g/dL, and mean corpuscular volume (MCV) of 69 fL, with peripheral smear showing both microcytes and macrocytes. Which of the following is the most appropriate intervention?
a. A trial of pyridoxine
b. Hematology consultation for bone marrow biopsy
c. Hematology consultation for iron chelation
d. Send blood for vitamin B12 and folate levels
Answer: a. Sideroblastic anemia may be acquired or inherited. It is typically a refractory anemia in the elderly characterized by hypochromia and microcytosis but a dimorphic smear also showing normal cells and macrocytes. Some of these patients are pyridoxine deficient and may respond to a course of vitamin B6. This anemia is a defect in porphyrin synthesis and is associated with rheumatoid arthritis, cancer, and infections. Lead poisoning is a subset. Elevated iron and ferritin levels are seen because the porphyrin defect does not allow iron incorporation and cells hemolyze in the bone marrow.
A 43-year-old woman presents with difficulty walking and complaints
of depression that have progressively worsened over several weeks. She has no past medical history, takes no medications, and does not drink alcohol or smoke. Physical examination is remarkable for clinical depression, a spastic gait, decreased extremity proprioception, and lower extremity hyporeflexia. What test should be
obtained next in this patient’s evaluation?
a. Complete blood count with differential diagnosis and RBC indices
b. Lumbar puncture
c. Magnetic resonance imaging (MRI) of the spine
d. Serum potassium and calcium levels
Answer: a. Vitamin B12 deficiency presents classically with very low
hemoglobin levels, a macrocytic picture, decreased proprioception or vibration, lower extremity spasticity/weakness with hyporeflexia, and often mental status changes, such as depression, forgetfulness, or even paranoia. Irritability and forgetfulness are also seen with folate deficiency.
Discuter du traitement d’un INR suprathérapeutique avec ou sans saignement
voir tableau
What platelet count is generally recommended prior to performance
of a lumbar puncture?
a. 100,000 mm3
b. 50,000 mm3
c. 40,000 mm3
d. 20,000 mm3
e. 10,000 mm3
Answer: B. A platelet count above 50,000 mm3 is recommended prior to performance of invasive spinal procedures such as lumbar procedure.
A platelet count above 20,000 mm3 is recommended for central line
placement. A platelet count above 100,000 mm3 is recommended prior
to neurosurgical procedures or invasive ophthalmologic interventions
A 52 year-old-female presents to the emergency department with confusion, shortness of breath, and fatigue. Laboratory evaluation reveals a platelet count of 14,000 mm3 and hemoglobin of 7.2. mg/dL, a low MCV, indirect bilirubinemia, and a creatinine of 1.4 mg/
dL. Electrocardiogram shows non-pecific
T wave abnormalities in the lateral leads. She denies chest pain and vitals are normal. What would be the most reasonable next step in management?
a. Obtain a blood smear and utilize a validated risk score for possible TTP.
b. Immediate platelet transfusion
c. Administer tranexamic acid
d. Start IVIG
e. Administer heparin
Answer: A. Early identification of TTP is imperative. This patient presents
with classic signs and symptoms (neurologic symptoms, fatigue,
thrombocytopenia, and microangiopathic hemolytic anemia). A validated score such as the PLASMIC score should be used to rapidly assess the likelihood of TTP, as early plasma exchange is the treatment
of choice. A platelet transfusion will more than likely exacerbate symptoms.
Tranexamic acid prevents fibrinolysis, and therefore would likely cause harm in the setting of microthrombi in TTP. IVIG has not been shown to be the front-line
treatment for TTP. Heparin will further
increase this patient’s bleeding risk. The nonspecific T wave changes
are likely due to TTP.
A 22-year-old patient with hemophilia A presents to the ED with a headache. He has a history of migraines but this feels different from his previous headaches. He denies any recent significant closed
head injury, though states he fell while skateboarding 1 week prior.
He is concerned about the possibility of bleeding. Neurologic examination
is unremarkable. Upon further review of available records, the patient has a history of severe hemophilia A with inhibitors. His headache is worsening. What would be the best next step in management?
a. Obtain imaging of brain and cervical spine.
b. Consult neurosurgery for possible intracranial bleed.
c. Administer factor VII as expeditiously as possible.
d. Administer factor VIII as expeditiously as possible.
e. Administer a migraine “cocktail” for his headache and reevaluate in 30 minutes.
Answer: C. Patients with known hemophilia should be treated empirically
with factor replacement when intracranial bleeding is first suspected. An atypical headache, particularly in the setting of trauma (even seemingly minor or incidental trauma), should be treated with factor replacement as expeditiously as possible. Neurosurgery may need to be
contacted, though consultation should not delay factor administration.
Although this patient has hemophilia A (factor VIII deficiency), the presence of inhibitors (antibodies toward factor VIII) limit the utility of factor VIII replacement. In the setting of hemophilia with known
inhibitors and bleeding, recombinant factor VIIa is recommended. Medications for treatment of routine migrainous symptoms may be considered secondary to exclusion of life-or disability-threatening intracranial hemorrhage.
- An isolated elevation in prothrombin time (PT) or INR indicates an abnormality or deficiency in which of the following?
a. Calcium
b. Factor VII
c. Factor XI
d. Fibrinogen
e. Platelets
Answer: B. The PT or INR tests the components of the extrinsic pathway,
and abnormalities or deficiency in factor VII will cause elevation in PT and INR. Calcium derangements do not typically cause aberrations in PT, INR, or PTT. Factor XI is a component of the intrinsic
pathway reflected in the PTT. Fibrinogen function is reflected in both prothrombin time (PT) and PTT values. Platelet deficiencies do not prolong the PT or INR.
Décrire la patho du syndrome de lyse tumorale. Nommer 4 trouvailles à l’ECG. Nommer 3 tx à amorcer
Patho
- lyse massive de cellules tumorales qui dépasse les mécanismes d’élimination.
ECG
- QT allongé
- T pointues hyperK
- QRS élargi
- onde T aplaties
Traitements
- hydratation
- gestion hyperkaliémie ; calcium IV coeur
- allopurinol
- hémodialyse
- autres tx pour hyperK
F62 ans connue HODGKIN transfusée il y a 6 jours ; diarrhées, douleur HCD, frissons, rash érythémateux disséminé
Dx? Tx? T
Graft vs host desease
profiléfation leucocytes transfusés qui attaquent l’hôte. Pronostic sombre, tx palliatif (95% mortalité)
Nommer 5 trouvailles cliniques et paracliniques associées au purpura thrombocytopénique thrombotique PTT
Nommer 2 traitements
Trouvailles cliniques et paracliniques
- Fièvre
- sx neuro (confusion)
- IRA (rare, seulement si SHU associé)
- Anémie hémolutique
- Thrombocytopénie
- Hématurie/protéinurie
Traitements
corticostéroïdes
aphérèse plaquettes si risque saignement
rituximab
ASA
Patient polytraumatisé, 70 kg, connu hémophilie A
Quelle qté de facteur recombinant devrez-vous lui administrer?
Il faut lui administrer 50 U/kg de facteur recombinant, car risque saignement majeur. Considérer 100% de fonction facteur VIII nécessaire (chez pt à 0%)
50 U /kg x 70 kg = 3500U donc 7 vials si 500 U/vial
Nommer 4 causes d’anémie microcytaire, normocytaire et macrocytaire
MICROCYTAIRE
- ferriprive
- thalassémie
- plomb
- anémie sidéroblastique
- maladie inflammatoire
NORMOCYTAIRE
- hémolyse (schistocytes, helmet cells)
- hémorragie
- hypothyroïdie
- inflammatoire
- myélodysplasie
- déficience en G6PD (bite cells)
- insuffisance rénale
MACROCYTAIRE
- Déficit B12
- Déficit folates
- ROH chronique
- maladie hépatique
- hypothyroïdie
Nommer des maladies hématologiques qui procurent une protection contre la malaria
thalassémies mineures et majeures (alpha/bêta)
anémie falciforme
Nommer 4 causes de fièvre NON-INFECTIEUSE
Paranéoplasique
chimiothérapie
interaction médicamenteuse
saignement IC
embolie pulmonaire
thyroïdite immunotx
réaction transfusionnelle
Outre les gourganes, nommer 6 médicaments/substances contre-indiqués chez un patient atteint d’une déficience en G6PD
BOX 109.1 rosen (à compléter)
ASA
quinine, nitrofurantoine
SMX, sulfonidés
bleu méthylène
gourganes
Patiente ayant eu résection polypes et 2 transfusion, devient dyspnéique, tachypnéique et au RXP, surcharge évidente 2 plages.
Quel est le phénomène et le tx?
TRALI
transfusion related acute lung injury
cesser transfusion
aviser banque de sang
support ventilatoire
bolus/inotropes si hypta
Nommer doses de facteur VIII à donner à un patient hémophile en fonction du site de saignement
voir tableau
outre facteur VIII, considérer DDAVP et concentrés facteurs coagulation (bériplex)
Chez un patient ayant une leucostase importante, nommer les traitements
Hydratation veineuse abbondante
leucophérèse ou chimiothérapie
hydroxyurée
Qu’est-ce que la maladie de Christmas?
Maladie de Bernard Soulier
D’où vient la protamine
Nommer les 4 facteurs vit-K dépendants
hémophilie B
Anomalie adhésion plaquettaire
sperme de saumon
2-7-9-10
Nommer 4 médications associés au purpura thrombocytopénique immun PTI
ASA
AINS
phénytoïne
quinine
quinidine
digoxine
bêta-lactames (PNC)
- liste similaire au steven-johnson / anémies à comparer
Nommer 3 alternatives de classes différentes à l’héparine IV si le patient a un ATCD de HIT et doit être traité pour une embolie pulmonaire
- pas de coumadin
argatroban
bivalirudine
AOD (rivaroxaban, dabigatran, apixaban, edoxaban)
fondaparinux
Nommer le traitement de l’hypercalcémie (contexte de néoplasie)
Hydratation IV
biphosphonates
calcitonine
dialyse
denosubam
diruétiques de l’anse si hypervolémie
Nommer 4 dx différentiel de l’anémie hémolytique aigüe
- anémie hémolytique auto-immune
- thalassémies
- déficience en G6PD
- anémie falciforme
- sphérocytose
INFECTIEUX
- EBV
- malaria
- Syphillis
- mycoplasma
- brûlures
- morsure serpent
- CIVD
- médicamenteux
Définir transfusion massive d’au moins 2 manières et nommer 3 complications pouvant en résulter
DÉFINITION
10 produits sanguins en 24h*
3 culots GR en 1h
4 produits sanguins en 30 min
COMPLICATIONS
hypothermie
hypocalcémie (citrate)
hypo/hyperkaliémie
acidose métabolique (citrate s’accumule et métabolisme hépatique dépassé) et ensuite une alcalose car citrate -> bics
hypomagnésémie
Nommer 5 manifestation/complications neurologiques de l’anémie falciforme
AVC
ICT
ischémie médullaire
hémorragie intra-crânienne spotanée
tr équilibre/vestibulaire
11 ans, connue anémie falciforme. Boiterie et douleur hanche G x 48h.
Nommer 3 complications de l’anémie falciforme expliquant ce tableau
crise-vaso occlusive
ostéomyélite (salmonelle)
nécrose avasculaire
Comment renverser l’anticoagulation des patients suivants ;
héparine IV
Dabigatran
Xarelto
Héparine ;Protamine IV
Dabigatran ; Idarucizumab
Xarelto ; andexanet alfa, si non-dispo considérer bériplex
Jeune patient 2 ans avec saignement gingival. Nommer 2 DDX
Scorbut
Hémophilie
Gingivostomatite herpes
vonWillebrand sévère
Thrombocytopénie importante PTI/PTT
Fille 3 ans connue anémie falciforme qui a de la fièvre 38,5 x 24h. Votre prise en charge?
- Bilan septique complet : FSC, CRP, RXP, SMU-DCA, hémoc x 1, bilan hémolyse
- Hydratation IV
- Ceftri 50-100 mg/kg die ad hémoc nég
- Consult ped
Nommer 5 causes non-infectieuses de leucocytose
- Leucémies
- stress physio (sports, etc)
- conso drogues
- thyrotoxicose
- trauma
- brûlures
- polycythémie
Nommer 3 raisons pour lesquelles un patient devrait recevoir un culot irradié
transfusion intrautérine
néo hémato
transplant cellules souches
lymphome hodgkin
Donner la définition de neutropénie fébrile
FSC avec < 500 neutro ou moins
+ 38,3 x 1 ou 38C pour plus d’une heure
Nommer 3 raisons de considérer le plasma frais congelés à un patient
- PTM
- hémophilie avec saignement
- correction coagulopathie (INR < 1)
- angioedème héréditaire (contient c1 estérase)
Nommer 3 trouvailles au labo d’une réaction hémolytique aiguë
Diminution Hb
LDH augmentés
Haptoglobine diminuée
Bilirubine indirecte augmentée
évidence CIVD ; d-dimère aug, fibrinogène dim, INR augmenté
schistocytes ou sphérocytes au frottis
