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Year 3 - Haematology > Week 3 > Flashcards

Week 3 Flashcards

1
Q

What is the lifespan of a neutrophil?

A

7-8 hours

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2
Q

What key factor separates haemopoietic stem cells from myeloid and lymphoid progenitor cells?

A

Haemopoietic stem cells are able to self-renew

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3
Q

What are the main sites of haemopoiesis at each of the following ages?

  • conception
  • week 6
  • week 16
  • adults
A

Conception - yolk sac

Week 6 - liver

Week 16 - bone marrow

Adults - marrow WITHIN THE AXIAL SKELETON, PELVIS AND PROXIMAL LONG BONES

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4
Q

Describe the bone marrow ‘sinus’

A

Arterioles within the bone drain into ‘sinuses’ - wide venous vessels which open into larger central sinuses

Importantly, sinuses have a discontinuous basement membrane and the presence of adventitial cells adjacent to these sinuses (which contain smooth muscle filaments) allow for the sinuses to open up upon contraction

This allows the release of mature red cells from the marrow into the circulation

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5
Q

What is the difference between red and yellow marrow?

A

Red marrow - haemopoietically active

Yellow - fatty and inactive.

At birth, all bone marrow is red. Amount of yellow marrow increases with age, resulting in a reduction in marrow cellularity in older individuals. Middle aged adults have about 50% red and 50% yellow marrow

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6
Q

What is the myeloid:erythroid ratio?

What might cause this ratio to increase?

A

M:E ratio is the relationship of neutrophils and precursors to the proportion of nucleated red cell precursors

M:E ratio is increased in anaemia (e.g. due to blood loss) and in haemolysis (basically anything that causes loss of red cells)

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7
Q

What molecule regulates neutrophil precursor maturation?

A

Granulocyte-colony stimulating factor (G-CSF)

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8
Q

What molecule regulates platelet growth and development (via megakaryocytes from their precursors)?

A

Thrombopoietin

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9
Q

When assessing haemopoeisis and examining blood, how are the following assessed?

  • non-lymphoid cells
  • lymphoid cells
A

Non-lymphoid - blood count/morphology is usually sufficient

Lymphoid - cannot be distinguished from one another by morphology (except plasma cells!), so antigen expression is studied via immunophenotyping

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10
Q

Where do B cells mature?

Where do T cells mature?

A

B cells - bone marrow

T cells - thymus

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11
Q

Which of the following are primary lymphoid tissues and which are secondary?

  • Bone marrow
  • Lymph nodes
  • Spleen
  • Thymus
  • Tonsils (Waldeyer’s ring)
  • Epithelio-lymphoid tissues
A

Primary

  • Bone marrow
  • Thymus

Secondary

  • Lymph nodes
  • Spleen
  • Tonsils
  • Epithelio-lymphoid tissues
  • Bone marrow
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12
Q

What complications could occur in the event of…

  • surgical removal of lymph nodes
  • damage to the central lymphatics system (cisterna chyli) e.g. trauma/obstruction
A

Surgical removal - Lymphoedema

Damage to central lymphatics - Chylous ascites

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13
Q

What marker can be used to identify…

  • B cells
  • T cells
A

B cells - CD20

T cells - CD3

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14
Q

After stimulation of an immune reaction within a lymph node, what might the following responses indicate?

  • Predominant B cell response
  • Predominant phagocytic response
  • Predominant T cell response
A

Predominant B cell response - autoimmune conditions or infection

Predominant phagocytic response - draining a tumour site

Predominant T cell response - viral infections or certain drugs e.g. phenytoin

*NB - this is just a general guide and not entirely specific

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15
Q

Is a diseased spleen more or less likely to rupture? Is this serious?

A

More likely, and yes it is serious! Surgical emergency due to being a very vascular organ, patients can bleed out very quickly

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16
Q

Describe the ‘red pulp’ and ‘white pulp’ of the spleen

A

Red pulp - contains sinusoids and cords

  • Sinusoids are fenestrated, lined by endothelial cells and supported by hoops of reticulin
  • Cords contain macrophages, some fibroblasts and cells in transit (RBCs, WBCs etc.)

White pulp - comprises the peri-arteriolar lymphoid sheath (PALS)

  • PALS is expanded by lymphoid follicles and may show reactive change
  • APCs in the white pulp present antigen to immune reactive cells, and T and B cell responses may occur as a result.
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17
Q

What is the triad of components that makes up hypersplenism (overactive spleen)?

A
  1. splenomegaly
  2. fall in one or more cellular components of blood
  3. correction of cytopenias by splenectomy
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18
Q

Name some conditions that may result in hyposplenism

A

Coeliac disease

Sickle cell disease

Sarcoidosis

Iatrogenic

Chronic folate deficiency

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19
Q

What histological sign might indicate a damaged or absent spleen?

A

Howell-Jolly bodies - basophilic nuclear remnants in erythrocytes that would usually be removed by a healthy spleen

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20
Q

What scoring system is used in patients with AF to determine their risk of stroke and need for anticoagulation?

What are the components of this scoring system and a score above what would indicate the need for treatment?

A

CHA2DS2VASc score

  • Congestive heart failure (1)
  • Hypertension (1)
  • Age equal to or greater than 75 (2)
  • Diabetes mellitus (1)
  • Prior stroke, TIA or thromboembolism (2)
  • Vascular disease e.g. PAD, MI, aortic plaque etc. (1)
  • Age 65-74 (1)
  • Sc, female sex (1)

Score of 2 and above indicates recommendation for treatment

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21
Q

When advising a patient on anticoagulation options, why might it be beneficial for a clinician if the patient opts for Warfarin?

A

Warfarin requires regular monitoring for INR - allows clinician to check for compliance

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22
Q

What does the mneumonic ‘CRAB’ stand for in relation to myeloma symptoms?

A

Calcium (raised)

Renal function (decline)

Anaemia

Bones (pain)

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23
Q

What are the causes of autoimmune haemolytic anaemia?

Which antibodies are implicated?

A

Warm antibody Haemolytic Anaemia (IgG, mainly)

Cold antibody Haemolytic Anaemia, aka Cold Agglutinin Disease (IgM, mainly)

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24
Q

What molecule is present on the cell surface of stem cells and can be used to identify them during immunophenotyping?

A

CD34

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25
Q

How does the mechanism of disease differ between acute and chronic leukaemias?

A

Acute leukaemias - proliferation of abnormal progenitors with a block in differentiation/maturation. Lots of cells that look similar

Chronic leukaemias - proliferation of abnormal progenitors but NO differentiation/maturation block. Increased number of cells but they all look different

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26
Q

One of the ways in which haematological malignancies develop is through somatic mutations in regulatory genes i.e. driver mutations vs passenger mutations. How do driver mutations influence ‘clones’ in haemopoiesis?

A

Clone: population of cells derived from a single parental cell. Parent cells have a genetic marker that is shared by daughter cells.

Normally, clones can diversify but will contain a similar genetic backbone.

Driver mutations can select clones, meaning that malignant haemopoiesis is usually MONOCLONAL, as opposed to normal haemopoiesis which is polyclonal

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27
Q

Why are driver mutations selected by cells during the evolution of cancer?

A

Because they confer growth advantages

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28
Q

How can haematological malignancies be classified?

A
  1. based on lineage e.g. myeloid or lymphoid
  2. based on developmental stage (precursor) within lineage e.g. acute lymphoblastic (problem with progenitor cell) vs chronic lymphocytic (problem with daughter cell)
  3. based on the anatomical site involved e.g. myeloma (plasma malignancy in marrow), lymphoma (lymph nodes), leukaemia (blood involvement)
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29
Q

Which of the following are included in the sites of acute lymphoblastic leukaemia?

  • blood
  • bone marrow
  • lymph nodes
A

Blood and bone marrow

Lymph nodes are NOT included in acute leukaemias

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30
Q

How do high-grade lymphomas and acute leukaemias compare to low-grade lymphomas and chronic leukaemias in terms of aggression?

What are some of the characteristic histological signs of a more aggressive malignancy?

A

High-grade lymphomas and acute leukaemias are MORE aggressive

Characteristic features include…

  • large cells
  • high nuclear-cytoplasmic ratio
  • prominent nucleoli due to increased RNA and protein
  • open chromatin
  • rapid proliferation
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31
Q

What cell type is affected in myeloma?

A

Plasma cells in the bone marrow

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32
Q

How is acute leukaemia defined and what are the two main types?

A

Rapidly progressive clonal malignancy of the marrow/blood with maturation defects. Results in an increased number of blasts in either the peripheral blood or bone marrow

There is also always a decrease in the normal haemopoietic reserve

Two types: Acute Myeloid Leukaemia (AML) and Acute Lymphoblastic Leukaemia (ALL)

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33
Q

Which age groups are most commonly affected by a) AML and b) ALL?

A

ALL - most common childhood cancer

AML - most common in the elderly (>60 years)

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34
Q

How does ALL present?

A

Disease of primitive (lymphoblastic) progenitor cells

Most commonly presents in children

Symptoms are…

  • effects of marrow failure - anaemia, infections, bleeding
  • leukaemic effects - high count with obstruction of cells, meaning involvement of areas outside the marrow and blood e.g. CNS, testis etc.
  • Bone pain
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35
Q

How does AML present?

A

More common in elderly patients

Similar marrow failure presentation to that seen in ALL (anaemia, infections, bleeding etc.)

Subgroups may have characteristic presentation…

  • DIC

- gum infiltration

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36
Q

Acute leukaemia - investigations

A
  1. FBC followed by blood film (looking at cell morphology and presence of primitive cells) - there will be a reduction in normal and presence of abnormal
  2. Coagulation screen
  3. Bone marrow aspiration - morphology and immunophenotyping
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37
Q

What telltale histological sign may be seen on blood film that allows differentiation between AML and ALL?

A

Auer rods are seen in acute myeloid leukaemia

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38
Q

Morphologically, bone marrow aspiration samples of AML and ALL may look identical. How else could they be separated?

A

Using immunophenotyping via flow cytometry

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39
Q

How does treatment of ALL compare to AML?

A

ALL - can last up to 2-3 years with different phases of varying intensity

AML - normally intensive with 2-4 cycles of chemo (5-10 days followed by 2-4 weeks recovery). Requires prolonged hospitalisation

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40
Q

What is one of the main complications to be aware of regarding bone marrow suppression in the treatment of acute leukaemias?

A

Neutropenia meaning increased severity and duration of infections

This could also result in gram-NEGATIVE bacteria causing fulminant life-threatening sepsis

Also anaemia and thrombocytopenia (causing bleeding in the form of pupura and petechiae)

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41
Q

What are some of the complications and side effects associated with chemotherapy?

A

Nausea and vomiting

Hair loss

Liver and renal dysfunction

Tumour lysis syndrome (during the first course of treatment)

Infection - bacterial, fungal and protozoan

Later effectss - loss of fertility, cardiomyopathy associated with anthracyclines

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42
Q

What should be done as soon as a patient undergoing chemotherapy develops neutropenic fever?

A

Begin empirical treatment with broad spec. antibiotics - especially covering gram negative bacteria.

Begin the treatment quickly and don’t wait for results to come back before commencing

43
Q

What is the prognosis for children being treated for ALL?

How about adults with AML?

A

Children with ALL - Very good! >85-90% cure rates

Adults with AML - Not as good… if under 60, 40-50%, if over 60 then 10% or less

44
Q

What are B symptoms?

A

Associated with lymphoma

- PAINLESS LYMPHADENOPATHY

- Night sweats

- Weight loss

- Fever

45
Q

What are some of the things on your list of differentials when you see a patient with lymphadenopathy?

A

‘Reactive’ lymphadenopathy

Bacterial infection causing regional swelling

Viral infection causing generalised swelling

Metastatic malignancy

Lymphoma

46
Q

Should you be worried/what is the likely diagnosis?

Non-tender nodes, rubbery/soft texture, smooth surface, no skin inflammation and not tethered

A

Yes - presentation is that of lymphoma

47
Q

Should you be worried/what is the likely diagnosis?

Non-tender nodes, hard, irregular surface, no skin inflammation and tethered to underlying structures

A

Yes - presentation is that of metastatic carcinoma

48
Q

Can CT be used to diagnose lymphoma?

A

No - can’t differentiate between lymphoma and metastatic disease

49
Q

What investigations can be used to investigate lymph node pathology?

A

Histological appearance

Immunohistochemistry (looking at expressed proteins), done on solid sample

Immunophenotyping, similar to the above but uses a fluid blood/marrow sample

Genetic analysis

Molecular analysis

50
Q

What pathology are Reed-Sternberg cells indicative of?

A

Hodgkin’s Disease

RS cells cause inflammatory response and resulting nodular sclerosis

51
Q

What CD molecule is expressed on B cells and would indicate the presence of a non-Hodgkin’s lymphoma when performing immunohistochemistry?

A

CD20

52
Q

What CD molecule is seen on Reed-Sternberg cells in Hodgkin’s Lymphoma?

A

CD30

53
Q

What lab test is useful in determining the pattern of CD numbers in cells, and as such is useful in identifying various leukaemias and lymphomas involving the marrow?

How does this investigation work?

A

Immunophenotyping

Cells are tagged with antibodies and emit a specific colour of light when a laser is shone on them

Useful for identifying Burkitt’s Lymphoma (t 8:14)

54
Q

Regarding treatment aims, how do Hodgkin’s Lymphoma/High grade NHL and Low grade NHL differ?

A

Hodgkin’s Lymphoma/High grade NHL - can be cured

Low grade NHL - cannot be cured, aim is to make the disease indolent

55
Q

How does the treatment for Burkitt’s lymphoma differ from standard chemo given in other forms of lymphoma?

A

Burkitt’s is very aggressive and requires specialised treatment

Chemo is given every day for several days, rather than having breaks

56
Q

Non-Hodgkin’s Lymphoma can be classified into B cell and T cell types. What are some examples of each?

Which is most numerous in practice?

A

B-NHL

  • precursor B-ALL
  • B-ALL, lymphoblastic NHL
  • Burkitt’s
  • DLCL
  • Mantle cell lymphoma
  • Follicular lymphoma
  • MALToma

T-NHL

  • precursor T-ALL, lymphoblastic lymphoma
  • T-PLL
  • T-cell LGL
  • ATLL
  • CTCL
  • ……..

In practice, B cell NHLs are by far the most common (both low and high grade)

57
Q

What is pancytopenia?

A

Deficiency of blood cells of all lineages

Importantly, it is not a diagnosis and doesn’t always mean bone marrow failure

58
Q

Generally, causes of pancytopenia can be broken down into increased destruction or reduced production.

What can reduced production further be broken down into?

A

Inherited syndromes (incredibly rare)

Acquired (either primary or secondary)

59
Q

Give an example of an inherited syndrome of marrow failure.

How does this condition present?

A

Fanconi’s anaemia - inherited cause of reduced production of all cells

Short stature

Skin pigment abnormalities (cafe au lait spots)

Hypogenitalia

Endocrinopathies

GI,cardiovascular, renal and haematological defects

60
Q

What associated haematological abnormalities are seen in someone with Fanconi’s anaemia?

A

Bone marrow failure (aplasia) - 84% of patients by 20 years

Leukaemia - 52% of patients by 40 years

61
Q

What is the main mechanism of pathophysiology in Fanconi’s anaemia?

A

Inability to correct inter-strand cross-links (DNA damage)

62
Q

What are some examples of acquired primary bone marrow failure?

A

Idiopathic aplastic anaemia

Myelodysplastic syndromes (MDS)

Acute leukaemia

63
Q

What is the (believed) mechanism behind idiopathic aplastic anaemia?

A

Autoimmune attack against haematopoietic stem cells - INF-gamma and TNF-alpha damage the stem cell compartment, especially the myeloid branch

64
Q

Myelodysplatic syndromes are a form of acquired primary bone marrow failure and subsequent pancytopenia.

What are the key features of myelodysplastic syndromes? What does it have a propensity to evolve into?

A

Dysplasia (disordered development of blood cells)

Hypercellular marrow + increased apoptosis of progenitor and mature cells = ineffective haemopoiesis

Propensity for evolution into AML

65
Q

How does acute leukaemia cause pancytopenia?

A
  1. Proliferation of abnormal cells (blasts) from leukaemia stem cells > failure to differentiate or mature into normal cells
  2. Prevention of normal haemopoietic stem/progenitor cells development by “hijacking” the haemopoeitic niche and marrow microenvironment
66
Q

What are the clinical features of pancytopenia?

A

Anaemia - fatigue, SoB, cardiovascular issues

Neutropenia - infections with increased severity and duration

Thrombocytopenia - bleeding (petechiae and purpura)

67
Q

In a patient with pancytopenia, if a marrow biopsy was done what would the following suggest?

  • hypocellularity
  • hypercellularity
A

Hypocellular = aplastic anaemia

Hypercellular = MDS, B12/Folate deficiency, hypersplenism

68
Q

What investigations would you perform in a patient presenting with pancytopenia?

A

History, including family, as well as clinical exam

FBC and blood film

B12/Folate, LFTs, virology, autoantibody tests

Bone marrow examination (aspiration of trephine biopsy)

69
Q

What is the shape of the following immunoglobulins?

  • IgG
  • IgE
  • IgD
  • IgA
  • IgM
A

IgG, IgE and IgD = monomer

IgA = dimer

IgM = pentamer

70
Q

What are paraproteins?

What are they a marker of?

A

Monoclonal immunoglobulins all derived from clonal expansion of a single B-cell

Have identical structure and specificity (size and charge)

Marker of an underlying CLONAL B cell disorder

71
Q

Serum electrophoresis can be used to detect immunoglobulins and abnormal proteins, with separated serum proteins appearing as distinct bands.

Within which region to all immunoglobulins appear?

How does this region appear normally (i.e. polyclonally)?

A

Immunoglobulins appear within the gamma region

This is a broad region that, if polyclonal, shows a diffuse stain with no specific areas of banding

72
Q

What procedure is then done following serum electrophoresis to classify the abnormal bands?

A

Immunofixation

73
Q

What is a Bence Jones protein and what condition(s) might its appearance suggest?

A

BJ proteins are excess immunoglobulin light chains that are detected in the urine with electrophoresis

Their presence is particularly associated with Multiple myeloma (and also Waldenstrom’s macroglobulinaemia)

74
Q

What is the most common cause of paraproteinaemia?

A

MGUS (monoclonal gammopathy of undetermined significance)

75
Q

Describe the progression of development from normal plasma cells to myeloma

A

Normal plasma cells pick up a “genetic hit” and MGUS develops (benign/pre-malignant)

MGUS picks up more genetic hits and becomes Asymptomatic myeloma (malignant, but with no organ damage)

Asymptomatic myeloma picks up even more genetic hits and develops into overly malignant myeloma

76
Q

Myeloma affects the body through both direct tumour cell effects and paraprotein-mediated effects. What are some of the direct tumour cell effects?

A

Bone lesions

Increased calcium

Bone pain

Replacement of normal bone marrow and subsequent marrow failure

77
Q

Myeloma affects the body through both direct tumour cell effects and paraprotein-mediated effects. What are some of the paraprotein-mediated effects?

A

Renal failure

Immune suppression

Hyperviscosity

Amyloid accumulation

78
Q

Myeloma is classified by the type of antibody produced. Which is the most commonly produced antibody?

A

IgG (59%)

79
Q

What are some of the clinical signs and symptoms of hypercalcaemia?

A

Stones (kidney)

Bones

Abdominal groans (N+V, constipation, indigestion)

Psychiatric moans (lethargy, fatigue, memory loss, depression)

Thirst

Dehydration

Renal impairment

80
Q

What major organ in the body is commonly affected in patients with myeloma?

How has it become damaged?

A

The kidneys - 30% of patients present with renal impairment at diagnosis

Causes

  • Tubular cell damage by light chains
  • Light chain deposition, “cast nephropathy”
  • Sepsis
  • Hypercalcaemia and dehydration
  • Drugs, NSAIDs
  • Hyperuricaemia
  • Amyloid deposition
81
Q

How is myeloma treated and what is used to measure response?

A

Combination chemo is the mainstay of treatment (+symptom control)

  • Corticosteroids: dexamethasone or prednisolone
  • Alkylating agents: cyclophosphamide, melphalan
  • ‘Novel’ agents: thalidomide, bortezomib, lenalidomide
  • High dose chemo/autologous stem cell transplant in patients that are fit enough

Paraprotein levels are used to monitor response to treatment

82
Q

What is MGUS (monoclonal gammopathy of undetermined significance)?

A

“Everything that myeloma is not”

Paraprotein is present, but in small amounts (<30 g/l)

Bone marrow plasma cells are <10%

No evidence of myeloma/organ damage

  • normal Ca
  • normal renal function
  • normal Hb
  • no lytic lesions
83
Q

What antibody is associated with Waldenstrom’s macroglobulinaemia?

How does this condition present?

A

IgM paraprotein is seen

Tumour Effects

  • Lymphadenopathy
  • Splenomegaly
  • Marrow failure

Paraprotein Effects

  • Hyperviscosity - fatigue, visual disturbance, confusion, coma
  • Neuropathy

B symptoms - night sweats, weight loss

84
Q

Myeloproliferative disorders and acute leukaemia are both clonal haematopoietic stem cell disorders with an increased production of one or more types of haemopoietic cells. How do MPD and acute leukaemia differ?

A

In contrast to acute leukaemia, maturation is relatively preserved in MPDs

Cells in acute leukaemia are monomorphic, while cells in MPDs will appear at various stages of maturation i.e. not monomorphic

85
Q

MPDs can be classified into one of two subtypes based on whether they are BCR-ABL1 positive or BCR-ABL1 negative

Provide examples of both

A

BCR-ABL1 positive

  • Chronic Myeloid Leukaemia (CML) - overproduction of granulocytes, associated with the Philadelphia chromosome

BCR-ABL1 negative

  • Polycythaemia Rubra Vera - overproduction of red cells
  • Idiopathic Myelofibrosis
  • Essential Thrombocythaemia - overproduction of platelets
86
Q

What features on a blood count might make you suspect a MPD?

A

High granulocytes

+/-

High red cell count/haemoglobin

+/-

High platelet count

+/-

Eosinophilia/Basophilia - especially in CML

Splenomegaly

Thrombosis in unusual places

87
Q

What types of cells do you see in excess in chronic myeloid leukaemia (CML)?

A

Proliferation of myeloid cells, meaning both granuloctes and their precursors, but also other lineages (platelets)

88
Q

What are some of the clinical features of Chronic Myeloid Leukaemia?

A

May be asymptomatic

Splenomegaly and associated symptoms

Hypermetabolic symptoms e.g. weight loss, night sweats, fatigue

Gout (due to high cellular turnover)

Miscellaneous other problems related to hyperleucocytosis (‘sludging up’ of small vessels) e.g. eye pathology, priapism etc.

89
Q

What would be the main differences if you compared a blood screen in a patient with MPD (CML) with a patient that had reactive change e.g. infection?

A

In both, WBCs, platelets and granulocytes would be raised.

However, in CML the rise in granulocytes (and their precursors, myelocytes) and WBCs would be considerably higher (especially eosinophils/basophils)

90
Q

What chromosome is the hallmark of CML?

What does this result in and why is it clinically very important?

A

Chromosome 22 (the Philadelphia chromosome) - translocation with chromosome 9

Results in a new chimaeric gene: BCR-ABL1

This gene produces tyrosine kinase which causes abnormal phosphorylation, resulting in the haematological changes seen in CML. This one mutation means that only one pathway is affected, and so giving a tyrosine kinase inhibitor (such as imatinib) provides a very good response

91
Q

What is Polycythaemia rubra vera (PRV)?

What other conditions is it important to distinguish it from?

A

PRV - high haemoglobin/Hct accompanied by erythrocytosis, but may also have excessive production of other lineages. Results in the blood becoming thicker

Important to distinguish from secondary polycythaemia (chronic hypoxia, smoking, EPO-secreting tumours etc.) and pseudopolycythaemia (dehydration, diuretic therapy, obesity)

92
Q

What test can be performed to distinguish between true and pseudopolycythaemia?

A

Centrifugation of whole blood to give packed red cells and plasma, then leave to sit

93
Q

What are some of the clinical features of Polycythaemia rubra vera (PRV)?

A

Those common to MPD (increased cellular turnover, splenomegaly, marrow failure, thrombosis)

Headache, fatigue

Itch (aquagenic pruritis - worse in warm water)

94
Q

What investigations might you perform if you suspected PRV?

What mutation in particular might you look for?

A

History (exclude secondary polycythaemia)

Examination (look for signs of splenomegaly)

FBC and blood film

Test for the JAK2 mutation (strongly associated with PRV, present in over 95% of cases)

95
Q

How is PRV treated?

A

Venesect to Hct of <0.45

Aspirin

Cytotoxic oral chemotherapy (e.g. hydroxycarbamide)

96
Q

What is Essential Thrombocythaemia (ET)?

A

Uncontrolled production of abnormal platelets

Abnormal platelets have abnormal function, meaning more likely to cause thrombosis and at higher leels can also cause bleeding due to acquired vW Disease)

97
Q

When a patient presents with possible Essential Thrombocythaemia, what condition should be ruled out initially?

Why might this condition present?

A

Need to rule out Reactive Thrombocytosis

Seen with blood loss, inflammation, malignancy and iron deficiency

98
Q

The appearance of teardrop-shaped RBCs in peripheral blood would indicate which condition?

A

Myelofibrosis

99
Q

How might myelofibrosis present?

A

Bone marrow fibrosis - anaemia, bleeding, infection

Extramedullary haemopoiesis causing hepatosplenomegaly - LUQ pain, portal hypertension

Clinical hallmarks are leukoerythroblastosis and splenomegaly

100
Q

What are the causes of a leukoerythroblastic blood film (3)?

A

Reactive (sepsis)

Marrow infiltration

Myelofibrosis

101
Q

Reactive causes of high cell counts are much more common than myeloproliferative disorders. What are some of the reactive causes of raised granulocytes?

A

Infection

Physiological e.g. post-surgery, use of steroids

102
Q

Reactive causes of high cell counts are much more common than myeloproliferative disorders. What are some of the reactive causes of raised platelets?

A

Infection

Iron deficiency

Malignancy

Blood loss

103
Q

Reactive causes of high cell counts are much more common than myeloproliferative disorders. What are some of the reactive causes of raised red cells?

A

Dehydration (+ use of diuretics) resulting in pseudopolycythaemia

Secondary polycythaemia e.g. hypoxia, smoking, EPO-secreting tumours

Year 3 - Haematology (5 decks)

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