Haematology Flashcards
Exposure to asbestos is a risk factor for
bronchial carcinoma as well as mesothelioma , laryngeal cancer and ovarian cancer
Exposure to asbestos also increases the risk of some benign diseases, including pleural plaques, diffuse pleural thickening, asbestos related benign pleural effusions and asbestosis.
*******raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
desmopressin (DDAVP):
Hereditary angioedema (HAE) is an
autosomal dominant
It should be suspected in any patient presenting with an acute kidney injury in the presence of a high phosphate and high uric acid level.
TLS , hypocalcemia and hyperkalemia
Cisplatin causes which electrolyte abmormality
Hypomagnesemia
. This condition is an X-linked recessive disorder characterized by the triad of eczema, recurrent infections, and thrombocytopenia.
history of recurrent infections, which can be explained by the low immunoglobulin M levels.
Wiskott-Aldrich syndrome
*** is an X-linked disorder that results in a lack of mature B cells and therefore very low levels of all immunoglobulins.
Bruton’s congenital agammaglobulinaemia
Ataxic telangiectasia is an autosomal recessive disorder characterized by progressive cerebellar ataxia, oculocutaneous telangiectasias, immune deficiency, and increased risk for malignancies.
patients with ataxic telangiectasia may have recurrent infections due to immune deficiency, they do not typically present with thrombocytopenia or eczema
is an autosomal recessive disorder characterized by partial albinism, peripheral neuropathy, and neutropenia.
Chediak-Higashi syndrome
Patients with Chediak-Higashi syndrome often have recurrent infections due to impaired neutrophil function
they do not usually present with thrombocytopenia or eczema as seen in this case.
**** is a genetic disorder caused by a deletion in chromosome 22q11.2, leading to thymic hypoplasia or aplasia and resulting in T-cell deficiency.
DiGeorge syndrome
Patients with DiGeorge syndrome often have recurrent infections due to their impaired T-cell function, but they do not typically present with thrombocytopenia or eczema.
fever, neurological signs, thrombocytopenia, haemolytic anaemia and renal impairment
TTP
**** that is secondary to certain bacterial infections (shigella, enterohaemorrhagic E. coli ), therefore the presentation is usually preceded by a period of diarrhoea. The triad of symptoms includes: thrombocytopenia, haemolytic anaemia and renal impairment.
haemolytic-uraemic syndrome is thrombotic microangiopathy
. The patient has polycythaemia rubra vera as evidenced by a ruddy complexion, polycythaemia and a positive mutation for JAK2V617F. These patients are at risk of developing myelofibrosis or acute myeloid leukaemia.
Type I cryoglobulinaemia
CRAB mnemonic: >65 years old, hypercalcaemia with abdominal pain and dehydration, renal failure, anaemia and bone pain in her back)
coupled with the secondary Raynaud’s phenomenon evidenced by the hand symptoms/signs being indicative of the cryoglobulins which precipitate at cold temperatures. Type I is the only cryoglobulinaemia that presents with Raynaud’s and is also most strongly associated with multiple myeloma out of the three types.
Type 2 cryoglobulinemia
A/w hepatitis
**** is a monoclonal antibody that binds to HER2 receptors on the cancer cells, thereby suppressing the growth and proliferation of the tumour. It is effective in HER2-positive breast cancers.
Trastuzumab
****is a type of selective oestrogen receptor degrader that binds to and breaks down oestrogen receptors
Fulvestrant
This is characterised by a leukocytosis over 50 * 109/L. Typically, there is a neutrophil predominance. This reaction occurs in a variety of conditions, such as infections, reactions to medications, diabetic ketoacidosis, but also certain malignancies, including lung adenocarcinoma
Leukemoid reaction
Leucocyte alkaline phosphatase is raised, unlike in chronic myeloid leukaemia (CML) or acute myeloid leukaemia (AML) where it is lowered.
Infectious mononucleosis would have presented with signs of infection and is generally seen in younger patients, with lymphadenopathy, fever and sore throat. It would cause a***** leukocyte alkaline phosphatase, not elevated.
low
CKD is the most common cause of *** deficiency
antithrombin III
Antithrombin III inhibits several clotting factors, primarily thrombin, factor X and factor IX. It mediates the effects of heparin
Autosommal dominant - Antithrombin 3 defriciency
Beta Thalassemia
HbA2 levels are elevated in beta thalassaemia major and minor. It is a variant of haemoglobin A with two delta chains replacing the normal two beta chains. It is found in small amounts in healthy adults at around 1.5 - 3% of total haemoglobin. It is increased in beta thalassaemia because of reduced production of haemoglobin beta chains.
. This is the investigation of choice when an anaphylactic reaction is likely. Guidance is that levels should be taken at the time of the reaction, as soon as possible after emergency treatment has commenced, with a second level taken ideally within one to two hours (but no later than four).
Mast cell tryptase is incorrect
The occurrence of shortness of breath and headaches in conjunction with discordance between the peripheral oxygen saturation and pO2 of oxygen on the blood gas suggests
methaemoglobinaemia.
**is used in the treatment of lidocaine toxicity. Lidocaine toxicity is not likely in this case as this will typically present with neurological symptoms including numbness, tingling, vertigo and seizures.
Lipid emulsion therapy
Ascorbic acid is used in the management of congenital methaemoglobinaemia.
Dicobalt edetate and hydroxocobalamin are both treatments for cyanide poisoning
cyanide poisoning tends to cause raised peripheral oxygen saturations with low circulating pO2 along with a cherry-red hue on the skin.
Acquired causes
drugs: sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite ‘poppers’), dapsone, sodium nitroprusside, primaquine
chemicals: aniline dyes
Methaemoglobinemia
. This is a reversal agent for the anticoagulant agent dabigatran
Idarucizumab
. Antibiotic prophylaxis with***** during cyclophosphamide treatment is designed to prevent Pneumocystis jirovecii pneumonia.
Septrin
*** are sometimes taken in such patients as they remain elevated for up to 12 hours following an acute episode of anaphylaxis
Serum tryptase level
Low haptoglobin levels are found in haemolytic anaemias
is now considered first-line treatment ,inhibitor of the tyrosine kinase associated with the BCR-ABL defectvery high response rate in chronic phase CML
imatinib
Leukaemoid reaction
high leucocyte alkaline phosphatase score
toxic granulation (Dohle bodies) in the white cells
‘left shift’ of neutrophils i.e. three or fewer segments of the nucleus
t(8;14)
seen in Burkitt’s lymphoma
MYC oncogene is translocated to an immunoglobulin gene
Common variable immunodeficiency - low antibody levels, specifically in immunoglobulin types IgG, IgM and IgA. → recurrent chest and other infections
Bruton’s agammaglobulinaemia is a rare X-linked recessive immune disorder resulting in poor B-cell development. Whilst it also presents with recurrent infections and low immunoglobulins of all classes, it is much less common overall than CVID and mainly affects male patients due to the X-linked nature of its inheritance.
Wiskott-Aldrich syndrome, like SCID, also affects both T-cells and B-cells. As well as recurrent infections, it is also linked to eczema and thrombocytopaenia, it is X-linked recessive,disease
SCID, as the name implies, is a much more severe immunodeficiency than the presentation described here, affecting both T-cells and B-cells. The most common form is X-linked SCID, usually due to mutations in the gene coding the common gamma chain of multiple interleukin receptors. Patients are afflicted with significant infections from infancy and do not survive into childhood unless the condition is diagnosed early and treated with bone marrow transplantation
Thymoma is a/w
Associated with
myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH
B’ symptoms in Hodgkin’s lymphoma are associated with a poor prognosis
weight loss > 10% in last 6 months
fever > 38ºC
night sweats
Hereditary angioedema (HAE) is pathophysiologically separate from anaphylaxis and is treated differently.
Therapeutic options are: intravenous infusion of human C1-esterase inhibitor or subcutaneous injection of the bradykinin receptor inhibitor icatibant
NICE updated their guidelines on the investigation and management of venous thromboembolism (VTE) in 2020. Some of the key changes include recommending the following:
the use of direct oral anticoagulants (DOACs) as first-line treatment for most people with VTE, including as interim anticoagulants before a definite diagnosis is made
the use of DOACs in patients with active cancer, as opposed to low-molecular weight heparin as was the previous recommendation
routine cancer screening is no longer recommended following a VTE diagnosis
Disproportionate microcytic anaemia - think
beta-thalassaemia trait
Post-infectious haemolysis can occur with
atypical pneumonias such as Mycoplasma (cold-agglutinin disease) and infections that induce hypersplenism such as mononucleosis.
Pyruvate kinase deficiency is the next most common inherited metabolic disorder after G6PD deficiency.
Haemolysis in these patients tends to be triggered in times of significant physiological stress.
Some drugs causing haemolysis
(G6PD deficiency)
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
Rates of bacterial contamination of platelet transfusion are relatively speaking high, given that platelets are stored at between 20-24ºC. Risk of bacterial contamination of the platelet pool increases with increasing time ex vivo and is the main factor contributing to the short shelf life of platelets.
If no active bleeding or planned invasive procedure
A threshold of 10 x 109 except where platelet transfusion is contradindicated or there are alternative treatments for their condition
For example, do not perform platelet transfusion for any of the following conditions:
Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin-induced thrombocytopenia, or
Thrombotic thrombocytopenic purpura.
Combined B- and T-cell disorders:
SCID WAS ataxic (SCID, Wiskott-Aldrich syndrome, ataxic telangiectasia)
Polycythaemia rubra vera - around 5-15% progress to myelofibrosis or AML
AML varient acute promyelocytic leukaemia can present as DIC
Burkitt’s lymphoma - translocation
t(8:14)
The condition being described here is Burkitt’s lymphoma, given the demographic, positive Epstein-Barr virus and the ‘starry sky’ appearance on microscopy - lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells. Burkitt’s lymphoma is associated with the c-myc gene translocation, usually t(8:14).
Leucocyte alkaline phosphatase is low in CML but raised in myelofibrosis
HUS or TTP? Neuro signs point towards TTP
The combination of neurological features, renal failure, pyrexia and thrombocytopaenia point towards a diagnosis of thrombotic thrombocytopenic purpura
This condition is characterised by the triad of chronic complement-mediated haemolysis, thrombosis, and bone marrow failure.
paroxysmal nocturnal haemoglobinuria (PNH).
Eculizumab is a recombinant monoclonal antibody that inhibits terminal complement activation at the C5 protein and thereby reduces complement-mediated cell damage
Indications for splenectomy in HS include
severe symptomatic anaemia, transfusion dependence, or gallstone disease.
Itching and tingling of the lips, tongue and mouth are the most common symptoms of oral allergy syndrome
