Haematology/Immunology Flashcards by Harisanth Rajaram

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

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Cancer patients with VTE

6 months of a DOAC

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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

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Thrombotic thrombocytopenic purpura - features

Fever

Fluctuating neuro signs (micro-emboli)

Microangiopathic haemolytic anaemia

Thrombocytopenia

Renal failure

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Initial treatment for CLL

Fludarabine, cyclophosphamide and rituximab (FCR)

Ibrutinib may be used in patients who have failed a previous therapy

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The following groups of patients require CMV-negative blood

Intra-uterine transfusions

Neonates up to 28 days post expected date of delivery

Pregnancy

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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.

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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.

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Hereditary spherocytosis - inheritance and pathophysiology

Northern European descent

Autosomal dominant

Red blood cell survival reduced as destroyed by the spleen

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Diagnosis of Hereditary spherocytosis

EMA binding test

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Long term treatment of Hereditary spherocytosis

Folate replacement

Splenectomy

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1st line imaging in suspected multiple myeloma

Whole body MRI

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Extravascular haemolysis: causes

Haemoglobinopathies: sickle cell, thalassaemia

Hereditary spherocytosis

Haemolytic disease of new-born

Warm autoimmune haemolytic anaemia

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Smear/Smudge cells

Chronic lymphoblastic leukaemia (CLL)

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Auer rods

Acute promyelocytic leukaemia

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Basophilic stippling

Lead poisoning

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Management of Lead Poisoning

Dimercaptosuccinic acid (DMSA)

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Heinz bodies, bite and blister cells

G6PD deficiency

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Hairy cell leukaemia - gene

BRAF

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Management of Hereditary angioedema

IV C1-inhibitor concentrate or fresh frozen plasma if this is not available

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Secondary causes of Polycythaemia

COPD

Altitude

Obstructive sleep apnoea

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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.

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Reversal agent - Rivaroxaban and apixaban

Andexanet alfa

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Reversal agent for dabigatran

Idarucizumab

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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

Haematology/Immunology Flashcards

(60 cards)