Haematology/Immunology Flashcards
Hereditary angioedema
Autosomal dominant
Low plasma levels of the C1 inhibitor (C1-INH, C1 esterase inhibitor)
Serum C4 is the most reliable and widely used screening tool
Cancer patients with VTE
6 months of a DOAC
Pathogenesis of thrombotic thrombocytopenic purpura (TTP)
Abnormally large and sticky multimers of von Willebrand’s factor cause platelets to clump within vessels
In TTP there is a deficiency of ADAMTS13 (a metalloprotease enzyme) which breakdowns (‘cleaves’) large multimers of von Willebrand’s factor
Thrombotic thrombocytopenic purpura - features
Fever
Fluctuating neuro signs (micro-emboli)
Microangiopathic haemolytic anaemia
Thrombocytopenia
Renal failure
Initial treatment for CLL
Fludarabine, cyclophosphamide and rituximab (FCR)
Ibrutinib may be used in patients who have failed a previous therapy
The following groups of patients require CMV-negative blood
Intra-uterine transfusions
Neonates up to 28 days post expected date of delivery
Pregnancy
Fresh frozen plasma
Prepared from single units of blood.
Contains clotting factors, albumin and immunoglobulin.
Unit is usually 200 to 250ml.
Usually used in correcting clotting deficiencies in patients with hepatic synthetic failure who are due to undergo surgery.
Cryoprecipitate
Formed from supernatant of FFP.
Rich source of Factor VIII and fibrinogen.
Allows large concentration of factor VIII to be administered in small volume.
Hereditary spherocytosis - inheritance and pathophysiology
Northern European descent
Autosomal dominant
Red blood cell survival reduced as destroyed by the spleen
Diagnosis of Hereditary spherocytosis
EMA binding test
Long term treatment of Hereditary spherocytosis
Folate replacement
Splenectomy
1st line imaging in suspected multiple myeloma
Whole body MRI
Extravascular haemolysis: causes
Haemoglobinopathies: sickle cell, thalassaemia
Hereditary spherocytosis
Haemolytic disease of new-born
Warm autoimmune haemolytic anaemia
Smear/Smudge cells
Chronic lymphoblastic leukaemia (CLL)
Auer rods
Acute promyelocytic leukaemia
Basophilic stippling
Lead poisoning
Management of Lead Poisoning
Dimercaptosuccinic acid (DMSA)
Heinz bodies, bite and blister cells
G6PD deficiency
Hairy cell leukaemia - gene
BRAF
Management of Hereditary angioedema
IV C1-inhibitor concentrate or fresh frozen plasma if this is not available
Secondary causes of Polycythaemia
COPD
Altitude
Obstructive sleep apnoea
Warfarin-induced skin necrosis
While warfarin blocks the activation of clotting factors II, VII, IX, and X, it also deactivates protein C and protein S, two endogenous anticoagulants.3
Since protein C has a short half-life (8 hours) and warfarin initially decreases protein C levels faster than the coagulation factors, it can paradoxically increase the coagulation tendency when treatment is first begun, leading to massive thrombosis with skin necrosis and gangrene of limbs.
Reversal agent - Rivaroxaban and apixaban
Andexanet alfa
Reversal agent for dabigatran
Idarucizumab
Useful marker of prognosis in multiple myeloma
B2-microglobulin
Acute intermittent porphyria - diagnosis
Raised urinary porphobilinogen
Management of Acute intermittent porphyria
IV haematin/haem arginate
First-line treatment for ITP
Oral prednisolone
Pooled normal human immunoglobulin (IVIG) can also be used - faster inset if needed.
Basophilic stippling
Lead Poisoning
Features of Beta-thalassaemia trait
Mild hypochromic, microcytic anaemia
(microcytosis is characteristically disproportionate to the anaemia)
HbA2 raised (> 3.5%)
1st line imaging in suspected multiple myeloma
Whole body MRI
Polycythaemia vera: management
Aspirin
Venesection - aim PCV >0.45
Hydroxyurea - slight increased risk of secondary leukaemia
Investigation of choice for CLL
Immunophenotyping (Flow cytometry)
Treatment for acquired methaemoglobinaemia
IV methylthioninium chloride
Treatment of choice for NADH methaemoglobinaemia reductase deficiency.
(Congenital Methaemoglobinaemia)
Ascorbic Acid
Causes of Microcytic anaemia
Iron-deficiency anaemia
Thalassaemia
Congenital sideroblastic anaemia
Poor prognostic features in AML
> 60 years
> 20% blasts after first course of chemo
cytogenetics: deletions of chromosome 5 or 7
Ki-67 High (>45%)
Diffuse Large B Cell Lymphom
Ki-67 Low (<40%).
Follicular Lymphoma
Acute myeloid leukaemia (AML) is characterised by a peripheral blast count > ?%
> 20%
Most common inherited bleeding disorder
Von Willebrand’s disease
Wiskott-Aldrich syndrome
Characterised by a triad of:
Recurrent chest infections/recurrent sinus infections
Atopic dermatitis
Thrombocytopenia and platelet dysfunction
X linked recessive
Reversal agent for Apixaban/Rivaroxaban
Andexanet alfa
Triad of:
Cholestatic jaundice (high ALP/GGT/bilirubin)
DAT-negative haemolytic anaemia
Hyperlipidaemia
Zieve’s syndrome
Treatment of paroxysmal nocturnal haemoglobinuria (PNH)
Eculizumab
Lack of NADPH oxidase reduces ability of phagocytes to produce reactive oxygen species
Chronic granulomatous disease
Most common primary antibody deficiency. Recurrent sinus and respiratory infections
Associated with coeliac disease and may cause false negative coeliac antibody screen
Severe reactions to blood transfusions may occur (anti-IgA antibodies → analphylaxis)
Selective immunoglobulin A deficiency
Gold standard investigation in PNH
Flow cytometry of blood to detect low levels of CD59 and CD55
Methaemoglobinaemia - define
Haemoglobin which has been oxidised from Fe2+ to Fe3+
Most common inherited thrombophilia
Activated protein C resistance (Factor V Leiden)
Management of Von Willebrand’s disease
Tranexamic acid for mild bleeding
Desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
Factor VIII concentrate
Indications for gamma irradiated blood products include:
Immunocompromised marrow or organ transplant recipients
Patients with haematological disorders who will be undergoing allogeneic marrow transplantation imminently
Intrauterine transfusions
Patients with Hodgkin’s disease
Patients treated with purine analogue drugs (e.g. fludarabine)
Prophylaxis for Hereditary angioedema
Anabolic steroid - Danazol
All patients should be screened for ? before treatment with rituximab
Hepatitis B (HBV)
? mainstay of imaging for staging Hodgkin’s lymphoma
PET/СT
? with ondansetron is effective in refractory chemotherapy-induced vomiting
Dexamethasone
Squamous cell cancer (Lung) - where?
Typically central
Adenocarcinoma (Lung) - where?
Typically peripheral
? to treat hiccup
Metoclopramide
Chlorpromazine
Dexamethasone
The most common side effect of immune checkpoint inhibitors is
Dry itchy skin and rashes
