Haematology Flashcards by Laura Brown

Translocation associated with mantle cell lymphoma

(11;14) translocation

(q13;q32) cyclin D1 (CCDN1) and immunoglobulin heavy chain (IgH)

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List 2 GIIB/IIIB inhbitors

abciximab
eptifibatide

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Deficiency in:
- Bernard soulier
- grey platelet syndrome
- glanzmans thrombesthenia

BS - glycoprotein abnormalities
GPS - alpha granule deficiency
GT - GIIb/IIIB defect

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Treatment of essential thrombocytosis

If no JAK mutation, age <60yo, no vasomotor sx or CVS risk factors, can observe

Otherwise, aspirin daily (BD if CVS risk factors)

ADD hydroxyurea if high risk (i.e. previous clots, >60YO) with target platelets <400

ADD anticoagulation if previous VTE

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What conditions is eculizumab inducated for?

PNH
Atypical HUS (complement mediated TMA)

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Hepcidins action on iron

What effect does inflammation have on it?

Decreases serum iron

Inhibits ferroportin (major role)
- decreased recycling of iron from old RBCs/macs via the reticuloendothelial system
- decreased release of iron into the circulation at basolateral membrane of enterocytes

Inhibits intestinal iron absorption (minor role

Inflammation increases hepcidin

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Common mutations in myelodysplastic syndromes

Which are associated with a poor prognosis?

TET2
SRSF2
ASXL1
DNMT3A
RUNX1

SRSF2, RUNX1 and TP53 = poor prognosis

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Which form of MDS does lenalidomide work best in

isolated del5q

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Poor prognostic markers in MM

High beta 2 microglobulin
Low albumin
t(4;14)

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Standard treatment for MM if ineligible for transplant

What can be added if high risk?

Bortezomib (proteosome inhibitor)
Melphalan (akylating agent) or lanalidomide
Prednisone

Antibodies
- antiCD38: daratunumab, isatuximab

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Most common feature of MM

Anemia

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Hodgkins lymphoma with best prognosis

worst prognosis?

Nodular sclerosing’

Lymphocyte depleted (worst)

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What are Dohle bodies and when do they occur

Toxic granulation of neutrophils

Leukomoid rection - associated with high LAP socre and differentiating feature from CML

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Features of WM

Funduscopic abnormalities
Constitutional “B” symptoms
Bleeding
Neurologic symptoms
Hyperviscosity
Lymphadenopathy
Hepatomegaly
Splenomegaly

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What does TCT test?

Name conditions where it will be prolonged

Fibrinogen conversion to fibrin (i.e. the action of thrombin)

Unfractionated heparin
Direct thrombin inhibitors (dabigatran)
Liver disease
DIC and acquired fibrinogen disorders
Hypoalbuminemia
Paraproteinemia

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Abelacimab mechanism of action

Factor XI inhbitor

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Prolonged APTT with normal reptilase time indicates what

Heparin contamination

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Test to distunguish secondary and primary polycythemia

EPO level
- low in primary
- high in secondary

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Spindle cells and fibrosis on BM biopsy with elevate tryptase

Mutation?

Systemic mastocytosis

KIT mutations (D816V common)

Empiric antibiotics for patients with febrile neutropenia life threatening penicillin allergy

Non-life threatening penicillin allergy?

Life threatening:
- aztreonam OR cipro
- AND vancomycin

Non-life threatening
- cefepime (no vanc)

Empiric antibiotics for patients with febrile neutropenia and systemic compromise

As for non life threatening (pip-taz) and:
- ADD gentamycin
- ADD vanc if signs of cellulitis

If GI features and not on pip-taz/mero, need to cover for anaerobes with metronidazole

What is daratunumab?
What is the major issue?

Anti-CD38, used in MM

Interferes with pre-transfusion cross match due to CD38 markers on RBCs

Malignancy associated with “starry sky” picture on blood film

Mutation?

Burkitts Lymhoma

MYC;IgH translocation (8;14)

What is gemtuzumab and when is it used?
Major issue?

Anti-CD33, used in AML (less common now)

causes hepatic sinusoidal obstructive(veno-occliusive) syndrome
Treat with defibrotide

CML translocation

t(9;22)

APML translocation

t(15;17)

Indications for treatment in CLL

Worsening anemia/thrombocytopenia Splenomegaly Lymphadenopathy Progressive lymphocytosis - doubling time <6 months OR increase >50% over 2 months Autoimmune anemia/thrombocytopenia poorly responsive to steroids Symptomatic/functional extranodal involvement (skin, kidney, lungs, spine) Constitutional symptoms - unintentional LOW, fevers, fatigue, night sweats

Poor prognostic markers in CLL

BIG ONE - Del(17p)/TP53 Lymphocyte doubling time <12 months Del(11q) Unmutated IGHV Age >60 High B2 microglobulin

Best evidence for prevention of post thrombotic syndrome

Anti-coagulation

Definition of MM

>10% clonal plasma cells + either of: - End organ damage (1 or more) - CRAB sx - presence of a biomarkers (1 or more) - ≥60% clonal plasma cells in BM, FLC ratio ≥100, MRI with >1 focal bone lesion

Chains in: - HbA - HbA2 - HBF - HBH - Hb Barts

HBA: 2 alpha, 2 beta (normal) HBA2: 2 alpha, 2 delta (beta thal) HBF: 2 alpha, 2 gamma HBH: 4 beta (alpha thal) Hb Barts: 4 gamma (alpha thal)

hemoglobin in sickle cell

HBS - 2 alpha, 2 beta Mutated beta chain

Mutation in sickle cell disease

missense mutation [Glu6Val, rs334] in the β-globin gene [HBB]

What is hemoglobin E? When is it significant?

HbA chains (2 alpha, 2 beta) with mutated beta chain Significant when occurs with beta thalasamia; significant beta chain reduction

Features of red cell aplasia

Normochromic, normocytic anemia elevated EPO Absent erythroblasts and f giant pronormoblasts in the bone marrow.

Microspherocytes are associated with what

Autoimmune hemolytic anemia

Poor prognostic features in ALL

Philadelphia chromosome (9;22) Male gender Age <2 or >10 Initial WCC >100 CNS involvement Non-caucasian FAB L3 T or B cell surface markers

Good prognostic features in ALL

FAB L1 Common ALL Pre-B phenotype Low initial WCC

In what organ is thrombopoetin mostly produced

Liver Small amounts produced in kidney and bone marrow

Effects of anticoagulants on coagulation studies: - Warfarin - Apixaban - Rivaroxaban - Dabigatran - Heparin

Warfarin - prolonged INR, variable APTT Apixaban, rivaroxaban - PT>APTT, TT normal Dabigatran - TT>APTT>PT Heparin - APTT>TT, normal reptilase, normal PT

Reason for increased VTE risk in malignancy

Increased tissue factor production by cancer cells --> excessive extrinsic pathway activation

Warm Autoimmune Haemolytic Anaemia? - Mech - Disease Assoc - Coombs? and BLood film - Tx

IgG and complement D: Drugs, Virus, Autoimmune, CLL - Direct commobs anti-IgG and anti-C3 positive - Microsphereocytes, Extravascular main in spleen Tx: steriods, splenectomy if refractory

Cold Autoimmune Haemolytic Anaemia? - Mech - Disease Assoc - Coombs? and BLood film - Tx

IgM bings + complement - destruction mainly in liver or intravascular for acute. Tx: rituxibab D: post infection, paroxysmal, lymphoproliferative diease Intravscular, agglutination.

Factor V Leiden mechanism?

Resitance to action of protien C factor. Most common iherited thrombophilia. Hetro = 5 x Homo - 50 fold risk

Polycythaemia rubra vera common transformations?

Myelofibrosis + AML Mx: veneestction, aspirin, hydroxyurea.