Haematology Flashcards by William Li

Blood film - splenectomy

Howell-Jolly bodies - nuclear fragments in RBC
Acanthocytes - spur cells, thorn/spicules at irregular intervals on surface of RBC
Target cells - central density with halo of pallor in RBC

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Blood film - iron deficiency anaemia (IDA)

Microcyctic (small) RBC
Hypochromic (thin rim of pink) RBC
Poikilocytosis - abnormal shaped RBC, eg. “pencil” shaped cells

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Blood film - hypothyroidism

Normocytic, normochromic, hypoproliferative, anaemia

- In chronic autoimmune thyroiditis - pernicious anaemia (macrocytic, marrow megaloblastosis)

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Blood film - B12 and folate deficiency

Macrocytosis - macro-ovalocytes, occasional megaloblast
Teardrop cells
Hypersegmented neutrophils

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Coagulation studies - acquired factor VIII inhibitor

Prolonged APTT

Mixing test - corrects initially, no correction in 2 hour incubation (characteristic)

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Haemophilia A

Congenital factor VIII deficiency

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Haemophilia B

Congenital factor IX deficiency

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Most common haemophilia bleeding sites

Haemarthrosis (70-80%) - primarily knee and elbow
Muscle / soft tissue (10-20%)
Other major bleed (5-10%)
CNS bleed (rare, less than 5%)

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Most common Von Willebrand disease bleeding sites

Bruising
Skin bleeding
Mucosal bleeding

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Coagulation studies - disseminated intravascular coagulation (DIC)

Prolonged APTT
Prolonged PT/INR
Low fibrinogen
High d-dimer

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Coagulation studies - heparin

Prolonged TCT (thrombin clotting time)
Normal reptilase

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Coagulation studies - dysfibrinogenaemia

Prolonged TCT

Prolonged reptilase test

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Lugano classification (for non-Hodgkin’s lymphoma)

Stage I - disease on one side of diaphragm
Stage II - more than one area same side diaphragm
Stage III - lymph nodes above and below diaphragm
Stage IV - extranodular disease
E = extranodal contiguous extension

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Choice of treatment modality in non-Hodgkin’s lymphoma

Based on Lugano classification:
Stage I - radiotherapy (curative in 50%)
Stage II - consider radiotherapy if low bulk and within field, otherwise treat as advanced
Stage III/IV (advanced) - chemooimmunotherapy ± radiotherapy, for symptom control

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Intravascular haemolysis - investigations

Presence of urine haemosiderin - most specific
Increased LDH
Increased bilirubin
Decreased haptoglobin

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Most common lymphoproliferative disorders associated with Epstein-Barr virus (EBV)

Hodgkin’s lymphoma

Burkitt’s lymphoma

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Cytogenetics - follicular lymphoma

t(14;18) - bcl-2 / IgH

overexpression of BCL-2, an anti-apoptosis protein

Cytogenetics - diffuse large B-cell lymphoma (DLBCL)

No single typical abnormality.
BCL-6 - chromosome 3, numerous alterations seen
t(14;18) - bcl-2 (30%)
t(8;14) - c-MYC (5-15%)

Cytogenetics - mantle cell lymphoma

t(11;14) - cyclin D1 / IgH

abnormal dysregulated expression of cyclin D1 in B lymphocytes, involved in control of G1 phase of cell cycle

Cytogenetics - Burkitt lymphoma

t(8;14) - 80%, c-MYC / IgH
t(2;8) and t(8;22) - also seen

(c-MYC dysregulation driven by IgH enhancer)

Cytogenetics - acute myeloid leukaemia (AML)

Recurrent genetic abnormalities in 11% of cases.
t(8;21) - 7% adults, most frequent in children
t(16;16), inv(16) - 5% adults, chromosome 16 abnormality

Cytogenetics - acute promyeloid leukaemia (APL)

t(15;17) - PML-RARA fusion protein

aberrant retinoid receptor with altered DNA binding and transcriptional regulatory properties

Cytogenetics - acute lymphocytic leukaemia (ALL)

Hyperdiploidy - more than 46 chromosomes

t(9;22) - confers worse prognosis

Cytogenetics - chronic myeloid leukaemia (CML)

t(9;22) - Philadelphia chromosome

bcr-abl leads to increased PO4 of TK residues on substrates, switches on signalling pathways

Cytogenetics - chronic lymphocytic leukaemia (CLL)

del(17p) - 7-10%, worse prognosis del(11q) - 17-20%, worse prognosis TP53 mutation - 10-47%, worse prognosis del(13q) - 45-55%, favourable prognosis

Blood film - primary myelofibrosis

- Teardrop cells (dacrocytes) - Poikilocytosis - abnormal shaped RBC - Nucleated RBC - Leukopenia/ thrombocytopenia (can have leukocytosis/ thrombocytosis)

Most common myeloproliferative disorder associated with Budd Chiari syndrome

Polycythaemia rubra vera (PCV)

Blood film - myelodysplasia

- Anaemia (with anisocytosis) - Leukopenia - Thrombocytopenia (can transform to AML)

Polycythaemia rubra vera (PRV) - diagnostic criteria

WHO critera - 2 major + 1 minor, or 1 major + 2 minor Major: - Hb more than 185 men, 165 women - JAK2 mutation present Minor: - Bone marrow biopsy (hypercellularity, trilineage growth) - Serum EPO level low - Endogenous erythroid colony formation in vitro

Myelodysplastic syndrome (MDS) - treatment

Azacitidine | Lenalidomide - in deletion 5q

Blood film - autoimmune haemolytic anaemia (AIHA)

- Spherocytes - dark red, sphere shaped RBC (rather than biconcave) - Microspherocytes - dark red, small spherocyte

Blood film - microangiopathic haemolytic anaemia (MAHA)

- Schistocytes - fragmented RBC including helmet cells, triangle cells - Helmet cells - RBC shaped as bicycle, combat or football helmets (Seen in TTP, HUS)

Blood film - thalsassaemia

- Target cells - central density with halo of pallor - Hypochromia - Microcytosis - Teardrop cells - Basophilic stippling - blue granules on RBC

Blood film - liver disease

- Target cells - central density with pale halo (due to membrane excess)

Thrombotic thrombocytopenic purpura (TTP) - presentation

- Microangiopathic haemolytic anaemia - Thrombocytopenia - Fever - Neurological manifestations - Minimal renal abnormalities

Haemolytic uraemic syndrome (HUS) - presentation

- Microangiopathic haemolytic anaemia - Thrombocytopenia - Acute kidney injury - Bloody diarrhoea - in typical (secondary) HUS

ADAMTS13 (A Disintegrin And Metalloprotease with a ThromboSpondin type 1 motif, member 13) deficiency

- Causes TTP - Activity level <10% - Due to ADAMTS13 gene mutation, or inhibitory antibodies

Plasma exchange / plasmapheresis - indications

- Guillain-Barre syndrome - ANCA RPGN - Anti-GBM (Goodpasture's) - CIDP - Cryoglobulinaemia - FSGN - Atypical HUS - TTP - Wilson's disease - Hyperviscosity in monoclonal gammopathy - Renal or liver transplantation - desensitization, Ab rejection - Myasthenia gravis - PANDAS - Paraproteinemic polyneuropathy

Promoter of neutrophil production

G-CSF (granulocyte colony stimulating factor)

Promoter of platelet production

TPO (thrombopoietin)

Heparin - mechanism of action

- Binds and activates anti-thrombin III - Activated ATIII inactivates other factors (mainly Xa) - Simultaneous binding of ATIII and thrombin to inactivate thrombin (LMWH cannot do this)

Protein C - mechanism of action

- Activated by thrombin-thrombomodulin complex - Protein S is co-factor for activation - Activated protein C (APC) inactivates factor V and VIII