Hematology

Question 1

Metalloprotease ADAMSTS13

Answer 1

Cleaves Willebrand factor

Question 2

PLASMIC SCORE

Answer 2

Scoring system that aids in diagnosing iTTP

Question 3

Thrombotic anti phospholipid syndrome

Answer 3

Eculizumab and ravulizumab

Question 4

Antiphospholipid

Answer 4

Lupus anticoagulant, anticardiolipin, anti-beta-2-glycoprotein antibodies

Question 5

Complement activation

Answer 5

1. Classical (Aby-atgn complex)
   2.Lectin pathway activ. Mannose binding lectin
2. Alternative pathway

Question 6

Main players of complement

Answer 6

C3, C5a (potent pro inflammatory),C5b-9 ( membrane attack complex)

Question 7

Complement mediated disorders

Answer 7

Paroxysmal nocturnal hemoglobinuria, cold agglutinin, aHUS,

Question 8

Anaphylatoxins C3a, C5a, C5b-9

Answer 8

C3a, C5a, C5b-9

Question 9

C3a and C5a release

Answer 9

Pro inflammatory & procoagulant cytokines such: tumor necrosis factor,
Interleukin-6 from monocytes & endothelial cells

Question 10

Monocytes & endothelial cells induce

Answer 10

TNF & interleukin -6 which induce tissue factor

Question 11

C5a recruits

Answer 11

Neutrophils which induces neutrophil tissue factor dependent procoagulant.

Question 12

C5a-9 induces secretion via endothelial and platelets

Answer 12

VWF, p selectin, pro- inflammatory cytokines, procoagulant extracellular vesicles

Question 13

Monocytes release

Answer 13

TNF & Interleukin 6

Question 14

Neutrophil release

Answer 14

Tissue factor dependent procoagulant activity

Question 15

Neutrophils, monocytes, platelets

Answer 15

Hypercoagulable

Question 16

C3 activates platelets and fibrin formation

Question 17

Complementopathies cause thrombosis often refractory to anticoagulation and seen triggered in:

Answer 17

Pregnancy, inflammation, cancer, PNH, cold agglitinin, aHUS, transplant microangiopathy, HELLP, APS, CAPS

Question 18

Complement inhibitors

Answer 18

Eculizumab, ravlizumab

Question 19

Complement inhibition

Answer 19

CAPS, HELLP

Question 20

Waldenström macroglobulinemia

Answer 20

1. Indolent B cell lymphoma
2. Hepatosplenomegaly
3. Lymphadenopathy
4. Circulating IgM

Question 21

ITP

Answer 21

Anti-CD 38 monoclonal antibody
CM313

Question 22

Iron deficiency (hepcidin controls release of iron)

Answer 22

1. Ferritin <100
   OR
2. Ferritin 100-300 and total iron saturation <20%

Question 23

HELLP syndrome

Answer 23

Hemolysis
Elevated LFT
LOW PLATELETS

Question 24

Autoimmune cytopenia

Question 25

Cryoglobulinemia

Answer 25

Diagnostic testing; cbc with diff, SPEP, serum protein immunofixation,CT scan (thoracic, abdomen), PET scan, bone marrow biopsy. Type II,III VIRAL PANEL B,C ANA, anti double dna, salivary gland biopsy

Question 26

Flow cytometry

Answer 26

Helps distinguish malignancy

Question 27

Tumor lysis

Answer 27

Low calcium High phosphorus, uric acid, potassium Treatment: rasburicase 3mg, sevelamar 800mg tid

Question 29

HLH/MAS clinical manifestation

Answer 29

Inflammation: high ferritin. H CRP, splenomegaly, fever CNS - encephalopathy , seizures ,CSF (pleocytosis, high csf protein) Cytopenia: low hgb, leucopenia, low platelets Coagulopathy, DIC, high LFT, hi triglyceride

Question 31

HLH/MAS (MÁS CRITERIA/HS SCORE) Causes

Answer 31

Infection (CMV, cancer, HSV, HINI, Ehrlichea)immunotherapy, Covid 19, SLE, lymphoma, Ebola, Dengue, Crimean-Congo)

Question 32

Ferritin

Answer 32

Secreted by hepatocytes, in response to IL6, TNFa, growth differentiation factor 15.

Question 34

Consumptive coagulopathy

Answer 34

DIC

Question 35

Caplacizumab is a von Willebrand factor (vWF)-directed monoclonal antibody fragment which targets the A1-domain of vWF, inhibiting the interaction between vWF and platelets, reducing both vWF-mediated platelet adhesion and platelet consumption.

Answer 35

Treatment of TTP

Question 36

HIT TREATMENT

Answer 36

Fondaparinux

Question 37

Check glucose 6 phosphate dehydrogenase enzymes in hemolysis