CFPC 2027-Hemato

% blasts

< 20% = SMD or chronic

> 20% = acute

Acute leukemia vs. chronic leukemia

Acute: immature cells, blasts > 20% BMO

Chronic: mature/differentiated, blasts < 10%

Lymphoid vs. myeloid

ALL, AML, CLL, CML

Leukemia tests

FSC (leucocytose) + différentielle, frottis, bx moelle

**they all present with leucocytosis (except CLL that can sometimes present with leucopenia)

What can SMD turn into

AML

How to differentiate between AML and CML?

Thrombocytopnenia is more common in AML.

Tips for differentiating leukemias

ALL: children

CML: Chromosome (philadelphia), Massive splenomegaly

AML: Auer rods

CLL: Crushed Little Lymphocytes (smudge cells)

Réactions aiguës post-transfusion

-Allergie (urticaire, anaphylaxie)

-Réaction hémolytique aigue: fièvre, dlr abdo, hypoTA.

-Fébrile non hémolytique

-TRALI: OAP + SDRA

-TACO: fluid overload

Réactions tardives post transfusion

-Delayed hemolytic transfusion reaction: jour 5, ictère, fièvre.

-Iron overload

Cascade de coagulation

DVT treatment

1. HBPM x ≥ 5 jours puis switch to ACOD or warfarin.

Provoked: x 3 months

Unprovoked: 3-6 months or life, investiguer thrombophilie, SAP.

CIVD

Thrombus formation (in microvasculature) and depletion of clotting factors and platelets, which then results in bleeding.

CIVD etiology

CIVD tests

FSC (thrombocytopénie)

Frottis: schistocytes

Bilan de coag.: ↑PT, ↑PTT, ↓fibrinogen

↑d-dimers

CIVD tx

Tx cause ss jacente

Transfusions of platelets or clotting factors

Thrombocytose essentielle

F 50-70 A

Myeloproliferative disorder typified by dysregulated megakaryocyte proliferation.

Mutation JAK2 V617F (++)

TE key sx

50% asx

Erythromelalgie (red/blue discolouration of the extremities + burning sensation)

Livedo reticularis (net like purple rash)

TE tests

FSC: plaquettes ↑

JAK2 mutation

TE tx

aspirine ± hydroxycarbamide (si haut risque)

Déficience G6PD

X-link

Jews/Filipinos/Greeks/Sardinians/Kurds/black

Triggering factors: medications (AINS, aspirin), infections, or foods (fava beans)

Acute haemolytic episodes characterised by jaundice, anaemia, and dark urine.

Déficience G6PD tests

FSC: anaemia, reticulocytosis

Frottis: Heinz bodies

Elevated bilirubin levels

Déficience G6PD tx

support

Anemia classification

Hb <135 for men <120 for women

Microcytic: TAILS (thalassemia, anemia chronic disease, IDA, lead, sideroblastic)

Normocytic: ABCD (acute blood loss, bone marrow failure, chronic disease, destruction)

Macrocytic: FATRBC (folate, alcohol, thyroid, reticulocytosis, B12 def, cytotoxic drugs)

When to consider transfusion

symptomatic, unstable, ongoing losses

Tests anemia

FSC, reticulocytes, iron profile, HBEP, frottis, LDH, haptoglobin, indirect bili, Coombs test (direct and indirect), TSH, B12, folate, INR, DIC panel (fibrinogen, d-dimer)

Bone marrow bx

Risk factors of anemia in children

poverty, poor weight gain, excessive milk intake + low iron-rich foods [malnutrition], malabsorption, GI blood loss, obesity

screen in all children between 9-12 months

IDA investigation

• CBC + ferritin +/- TIBC

• Consider reticulocytes

• Consider Hb Electrophoresis, Blood Smear

• Screen all for celiac (Anti-TTG, IgA)

• Consider complete iron studies (serum ferritin, total iron binding capacity [TIBC]), and serum iron

• Diagnostic trial of iron treatment x3 weeks in premenopausal women with history of menorrhagia or pregnant women

  • All men/postmenopausal women screened for GI malignancy

• B12/folate should be checked in normocytic, inadequate response to iron treatment, or suspected (malnutrition, malabsorption, elderly - pernicious anemia)

• Consider SPEP

Tx IDA

Diet

ferrous sulfate 300 die or q2d

± venofer IV

Re-check Hb in 2-4 weeks, then FU in 2-4 months

When normalized, continue tx x 3 months then d/c

Monitor periodically

Sx B12 deficiency

• Anemia

  • Fatigue, palpitations, skin pallor

• Glossitis

• Neuropsych

  • Symmetric peripheral neuropathy/paresthesias

  • Ataxia/gait abnormalities

  • Memory loss

  • Personality changes

  • Dementia

Hémophilie A/B

Inherited bleeding disorders caused by deficiencies in clotting factors VIII (A) and IX (B).

X-linked

Hémophilie A/B sx

Deep and severe bleeding into soft tissues, joints, and muscles.

Excessive bleeding post tonsillectomy

Hémophilie A/B tx

Desmopressine (A), recombinant factor VIII (A) or IX (B)

Lymphome Hodgkin présentation and dx

young adults (peak in 30s) with cervical or supraclavicular non-tender lymphadenopathy

Painful lymphadenopathy induced by ROH

Bx ganglion: Reed-Sternberg cells

Lymphome Hodgkin fdr

VIH, EBV, immunosuppression, tabagisme

PTI

Enfants

Triggered by viral infx or immunisation

Tx: observation ou CS/splenectomie si réfractaire

Anémie ferriprive tests

↓ferritine, ↓ iron, ↑ TIBC

Tx B12 deficiency

hydroxocobalamin IM

Order for tx folate and b12 deficiency

Treat b12 then add folate to avoid exacerbating neurological symptoms and precipitating subacute degeneration of the spinal cord (SACD).

SMD

Adults, > 70 ans ++

Ineffective haematopoiesis, leading to cytopaenias and dysplastic changes in blood cells.

Frottis: Howell Jolly

Bx: dysplasie ≥ 10%, < 20% blastes

Myelofibrose (aka splenomegalie myeloide)

Pancytopénie

“Dry tap” during bone marrow aspiration

frottis: tear drop poikilocytes (dacryocytes)

Non Hodgkin lymphoma

Commonly associated with infections and immunodeficiency.

Sx: symmetrical and painless lymphadenopathy, and is more likely to be associated with B symptoms.

Tests:

-LDH is the most important (poor prognostic marker)

-Absence of Reed-Sternberg cells

Polycythemia

Increase in haematocrit, red cell count, and haemoglobin concentration.

Sx: myalgie, céphalée, splénomégalie

Tests: ↑ hematocrit, Hb, leucocytose, thrombocytose, JAK2 +, hypercellular bone marrow

Tx: venesection, phlebotomy, aspirin, cytoreductive therapy (high risk)

Anémie falciforme

Mutation in the beta chain on chromosome 11 leads to the production of HbS - sticky and fragile (rather than HbA).

Sx: crise vaso-occlusive, crise respiratoire

Tests:

-Frottis: drepanocytes, target cells, Howell-jolly (hyposplénisme)

Thalassémie

Abnormal haemoglobin production

Autosomal recessive

a-thalassemia

Tx: blood transfusions, stem cell transplantation, and splenectomy

b-thalassemia

Tx: blood transfusions and iron chelating agents

Thrombophilie

Ajout: polycythémie (acquired)

PTT

Abnormal ADAMST13 activity

Tests:

-Frottis: reticulocytes, schistocytes

-LFT, LDH, D-dimer ↑

-Haptopglobins ↓

-Dx: ↓ ADAMST13 activity

Tx: CS, plasma exchange

Maladie von Willebrand

Prolonged bleeding from minor wounds, easy bruising, menorrhagia, epistaxis, and GI bleeding.

Test: ↓ fctr VIII, PT N, ↑ APTT

Tx: desmopressin, acide tranexamique if pre-op.

Anemia classification

how to reverse dabigatran

Idarucizumab