Haematology Flashcards

1
Q

Iron Deficiency Anaemia

A

Causes

  • excessive blood loss ( young F: Menorrhagia, Adult M& PostMeno F: GI bleeding ?cancer)
  • inadequate dietary intake (vegan, vegetarian)
  • poor intestinal absorption (coeliac disease)
  • increased iron requirements. (Children rapid growth, pregnancy

Features

  • Fatigue
  • SOB
  • pallor & palpitations
  • hair loss
  • atrophic glossitis (tongue)
  • nail changes: spoon shaped (Koilonychia)
  • angular stomatitis

Ix

  • Good history (look for signs of cancer)
  • hypochromic microcytic anaemia
  • Total iron-binding capacity (TIBC)/transferrin will be high. (due to low iron stores)
  • anisopoikilocytosis (diff shapes of RBCs)

Management

  • malignancy has been excluded
  • Oral ferrous sulfate: patients should continue taking iron for 3 months after the iron deficiency has been corrected in order to replenish iron stores.
  • Iron-rich diet: this includes dark-green leafy vegetables, meat, iron-fortified bread

Side fx of Iron tabs: nausea, abdominal pain, constipation, diarrhoea

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2
Q

What type of anaemia is: Anaemia of Chronic Disease

A

normocytic anaemia

Others include

  • chronic kidney disease
  • aplastic anaemia
  • haemolytic anaemia
  • acute blood loss

anemia that is found in people with certain long-term (chronic) medical conditions that involve inflammation

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3
Q

Sideroblastic Anaemia

microcytic

A
  • Red cells fail to completely form haem
  • Leading to deposits of iron in the mitochondria that form a ring around the nucleus called a ring sideroblast

Ix

  • full blood count
  • hypochromic microcytic anaemia (more so in congenital)
  • iron studies
  • high ferritin
  • high iron
  • high transferrin saturation
  • blood film: basophilic stippling of red blood cells
  • bone marrow: Prussian blue staining will show ringed sideroblasts

Management

  • Management
  • supportive
  • treat any underlying cause
  • pyridoxine may help

Congenital or Acquired

Acquired causes
* myelodysplasia
* alcohol
* lead
* anti-TB medications

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4
Q

macrocytic

Vit B12 Deficiency

Cobalamin deficiency

A

Background

  • Used in the body for red blood cell development and also maintenance of the nervous system
  • absorbed after binding to intrinsic factor in terminal ileum

Causes

  • pernicious anaemia: most common cause
  • post gastrectomy or resection in crohns
  • vegan diet or a poor diet
  • Metformin

Signs and Ix

  • macrocytic anaemia
  • sore tongue and mouth
  • Distal parasthesia

Management

  • 1 mg of IM hydroxocobalamin 3 times each week for 2 weeks then x1 every 3m
  • Treat B12 before treatinf B9
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5
Q

macrocytic

Vit B9 Deficiency

Folate Deficiency

A
  • Green, leafy vegetables are a good source of folic acid.

Causes

  • phenytoin
  • methotrexate
  • pregnancy
  • alcohol excess

Management

  • all women should take 400mcg of folic acid until the 12th week of pregnancy
  • women at higher risk of conceiving a child with a NTD should take 5mg of folic acid from before conception until the 12th week of pregnancy

High risk = prev history of neural tube defect (NTD) in either partner family, mother on antieplileptics, mother obese

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6
Q

Autosomal Recessive

Sickle Cell Anaemia

heterozygous condition offers some protection against malaria.

Heterozygotes dont show symptoms unless crisis
Homozygotes show symptoms after 4-6m, abnormal HbSS molecules take over from fetal haemoglobin.

A
  • synthesis of an abnormal haemoglobin chain termed HbS forming crescent shaped RBCs

Types

  • normal haemoglobin: HbAA
  • sickle cell trait: HbAS
  • homozygous sickle cell disease: HbSS.
  • milder form of sickle cell disease (HbSC) - C is deformed Hb

Ix & mngement

  • definitive diagnosis of sickle cell disease is by haemoglobin electrophoresries
  • hydroxyurea - prophylaxis
  • Pneumococcal vaxx every 5 years

Sickle Cell crisis

  • Thrombotic, ‘vaso-occlusive’, ‘painful crises’ - caused by infection, Dehydration, high altitudes, Diagnosed clinically
  • Acute chest syndrome - dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, low pO2
  • Aplastic crises - infection with parvovirus, sudden fall in haemoglobin + reticulocytes
  • Sequestration crises - sickling in organs like spleen, increased reticulocyte count

Management Crisis

  • analgesia e.g. opiates
  • rehydrate
  • oxygen
  • consider antibiotics if evidence of infection
  • blood transfusion
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7
Q

Thalassaemia

Alpha, Beta Major, Beta minor, Intermedia

Thalassaemia is caused by a genetic defect in the protein chains that make up haemoglobin. Normal haemoglobin consists of two alpha-globin and two beta-globin chains.

A

Types

  • Alpha: 2 separate alpha-globulin genes are located on each chromosome 16
  • Beta Major: absence of beta globulin chains
  • Beta Minor: one abnormal and one normal gene.
  • Beta Intermedia: two abnormal copies of the beta-globin gene.

Features

  • Fatigue
  • Pallor
  • Jaundice
  • Gallstones
  • Splenomegaly
  • Poor growth and development

Ix

  • Microcytic anaemia (Low MCV)
  • Haemoglobin electrophoresis is used to diagnose globin abnormalities.
  • DNA testing can be used to look for the genetic abnormality.
  • All pregnant women offered screening
  • B Major: HbA2 & HbF raised
  • B Minor: microcytosis is characteristically disproportionate to the anaemia

Management

  • B Major: repeated transfusion + iron chelation therapy (desferrioxamine)
  • Alpha: monitor,Blood transfusions, maybe bone marrow
  • B minor: Monitoring
  • Intermedia: Monitoring but may need transfusion
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8
Q

G6PD Deficiency

A
  • Heinz bodies on blood films. Bite and blister cells may also be seen
  • G6PD enzyme assay - diagnostic
    levels should be checked around 3 months after an acute episode of hemolysis
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9
Q

Hereditary Spherocytosis

most common hereditary haemolytic anaemia in people of northern European descent

A

Presentation

  • failure to thrive
  • jaundice, gallstones
  • splenomegaly
  • aplastic crisis precipitated by parvovirus infection

Ix

  • Clinical diagnosis with bloods

Management

  • treatment is generally supportive
  • transfusion if necessary
    longer term treatment:
  • folate replacement
  • splenectomy
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10
Q

Multiple Myeloma

CRAB

haematological malignancy characterised by plasma cell proliferation

A
  • Genetic mutations -> B-lymphocytes differentiate into mature plasma cells.
  • Median age 70

Features

  • C: Hypercalcaemia
  • R: Renal Failure (light chain deposition within the renal tubules)
  • A: Anaemia
  • B: Bleeding (thrombocytopenia), Bony Pain
  • I: Infections

Ix

  • Anaemia + renal failure on U&E
  • peripheral blood film: rouleaux formation
  • Diagnostic: Bone marrow aspiration: plasma cells is significantly raised
  • whole-body MRI: For bony leisions

Management
Chemo
* Bortezomib (a proteasome inhibitor)
* Thalidomide
* Dexamethasone

RF:

  • Older age
  • Male
  • Black ethnic origin
  • Family history
  • Obesity
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11
Q

MGUS

Monoclonal gammopathy of undetermined significance

Around 10% of patients eventually develop myeloma at 10 years, with 50% at 15 years

A
  • Causes a paraproteinaemia* and is often mistaken for myeloma.

Feautures
* Asymptomatic
* Demyelinating neuropathy in some
* Increased risk of Infection

MGUS VS Myeloma

  • normal immune function
  • normal beta-2 microglobulin levels
  • lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA)
  • stable level of paraproteinaemia
  • no clinical features of myeloma (e.g. lytic lesions on x-rays or renal disease)

*Excessive amounts of paraproteins in the blood. Paraproteins are immunoglobulin proteins that are produced by a clone of plasma cells in the bone marrow.

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12
Q

rapidly progressing cancer of the myeloid cell line

Acute Myeloid Leukaemia

most prevalent acute leukaemia in adult population

can be the result of a transformation from a myeloproliferative disorder, such as polycythaemia ruby vera or myelofibrosis.

A

Feautures

  • Fatigue
  • Fever
  • Pallor due to anaemia
  • Petechiae or bruising due to thrombocytopenia
  • Abnormal bleeding
  • Lymphadenopathy

Ix

  • blood film and bone marrow biopsy - Blast cells & Auer rods

Management

  • chemo
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13
Q

Acute Lymphocytic Leukemia

most common in children

affects one of the lymphocyte precursor cells, causing acute proliferation of a single type of lymphocyte, usually B-lymphocytes. Excessive accumulation of these cells replaces the other cell types in the bone marrow, leading to pancytopenia.

A

common with Down’s syndrome.

Poor Prognosis

  • age < 2 years or > 10 years
  • WBC > 20 * 109/l at diagnosis
  • T or B cell surface markers
  • non-Caucasian
  • male sex
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14
Q

Chronic Myeloid Leukemia

CML has three phases, including a long chronic phase, and is associated with the Philadelphia chromosome

A
  • Philadelphia chromosome - BCR ABL1
  • an increase in granulocytes at different stages of maturation +/- thrombocytosis
  • management: IMATINIB - inhibitor of the tyrosine kinase

Chronic phase
Accelerated phase
Blast phase

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15
Q

Chronic Lymphocytic Leukemia

Most common form in adults

Leukaemia is cancer of a particular line of stem cells in the bone marrow, causing unregulated production of a specific type of blood cell.

A
  • where there is slow proliferation of a single type of well-differentiated lymphocyte, usually B-lymphocytes. It usually affects adults over 60 years of age.
  • warm autoimmune haemolytic anaemia
  • blood film: **smudge cells **(also known as smear cells)
  • Ritcher’s transformation occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma. Patients often become unwell very suddenly.
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16
Q

Hodgkin’s Lymphoma

A
  • Malignant proliferation of lymphocytes accumulating in lymph nodes

Features

  • Painless Lymphadenopathy
  • B symptoms ( Weight loss, Pruitis, Night sweats, fever)

Ix

  • Normocytic anaemia
  • Raised Eosinophils
  • Lymph node biopsy: Reed Sternberg cells

Management

  • ABVD ( doxorubicin, bleomycin, vinblastine, and dacarbazine)
  • Or BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone)

Having B Symptoms is poor prognosis

17
Q

Non-Hodgkin Lymphoma

More common than Hodgkins

A
  • Malignant proliferation of lymphocytes accumulating in lymph nodes
  • can affect B or T cells
  • RF: elderly, previous infection of EBV or Hiv, SLE

Features

  • Painless Lymphadenopathy
  • B symptoms ( Weight loss, Pruitis, Night sweats, fever)

Ix

  • Biopsy mainstay example: Burkitt’s Lymphoma: Starry Sky appearance

Management

  • Rituximab
  • CHOP
18
Q

Pancytopenia

AKA Aplastic Anaemia

A

Pancytopenia is a medical condition characterized by a deficiency of all three major types of blood cells: red blood cells (erythrocytes), white blood cells (leukocytes), and platelets (thrombocytes). It can result from various underlying causes, including bone marrow disorders, nutritional deficiencies, autoimmune diseases, infections, medications, and certain cancers.

Features

  • normochromic, normocytic anaemia
  • leukopenia, with lymphocytes relatively spared
  • thrombocytopenia
  • may be the presenting feature acute lymphoblastic or myeloid leukaemia
  • a minority of patients later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia

Causes

  • idiopathic
  • congenital: Fanconi anaemia, dyskeratosis congenita
  • drugs: cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
  • toxins: benzene
  • infections: parvovirus, hepatitis
  • radiation
19
Q

Polycythemia Vera

myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets.

A

Features

  • pruritus, typically after a hot bath
  • splenomegaly
  • hypertension
  • hyperviscosity

Ix

  • JAK2 gene in 95%
  • full blood count/film: raised haematocrit; neutrophils, basophils, platelets

Management

  • aspirin: reduces the risk of thrombotic events
  • venesection: first-line treatment to keep the haemoglobin in the normal range
  • chemotherapy

Secondary Causes

  • COPD
  • altitude
  • obstructive sleep apnoea
  • excessive erythropoietin: cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids*
20
Q

Thrombocytopenia

A
21
Q

Immune Thrombocytopenic purpura

Immune (or idiopathic) thrombocytopenic purpura (ITP) is an immune-mediated reduction in the platelet count.

A
  • Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex.
  • Elderly females most affected
  • petechiae, purpura, bleeding (e.g. epistaxis)
  • full blood count: isolated thrombocytopenia
  • Management: Oral Prednislone
    *

Evan’s syndrome
ITP in association with autoimmune haemolytic anaemia (AIHA)

22
Q

Thrombotic Thrombocytopenic purpura

A
  • abnormally large and sticky multimers of von Willebrand’s factor cause platelets to clump within vessels
  • a deficiency of ADAMTS13

Features

  • fever
  • fluctuating neuro signs (microemboli)
  • microangiopathic haemolytic anaemia
  • thrombocytopenia
  • renal failure

Causes

  • post-infection e.g. urinary, gastrointestinal
  • pregnancy
  • drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir
  • tumours
  • SLE
  • HIV
23
Q

Autosomal dominant

Von Willebrand Disease

most common inherited bleeding disorder

Role of von Willebrand factor

  • large glycoprotein which forms massive multimers up to 1,000,000 Da in size
  • promotes platelet adhesion to damaged endothelium
  • carrier molecule for factor VIII
A

Ix

  • prolonged bleeding time
  • APTT may be prolonged
  • factor VIII levels may be moderately reduced

Management

  • 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
24
Q

Haemophilia A

Haemophilia is an X-linked recessive disorder of coagulation. Up to 30% of patients have no family history of the condition.

A
  • Haemophilia A is due to a deficiency of factor VIII (8)
  • haemoarthroses (blood in Joints)
  • haematomas
  • prolonged bleeding after surgery or trauma
  • Prolonged APTT
  • Diagnosis is based on bleeding scores, coagulation factor assays and genetic testing.

Management

  • The affected clotting factor (VIII) can be given by intravenous infusion, either regularly or in response to bleeding.
  • Up to 10-15% of patients with haemophilia A develop antibodies to factor VIII treatment.
25
Q

Haemophilia B

Haemophilia is an X-linked recessive disorder of coagulation. Up to 30% of patients have no family history of the condition.

A
  • Haemophilia B (Christmas disease) there is a lack of factor IX (9)
  • haemoarthroses (blood in Joints)
  • haematomas
  • prolonged bleeding after surgery or trauma
  • Prolonged APTT
  • Diagnosis is based on bleeding scores, coagulation factor assays and genetic testing.

Management

  • The affected clotting factor (IX) can be given by intravenous infusion, either regularly or in response to bleeding.
26
Q

Vitamin K Deficiency

A

Increase in: APTT, PT, Bleeding time

Treated with Vit K supplements

  • Vitamin K deficiency can contribute to significant bleeding, poor bone development, osteoporosis, and increased cardiovascular disease.
27
Q

Factor V Leiden

activated protein C resistance

mis-sense mutation

A
  • most common inherited thrombophilia
  • mutation in the Factor V Leiden protein.
  • Activated factor V (a clotting factor) is inactivated 10 times more slowly by activated protein C than normal - causing clots to form

Increased risk of clots

28
Q

Myelodysplastic Syndrome

MDS arises from genetic mutations in hematopoietic stem cells.

A

treat with blood transfusion

29
Q

Leucopenia

A

Leucopenia describes a decreased number of white cells.

30
Q

Malaria

Plasmodium falciparum
Plasmodium vivax
Plasmodium ovale
Plasmodium malariae

A

schizonts on a blood film

31
Q

Neutropenic Sepsis

occurs 7-14 days after chemotherapy

A
  • Complication of Cancer Chemo therapy
  • Low Neutrophil count + temp + other signs of sepsis
  • Cause: Staphylococcus epidermidis
  • Tx: piperacillin with tazobactam (Tazocin) immediately
32
Q

DVT risk in pregnancy

Preganancy is hypercoaguble state

A
  • Risk higher in last trimester

Pathophysiology
* increase in factors VII, VIII, X and fibrinogen
* decrease in protein S
* uterus presses on IVC causing venous stasis in legs

Tx
* warfarin contraindicated
* LMWH Subcut preferred over IV

33
Q

RF of developing VTE

A

General
* increased risk with advancing age
* obesity
* family history of VTE
* pregnancy (especially puerperium)
* immobility
* hospitalisation
* anaesthesia
* central venous catheter: femoral&raquo_space; subclavian

Underlying conditions
* malignancy
* thrombophilia: e.g. Activated protein C resistance, protein C and S deficiency
* heart failure
* antiphospholipid syndrome
* Behcet’s
* polycythaemia
* nephrotic syndrome
* sickle cell disease
* paroxysmal nocturnal haemoglobinuria
* hyperviscosity syndrome
* homocystinuria

Medication
* combined oral contraceptive pill: 3rd generation more than 2nd generation
* hormone replacement therapy: the risk of VTE is higher in women taking oestrogen + progestogen preparations compared to those taking oestrogen-only preparations
* raloxifene and tamoxifen
* antipsychotics (especially olanzapine) have recently been shown to be a risk factor

34
Q

Treating DVT

A
  • Apixaban 1st line
  • if contraindicated: LMWH followed by dabigatran or edoxaban OR LMWH followed by a vitamin K antagonist (warfarin)
  • if renal impairment is severe (e.g. < 15/min) then LMWH, unfractionated heparin

Length of Treatment

  • 3m for all pts
  • Provoked: 3m
  • Unprovoked: 6m

a provoked VTE is due to an obvious precipitating event e.g. immobilisation following major surgery. The implication is that this event was transient and the patient is no longer at increased risk
an unprovoked VTE occurs in the absence of an obvious precipitating event, i.e. there is a possibility that there are unknown factors (e.g. mild thrombophilia) making the patient more at risk from further clots

35
Q

Micro v Normo v Macro cytic anaemias

A