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Medicine - Finals > Haematology > Flashcards

Haematology Flashcards

1
Q

What is multiple myeloma?

A

Cancer of bone marrow PLASMA CELLS (differentiated B lymphocytes)

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2
Q

What is the peak incidence for multiple myeloma?

A

60-70 years

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3
Q

What are the clinical features of multiple myeloma?

A 
CRAB
Calcium (raised)
Renal impairment 
Anaemia
Bone lesions/disease

Also: bone disease, infection, paraprotein production, amyloidosis, macroglossia, etc

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4
Q

What are the SYMPTOMS of myeloma that patients will present with?

A 
Osteolytic bone lesions
Backache
Pathological fractures
Sx hypercalcaemia
Sx anaemia
Frequent infections
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5
Q

What test should be done in all patients over 50 with back pain?

A

Serum protein electrophoresis + ESR

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6
Q

Give 6 Ix that should be done for multiple myeloma

A

FBC (anaemia)

Serum IgG/IgA

Urine Ig (Bence Jones proteins)

BM biopsy (^plasma cells)

Blood film (rouleax formation)

XR - lytic ‘punched out’ lesions (raindrop skull) or MRI, PET scans

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7
Q

What 2 conditions are Bence Jones proteins found in?

A

Multiple myeloma

Waldenstrom’s macroglobulinaemia

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8
Q

In what bodily fluid are Bence Jones proteins found?

A

Urine

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9
Q

What is seen on BM biopsy in multiple myeloma?

A

Increased plasma cells

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10
Q

What type of anaemia is seen in multiple myeloma?

A

Normocytic normochromic

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11
Q

What is the definition of hypercalcaemia?

A

Serum corrected Ca++ >2.6 mmol

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12
Q

What is the treatment for symptomatic or marked (>3.5 mmol) hypercalcaemia?

A

Aggressive IV hydration with 0.9% saline
IV bisphosphonates
Calcitonin

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13
Q

What is seen on blood film in multiple myeloma?

A

Rouleax formation

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14
Q

In serum electrophoresis of a patient with multiple myeloma, which is the most common paraprotein (Ig) found?

A

IgG > IgA > IgD

IgM is associated with Waldenstrom rather than MM

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15
Q

What are the 3 major criteria for diagnosis of multiple myeloma?

A

Plasmacytoma

30% plasma cells in BM sample

Elevated levels of M proteins (paraproteins) in blood or urine

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16
Q

Which cells produce paraproteins?

A

Plasma cells

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17
Q

Give 4 poor prognostic features of multiple myeloma

A

> 2 osteolytic lesions
Beta-2 macroglobulin >5.5mg/L
Hb <11g/L
Albumin <30g/L

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18
Q

What are the principle of management for myeloma?

A

Only treat SYMPTOMATIC patients

For asymptomatic MM and MGUS - regular monitoring

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19
Q

What is MGUS?

A

Monoclonal gammopathy of unknown significance

Paraproteins present, but plasma cells only occupy <10% bone marrow

May progress to MM

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20
Q

What are the features of MGUS?

A

Usually asymptomatic
No bone pain/increased infections
10-30% have demyelinating neuropathy

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21
Q

Give 3 plasma cell dyscrasias

A

MGUS
Multiple myeloma
Waldenstrom macroglobulinaemia

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22
Q

What type of amyloidosis may occur with plasma cell dyscrasias?

A

Amyloid light chain (AL) amyloidosis

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23
Q

How is symptomatic multiple myeloma managed?

A

Induction treatment

  • chemo
  • immune modulation
  • protease inhibitors
  • steroid

+ autologous stem cell transplant (if suitable)

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24
Q

How can MGUS be differentiated from myeloma?

A 
Normal immune function
Normal beta-2 macroglobulin levels
Lower level of paraproteinaemia than myeloma
Stable level of paraproteinaemia
No clinical features of myeloma
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25
Q

What is lymphoma?

A

Malignant proliferation of lymphocytes

These accumulate in lymph nodes and cause lymphadenopathy (but can also be found in peripheral blood/other organs)

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26
Q

What is the cause of most lymphomas?

A

Unknown

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27
Q

Give some infections which are RFs for lymphoma

A

EBV
HTLV-1
Helicobacter pylori

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28
Q

Give 3 primary immunodeficiency causes of lymphoma

A

Ataxia telangiectasia

Wiscott-Aldrich syndrome

Common variable immunodeficiency

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29
Q

Give 2 secondary immune deficiency causes of lymphoma

A

HIV

Transplant patients

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30
Q

What is the clinical presentation of lymphoma?

A

Enlarged, painless, non-tender, ‘rubbery’ superficial lymph nodes
Asymmetrical

Nodes may fluctuate in size

Constitutional ‘B’ symptoms

Alcohol-induced lymph node pain

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31
Q

What is Pel-Ebstein fever?

A

One of the ‘B’ symptoms of lymphoma.

Cyclical fever - temp rises abruptly, stays high for a week, then drops and stays low for a week

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32
Q

What are the 2 peaks of incidence for lymphoma?

A

20s and 60s

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33
Q

What might blood tests show in Hodgkin’s lymphoma?

A

Normocytic anaemia
Eosinophilia
Raised LDH

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34
Q

What investigations should be done for lymphoma?

A 
LN biopsy (tissue diagnosis)
Bloods (FBC, film, ESR, LFT, LDH)
Immunophenotyping, cytogenetics, molecular techniques
Imaging 
\+/- BMBx
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35
Q

What is the histological cell type associated with Hodgkin’s lymphoma?

A

Reed-Sternberg cells

mirror-image nuclei appearance

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36
Q

What is the gold standard diagnosis for Hodgkin’s lymphoma?

A

Excision of a complete LN - submitted for detailed histological evaluation

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37
Q

What staging system is used for lymphoma?

A

Ann Arbor

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38
Q

According to the Ann Arbor classification, what is ‘Stage 1’ lymphoma?

A

Confined to ONE lymph node region

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39
Q

According to the Ann Arbor classification, what is ‘Stage 2’ lymphoma?

A

Confined to 2 or more LN regions on the SAME SIDE of the diaphragm

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40
Q

According to the Ann Arbor classification, what is ‘Stage 3’ lymphoma?

A

Affecting LN regions on BOTH sides of the diaphragm

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41
Q

According to the Ann Arbor classification, what is ‘Stage 4’ lymphoma?

A

Spread beyond lymph nodes (e.g. liver, bone marrow)

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42
Q

Each Ann Arbor stage of lymphoma is further split into either A or B. What is meant by this?

A

A - no systemic symptoms other than pruritus

B - symptoms such as weight loss, fever, night sweats present

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43
Q

What are the 4 subtypes of Hodgkin’s lymphoma?

A

Nodular Sclerosing (NSHL) - most common

Mixed-Cellularity (MCHL)

Lymphocyte-Depleted (LDHL) - worst prognosis

Lymphocyte-Rich classical (LRHL) - best prognosis

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44
Q

Which subtype of Hodgkin’s lymphoma is most common?

A

Nodular Sclerosing Hodgkin’s Lymphoma (NSHL)

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45
Q

Which subtype of Hodgkin’s lymphoma carries the worst prognosis?

A

Lymphocyte-depleted Hodgkin’s lymphoma (LDHL)

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46
Q

Which subtype of Hodgkin’s lymphoma carries the best prognosis?

A

Lymphocyte-rich classical Hodgkin’s lymphoma (LRHL)

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47
Q

Give 4 poor prognostic indicators of Hodgkin’s lymphoma

A

Lymohocyte-depleted subtype
Male
Increasing age
Stage 4 disease

Raised ESR and low Hb also indicate worse prognosis

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48
Q

How is Hodgkin’s lymphoma treated?

A

Stage 1-2A - short course combination chemo followed by radio

Stage 2B-4 - combination chemo

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49
Q

What is the combination chemotherapy used in the treatment of Hodgkin’s lymphoma?

A 
ABVD
Adriamycin (doxorubicin)
Bleomycin
Vinblastine
Decarbazine
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50
Q

Give 5 side effects of the ABVD chemo regime used to treated Hodgkin’s lymphoma

A 
Infertility
Lung damage (bleomycin)
Peripheral neuropathy (vinblastine)
Cardiomyopathy (doxorubicin)
Second cancers
Psychological issues
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51
Q

Which is more common, Non-Hodgkin Lymphoma (NHL) or Hodgkin Lymphoma (HL)?

A

NHL (90%)

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52
Q

What is the median age of presentation for NHL?

A

> 50 years

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53
Q

What is the term for the cyclical fever seen in Hodgkin’s lymphoma?

A

Pel-Ebstein fever

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54
Q

Which type of lymphoma is more likely to invade the bone marrow and cause cytopenias?

A

NHL

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55
Q

In the treatment of which type of lymphoma is tumour lysis syndrome most likely to occur?

A

NHL

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56
Q

What is tumour lysis syndrome?

A
  1. Dangerous complication of chemotherapy
  2. Destruction of cancer cells results in release of intracellular components into circulation
  3. Results in hyperkalaemia, hyperuricaemia, hyperphosphataemia (which causes hypocalcaemia)
  4. Can cause fatal arrhythmias, AKI, acute gout
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57
Q

What is given as prophylaxis for tumour lysis syndrome?

A

Allopurinol or Rasburicase (uric acid reducing drugs)

IV hydration

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58
Q

Give some features of Non-Hodgkin’s lymphoma

A 
Painless widespread lymphadenopathy
Hepatosplenomegaly
Raised LDH
Paraproteinaemia
Autoimmune haemolytic anaemia
Extra-nodal disease
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59
Q

What are the 2 main subtypes of NHL?

A
  1. Indolent (low-grade) NHL, e.g. Follicular

2. Aggressive (high-grade) NHL, e.g. Diffuse large B-cell (DLBCL), Burkitt lymphoma

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60
Q

Which type of lymphoma is usually preceded by H pylori infection?

A

Gastric mucosa associated lymphoid tissue (MALT) lymphoma (a low-grade NHL)

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61
Q

What is Mycosis fungoides?

A

Localised, cutaneous manifestation of T-cell NHL

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62
Q

What are some extra-nodal features of NHL in the:

1) Skin
2) Oropharynx
3) Gut

A

1) Cutaneous T-cell lymphomas, e.g. Mycosis fungoides
2) Waldeyer’s ring lymphoma (sore throat/obstructed breathing)
3) Gastric MALT, Non-MALT gastric lymphomas (usually DLBCL), Small bowel lymphomas (can be associated with coeliac disease)

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63
Q

What is the classic treatment regime seen for NHL?

A 
R-CHOP (up to 6 rounds)
Rituximab
Cyclophosphamide
Hydroxydaunorubicin (doxorubicin)
Oncovin (vincristine)
Prednisolone
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64
Q

How many different subtypes of NHL are there?

A

> 60

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65
Q

What is the role of rituximab in treating NHL?

A

Monoclonal antibody

Anti-CD20

  • Targets CD20 proteins expressed on cell surface of B cells
  • Kills CD20+ cells by antibody-directed cytotoxicity +/- apoptosis
  • Sensitises cells to the CHOP part of R-CHOP
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66
Q

What are some poor prognostic indicators for NHL?

A 
Age >60
Systemic symptoms
Bulky disease (abdomen mass >10cm)
Raised LDH
Disseminated disease at presentation

30% 5 year survival for high grade

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67
Q

Give 6 possible acute presentations of lymphoma?

A 
Infection
SVC obstruction
Sensation of 'fullness in the head'
Dyspnoea
Blackouts
Facial oedema
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68
Q

What is leukaemia?

A

Malignant proliferation of haematopoietic cells

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69
Q

What are the 4 types of leukaemia, and which cell line do they involve?

A

Acute myeloid leukaemia (AML) - myeloblasts

Chronic myeloid leukaemia (CML) - granulocytes (basophils, neutrophils, eosinophils)

Acute lymphoblastic leukaemia (ALL) - lymphoblasts (precursors to B and T cells)

Chronic lymphocytic leukaemia (CLL) - cancer of B lymphocytes

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70
Q

Which is the most common adult acute leukaemia?

A

AML

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71
Q

What is acute promyelocytic leukaemia? What is a key risk?

A

A subtype of AML typically seen in younger patients
Characterised by t(15:17) translocation
Often shows features of DIC
High risk of fatal haemorrhage during early stage of treatment
Best prognosis after initial phase

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72
Q

What is AML?

A

Clonal expansion of myeloblasts
May occur as primary disease
Or following secondary transformation of a myeloproliferative disorder

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73
Q

Give 5 risk factors for AML?

A 
Down's syndrome
Age
Previous chemo/radio
Myelodysplasia 
Myeloproliferative disorders
CML
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74
Q

What is the key gene mutation seen in myeloproliferative disorders?

A

JAK2

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75
Q

Give 3 examples of myeloproliferative disorders

A

Polycythaemia rubra vera
Primary (essential) thrombocythaemia
Primary myelofibrosis

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76
Q

What is myelofibrosis?

A

A myeloproliferative disorder

  1. Hyperplasia of abnormal megakaryocytes
  2. Release of PDGF (platelet derived growth factor)
  3. Fibroblasts stimulated
  4. Haematopoiesis develops in liver and spleen
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77
Q

What are some features of myelofibrosis?

A

Elderly person with anaemia, e.g. fatigue is most common presenting Sx

Massive splenomegaly

Hypermetabolic symptoms - weight loss, night sweats, etc.

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78
Q

What are 5 diagnostic findings of myelofibrosis?

A

Anaemia

High WBC and platelet count in early disease

‘Tear-drop’ cells on blood film

Unobtainable BMBx - ‘dry tap’ therefore trephine biopsy needed

High urate and LDH (reflect increased cell turnover)

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79
Q

How does AML present?

A

Features of bone marrow failure:

1) Anaemia - pallor, lethargy, weakness
2) Neutropenia - frequent infections (even though WCC may be high)
3) Thrombocytopenia - bleeding
4) Infiltration - hepatosplenomegaly, gum hypertrophy
5) Bone pain
6) Fever
7) Skin involvement

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80
Q

Give 3 poor prognostic features of AMK?

A

> 60 years
20% blasts after first course of chemo
Cytogenetics - deletions of chromosomes 5 and 7

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81
Q

How was AML previously classified?

A

French-American-British (FAB) classification

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82
Q

Which gene translocation is acute pre-myelocytic leukaemia associated with?

A

t(15:17)

Fusion of the PML and RAR-alpha genes

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83
Q

What age group does acute pre-myelocytic leukaemia tend to present in?

A

Younger patients - average age 25

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84
Q

What is commonly seen at presentation of a patient with acute pre-myelocytic leukaemia?
How is this complication treated?

A

DIC

ARTA (anti-trans-retinoic acid)

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85
Q

How is AML treated?

A

Supportive therapy

  • exercise can relieve fatigue
  • blood products
  • recognise and treat infections early

Chemotherapy

  • curative vs palliative
  • intensive, long periods of suppression, 5 cycles/week
  • main drugs: daunorubicin, cytarabine

Transplantation

  • allogenic bone marrow transplants (sibs, MUD)
  • cyclophosphamide + TBI to kill all leukaemia cells pre-transplant
  • cyclosporin +/- methotrexate to prevent GVHD post-transplant
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86
Q

What do the following stand for:

1) MUD
2) TBI
3) GVHD

A

1) matching unrelated donor
2) total body irradiation
3) graft vs host disease

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87
Q

What is the prognosis of AML?

A

Poor - rapid progression means death in 2 months without treatment, 20% 3yr survival with chemotherapy

Cure rates
60% in chikdren 
50% in adults <50
<20% in older adults
Decreased cure with age reflects ability of patient to tolerate the intensive treatment and disease itself
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88
Q

What is CML?

A

Chronic myeloid leukaemia

Uncontrolled clonal proliferation of myeloid cells

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89
Q

What percentage of leukaemias are CML?

A

15%

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90
Q

What age does CML tend to present?

A

60-70, slight male predominance

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91
Q

What causes CML?

A

Philadelphia chromosome (>95%) - t(9:22) created a fusion protein which has excess tyrosine kinase activity

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92
Q

What is the Philadelphia chromosome due to?

A

Translocation between long arm of chromosome 9 and 22 - t(9:22)
Creates a fusion protein which has excess tyrosine kinase activity

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93
Q

How does CML present?

A 
30% detected by CHANCE
Mostly chronic and insidious features
-weight loss
-tiredness
-fever and sweats
-may be GOUT due to purine breakdown
-bleeding
-abdo discomfort (due to splenomegaly)

Signs

  • splenomegaly in >75%
  • hepatomegaly, anaemia, bruising
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94
Q

What investigations should be done for CML?

A

Bloods (WCC high, Hb low, platelets variable, urate increased, B12 increased)

Bone marrow biopsy (hypercellular)

Cytogenetic analysis of blood/bone marrow - showing Ph Chr

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95
Q

How is CML treated?

A

Imatinib (Gilvec) = first line
Hydroxycarbamide
Interferon-alpha
Allogenic bone marrow transplant

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96
Q

What type of drug is Imatinib (Glivec), and give 2 other examples of drugs in this class

A

Tyrosine kinase inhibitor

Nilotinib, Bosutinib

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97
Q

What are the 3 phases of CML?

A

1) Chronic - lasting months or years, few if any symptoms
2) Accelerated phase - with increased symptoms, splenomegaly and difficulty controlling blood cell counts
3) Blast transformation - with features of acute leukaemia and death

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98
Q

What is the prognosis of CML?

A

Survival >90% at 5 years

Median survival 5-6 years

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99
Q

What is the most common malignancy affecting children?

A

Acute lymphoblastic leukaemia

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100
Q

What is the peak incidence of ALL?

A

2-5 years

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101
Q

What are some risk factors/associations with ALL?

A

Down’s syndrome

Ionising radiation, e.g. X-rays during pregnancy

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102
Q

What are the 3 main types of ALL?

A

Common ALL (75%) - CD10 present, pre-B phenotype

T-cell ALL (20%)

B-cell ALL (5%)

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103
Q

What are the features of ALL?

A

Bone marrow failure features

  • Anaemia: lethargy, pallor
  • Neutropenia: frequent/severe infections
  • Thrombocytopenia: easy bruising, petechiae

Other features

  • Bone pain (secondary to infiltration)
  • Splenomegaly/Hepatomegaly
  • Lymphadenopathy
  • Testicular swelling
  • Neurological (cranial nerve palsies, meningism)
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104
Q

What are some of the common and severe infections seen in children with ALL?

A 
Chest - PCP
Mouth - candidiasis 
Perianal
Skin infections
Bacterial septicaemia 
Zoster, measles, CMV
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105
Q

What tests are done for ALL?

A 
Blood film (blasts)
Bone marrow biopsy (blasts)
WCC usually very high 
CXR/CT - look for mediastinal and abdo LN involvement 
LP to look for CNS involvement
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106
Q

How is ALL treated?

A

Supportive

  • Blood/platelet transfusion
  • IV fluids
  • Allopurinol (prevents tumour lysis syndrome)
  • Insert SC port system/Hickman line for IV access

Infection management

  • Immediate IV Abx for infections
  • Start neutropenic regime (anti-fungals/virals/biotics)
  • Co-trimoxazole if PCP

Chemotherapy

  • Induce remission
  • Consolidation
  • CNS prophylaxis
  • Maintenance

Matched related allogenic marrow transplants

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107
Q

What is the prognosis for ALL?

1) in children
2) in adults

A

1) 70-90% cure rates for children

2) 40% cure rates for adults

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108
Q

Give 6 poor prognostic factors for ALL

A 
Age <2 or >10
WBC >20 at diagnosis
T or B cell surface markers
Non-caucasian
Male sex
Philadelphia chromosome present
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109
Q

Give 8 features in a person aged 0-24 should prompt a very urgent (within 48 hrs) FBC to investigate for leukaemia?

A 
Pallor
Persistent fatigue
Unexplained fever
Unexplained persistent infections
Generalised lymphadenopathy
Persistent or unexplained bone pain
Unexplained bruising
Unexplained bleeding
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110
Q

Give 2 features in someone aged 0-24 that would prompt urgent referral for ASSESSMENT for leukaemia?

A

Unexplained petechiae

Hepatosplenomegaly

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111
Q

Which is the most common leukaemia?

A

Chronic lymphocytic leukaemia

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112
Q

What is CLL?

A

Gradual accumulation of B lymphocytes in blood, bone marrow, spleen and lymph nodes

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113
Q

Is CLL more likely in men or women?

A

2x more likely in men

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114
Q

Give some features of CML

A

Often no Sx
Constitutional - anorexia, weight loss
Bleeding, infections
Lymphadenopathy more marked than CML

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115
Q

What is the clinical course of CLL?

A

Variable
Progressive lymphadenopahty/HSM
BM failure
Hypogammaglobulinaemia + infection
Some remain stable for years, or even regress
Death often due to infection - pneumococcus, haemophilus, meningococcus, candida, aspergillum or transformation to aggressive lymphoma (Richter;s syndrome)

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116
Q

What investigations are done to diagnose CLL?

A

Blood film - SMUDGE cells (aka smear cells

Immunophenotyping

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117
Q

Give 3 complications of CLL?

A

Hypogammaglobuinaemia leading to recurrent infections

Warm autoimmune haemolytic anaemia in 10-15% patients

Transformation to high-grade lymphoma (Richter’s transformation)

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118
Q

What is Richter’s transformation?

A

Occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing, NH-lymphoma

Patients often become unwell very suddenly

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119
Q

What are the symptoms of Richter’s transformation?

A 
LN swelling
Fever w/o infection
Weight loss
Night sweats
Nausea
Abdo pain
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120
Q

How is CLL staged?

A

Binet staging

A: lymphocytosis +/- < 3 nodal areas
B: 3 or more nodal areas
C: anaemia +/- thrombocytopenia

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121
Q

How is CLL treated?

A

Watch and wait
Chemotherapy
Monoclonal antibodies, e.g. anti-CD20 Rituximab
Targeted therapy, e.g. brutal kinase inhibitors, ibrutinib
BM transplant (autologous or allogenic)
Supportive care - transfusions, IVIG if recurrent infections

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122
Q

What is the prognosis of CLL?

A

Rule of thirds:
1/3 never progress
1/3 progress slowly
1/3 progress actively

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123
Q

What is the difference between autologous and allogenic stem cell transplants?

A

Autologous - enables escalation of chemo with stem cell ‘rescue’ - relatively straightforward, mortality 2%

Allogenic - much more toxic, mortality 15-30%.
Stem cells attack residual tumour (Graft vs Leukaemia - GVL) - GOOD
Stem cells also attack recipient (Graft vs Host Disease - GVHD) - BAD
Used in acute and chronic leukaemias

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124
Q

What is the appearance of Burkitt lymphoma on lymph node biopsy?

A

‘Starry sky’ appearance

Burkitt lymphoma is a rapidly proliferating B cell tumour

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125
Q

How does Burkitt lymphoma present?

A

Unusual ways due to its rapid proliferation

e.g. Nerve root compression due to the mass effect of the lesion

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126
Q

With which virus is Burkitt lymphoma associated?

A

EBV

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127
Q

Who is Waldenstrom’s macroglobulinaemia seen in?

A

Older men

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128
Q

What is Waldenstrom’s macroglobulinaemia?

A

Uncommon condition

Lymphoplasmacytoid malignancy characterised by secretion of monoclonal IgM paraprotein

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129
Q

How does Waldenstrom’s macroglobulinaemia cause an increase in the risk of ischaemic stroke?

A

Paraproteins cause hyperviscosity of the blood, which increases the risk of ischaemic strokes

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130
Q

Which immunoglobulin is over-produced in Waldenstrom’s macroglobulinaemia?

A

IgM

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131
Q

Give some clinical features of Waldenstrom’s macroglobulinaemia

A 
Systemic upset (weight loss, lethargy)
Hyperviscosity syndrome (visual disturbance, stroke, headache, vertigo, retinopathy, seizures, spontaneous bleeding from mucus membranes)
Hepatosplenomegaly
Lymphadenopathy
Cryoglobulinaemia (eg Raynauds)
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132
Q

What is anaemia?

A

Low Hb concentration

Either due to low red cell mass or increased plasma cell volume (e.g. in pregnancy)

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133
Q

Normal values for Hb in:

1) Men
2) Women

A

1) Men: 13.1 - 16.6 g/dL

2) Women: 11.0 - 14.7 g/dL

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134
Q

Give 3 symptoms of anaemia

A 
Fatigue
Dyspnoea
Faintness
Palpitations
Headache
Tinnitus
Anorexia
Angina
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135
Q

Give 2 signs of anaemia

A 
Pallor
Sx hyperdynamic circulation (if severe)
-tachycardia
-flow murmur
-cardiac enlargement
-retinal haemorrhage (rare)
-heart failure (late)
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136
Q

Give the 5 main causes of microcytic anaemia

A 
TAILS
Thalassaemia
Anaemia of chronic disease
Iron deficiency (most common)
Lead poisoning (rare)
Sideroblastic anaemia (rare)
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137
Q

Give 4 causes of iron deficiency anaemia

A

Blood loss (e.g. menorrhagia or GI bleed)
Insufficient dietary intake
Malabsorption (eg coeliac)
Hookworm (in tropics)

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138
Q

Give 4 SIGNS of iron deficiency anaemia

A

Koilonychia (spoon shaped nails)
Atrophic glossitis (big fat red tongue)
Angular stomatitis (sores/ulceration in corner of mouth)
Post-cricoid webs (Plummer-Vinson syndrome)

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139
Q

Give 2 things you would see on a blood film for iron deficiency anaemia?

A

1) Target cells

2) Pencil poikilocytes

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140
Q

How is iron deficiency anaemia treated?

How long do you continue this treatment?

A

Treat cause
Oral iron - ferrous sulphate
Continue until Hb is normal + for at least 3 months to replenish stores

(IDA with no obvious source of bleeding = careful GI workup)

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141
Q

Give 4 side effects of ferrous sulphate

A

Nausea
Abdo pain
Diarrhoea/constipation
Black stools

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142
Q

What is the most common cause of anaemia in hospital patients?

A

Anaemia of chronic disease

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143
Q

What investigation results will be seen for anaemia of chronic disease?

A

Normocytic anaemia
Ferritin normal/raised
Check haematinics as causes of chronic anaemia are often multifactorial

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144
Q

How is anaemia of chronic disease treated?

A

Treat underlying disease vigorously
EPO (effective at raising Hb and improves QoL in cancer pt)
Iron (parenterally)
Hepcidin inhibitors and inflammatory modulators

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145
Q

What is sideroblastic anaemia?

A

Red cells fail to completely form haem

Leads to deposits of iron in the mitochondria that form a ring around the nucleus called a ring sideroblast

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146
Q

What are the causes of sideroblastic anaemia?

A

Congenital
-Delta-aminolevulinate synthase 2 deficiency

Acquired

  • Myelodysplasia
  • Alcohol
  • Lead
  • Anti-TB medications
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147
Q

How is sideroblastic anaemia diagnosed?

A

Hypochromic microcytic anaemia

Bone marrow - sideroblasts and increased iron stores

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148
Q

How is sideroblastic anaemia managed?

A

Supportive
Treat any underlying cause
Pyridoxine (vitamin B6) may help
Transfusion for severe anaemia

149
Q

What will the blood results reflect for thalassaemia and sideroblastic anaemia?

A

Accumulation of iron, so:

  • Increased serum iron
  • Increased ferritin
  • Low total iron binding capacity (TIBC)
150
Q

Causes of normocytic anaemia?

A 
Anaemia of chronic disease
Sickle cell disease
Bone marrow failure
Pregnancy
Haemolytic anaemia
Hypothyroidism
151
Q

Causes of megaloblastic macrocytic anaemia?

A

B12 deficiency
Folate deficiency
Cytotoxics (hydroxycarbamide, phenytoin)

152
Q

Causes of non-megaloblastic macrocytic anaemia?

A

Alcohol/Liver disease
Hypothyroidism
Pregnancy
Reticulocytosis

153
Q

What is the difference between macrocytes and megaloblasts?

A

Macrocytes = red cells with volume >100 fL

Megaloblasts = abnormal, giant red cell precursors with disproportionately large, immature nuclei

154
Q

What is the most common cause of megaloblastic anaemia?

A

B12/folate deficiency

155
Q

What is the bioactive form of folate?

A

Tetrahydrofolate (THF)

156
Q

What is the bioactive form of folate (THF) required for?

A

Purine and pyrimidine synthesis (thus RNA and DNA)

157
Q

Why is it crucial to correct B12 deficiency before correcting folate deficiency?

A

To prevent neurological damage

158
Q

Which B vitamin is folate?

Where is it stored?

Where is it absorbed?

A

B9

Liver

Duodenum and jejunum

159
Q

What are the 4 broad causes of folate deficiency?

A

1) Insufficient intake
2) Excessive loss
3) Abnormal THF activation
4) Increased folate demand

160
Q

Why will a B12 deficiency always cause a functional folate deficiency?

A

Activation of folate into its active form (THF) is dependent on methionine synthase, a B12-dependent enzyme

161
Q

How long do body stores of folate last?

A

4 months

162
Q

Give 3 foods that provide a source of folate

A

Green veg
Nuts
Yeast

163
Q

What can maternal folate deficiency cause to the foetus?

A

Neural tube defects

164
Q

Give 2 drugs that can cause folate deficiency

A

Methotrexate

Trimethoprim

165
Q

What is the role of folate in:

1) Heart disease
2) Cognition

A

1) lowers homocysteine levels = decreased risk of CVD

2) benefits cognition

166
Q

How is folate deficiency treated?

A

Assess for underlying cause (poor diet, malabsorption)

Treat with oral folic acid - 5mg/day

167
Q

How is folate deficiency prophylaxis given in pregnancy?

A

0.4mg/day from conception until at least 12 weeks

168
Q

Give 3 types of food B12 is found in

A

Meat
Fish
Dairy products

169
Q

How long do body stores of vitamin B12 last for?

A

4 years

170
Q

How is B12 absorbed?

A

Binds to intrinsic factor in the stomach, and is absorbed in the terminal ileum

171
Q

Why does B12 deficiency cause a reduction in the rate of RBC production?

A

Synthesis of thymidine (hence DNA) is impaired

172
Q

Causes of B12 deficiency?

A

Dietary (eg vegans)

Malabsorption

  • Stomach, lack of IF (pernicious anaemia, post-gastrectomy, post-gastric-banding)
  • Terminal ileum (resection, Crohn’s, bacterial growth, tapeworms, metformin use)
  • Congenital metabolic errors
173
Q

Clinical features of B12 deficiency?

A

Normal features of anaemia (fatigue, etc)

Neuropsychiatric (irritability, depression, psychosis, dementia)

Neurological (paraesthesia, peripheral neuropathy, subacute degeneration of the spinal cord)

174
Q

What is the triad of features seen in subacute degeneration of the spinal cord?

A

1) Extensor plantars (UMN)
2) Absent knee jerks (LMN)
3) Absent ankle jerks (LMN)

175
Q

What is pernicious anaemia?

A

Autoimmune atrophic gastritis leading to achlorydia (absence of HCl in gastric secretions) and lack of intrinsic factor secretion

176
Q

In what age does pernicious anaemia tend to develop?

A

Middle to old age

177
Q

In which blood group type is pernicious anaemia more common?

A

Blood group A

178
Q

What are some associations with pernicious anaemia?

A 
Thyroid disease
Type 1 diabetes 
Addison's disease
Rheumatoid arthritis 
Vitiligo 
Hypoparathyroidism

Predisposes to gastric carcinoma

179
Q

What are the features of pernicious anaemia?

A

Lethargy
Weakness
Dyspnoea
Paraesthesia

Also: mild jaundice ‘lemon yellow’, diarrhoea, sore tongue

Possible signs: retinal haemorrhages, mild splenomegaly, retrobulbar neuritis

180
Q

Give 2 specific investigations for pernicious anaemia

A

1) Parietal cell antibodies

2) Intrinsic factor antibodies

181
Q

How is pernicious anaemia/B12 deficiency treated?

A

Treat underlying cause if possible
B12 injections (hydroxycobalamin)
-1mg IM every other day for 2 weeks/until CNS signs stop
-Maintenance = 1mg every 3 months for life

If cause is definitely dietary rather than malabsorption, oral B12 can be taken

182
Q

What is aplastic anaemia?

A

Bone marrow failure leading to pancytopenia

183
Q

What is the peak age at which aplastic anaemia is acquired?

A

30 years

184
Q

What are the features of aplastic anaemia?

A

Normochromic, normocytic anaemia
Leukopenia with lymphocytes relatively spared
Thrombocytopenia
May be the presenting feature of ALL or AML
Minority of patients develop paroxysmal nocturnal haemoglobinuria or myelodysplasia

185
Q

What are the causes of aplastic anaemia?

A 
Idiopathic
Congenital - Fanconi anaemia, dyskeratosis congenita
Drugs
Toxins - benzene
Infections - parvovirus, hepatitis
Radiation
186
Q

Give 4 medications that can cause aplastic anaemia

A

Phenytoin
Cytotoxics
Chloramphenicol
Sulphonamides

187
Q

Give 3 hereditary causes of haemolytic anaemia

A

Membrane - hereditary spherocytosis
Metabolism - G6PD deficiency
Haemoglobinopathies - sickle cell, thalassaemia

188
Q

What are the acquired causes of haemolytic anaemia?

1) Immune (3)
2) Non-immune (4)

A

1) Immune
Autoimmune (warm/cold Ab type)
Allo-immune (tranfusion reaction, haemolytic disease of the newborn)
Drugs (methyldopa, penicillin)

2) Non-immune 
Microangiopathic haemolytic anaemia (MAHA) - TTP/HUS, DIC, malignancy, pre-eclampsia
Prosthetic cardiac valves
Paroxysmal nocturnal haemoglobinuria
Infections - malaria
Drugs - dapsone
189
Q

What is G6PD deficiency?

A

Most common RBC enzyme defect
More common in people from the Mediterranean and Africa
X-linked recessive (affects males)
Many drugs can precipitate a crisis as well as infections and broad (fava) beans

190
Q

What is an important consideration when treating a patient with lymphoma who has G6PD deficiency?

A

They must not be given rasburicase as prophylaxis against tumour lysis syndrome (severe side effect of chemo treatment)

They can be given allopurinol instead

191
Q

Give 5 features of G6PD deficiency?

A 
Neonatal jaundice
Intravascular haemolysis
Gallstones
Splenomegaly
Heinz bodies on blood films
192
Q

Give 5 drugs that can precipitate haemolysis in G6PD deficiency

A 
Anti-malarials (primaquine)
Ciprofloxacin
Sulphonamides
Sulphasalazine
Sulfonylureas
193
Q

How is G6PD deficiency diagnosed?

A

G6PD enzyme assay

194
Q

What is hereditary spherocytosis?

How is it inherited?

A

Most common hereditary anaemia in North Europeans
AD-inheritance
Normal biconcave disc shape of RBC replaced by a sphere-shaped one
RBC survival reduced as destroyed by the spleen

195
Q

Give 7 ways hereditary spherocytosis presents?

A 
Failure to thrive
Jaundice, gallstones
Splenomegaly
Aplastic crisis precipitated by parvovirus infection
Degree of haemolysis variable
MCHC elevaged
Spherocytes on blood film
196
Q

How is hereditary spherocytosis diagnosed?

A

Osmotic fragility test

Spherocytes on blood film - round, lack of central pallor

197
Q

How is hereditary spherocytosis treated?

A

Folate replacement

Splenectomy

198
Q

What level of Hb must be reached for someone to require a blood transfusion?

1) Patient without ACS
2) Patient with ACS

A

1) 70g/L

2) 80g/L

199
Q

Why is the transfusion threshold lower in ACS?

A

Anaemia can worsen the ischaemia in ACS because there is less Hb to carry oxygen, therefore heart has to work harder

Important to provide immediate relief to anaemia of this level (<80) in someone with ACS

200
Q

What are some complications of blood transfusions?

A 
Acute haemolysis
Non0haemolytic febrile reaction
Allergy/anaphylaxis  
Infections
TRALI (transfusion-related lung injury)
Fluid overload
Hyperkalaemia
Iron overload
Clotting
201
Q

What causes an acute haemolytic transfusion reaction?

A

Mismatch of ABO blood group

Causes massive intravascular haemolysis

202
Q

How long after a transfusion does an acute haemolytic transfusion reaction?

A

Symptoms begin minutes after transfusion starts (fever, abdominal and chest pain, agitation, hypotension)

203
Q

What is the treatment for an acute haemolytic transfusion reaction?

A

Immediate transfusion termination
Generous fluid resus with saline
Inform lab

204
Q

Give 2 possible complications of an acute haemolytic transfusion reaction

A

DIC

Renal failure

205
Q

What causes a non-haemolytic febrile transfusion reaction?

A

White blood cell HLA antibodies

Often the result of sensitisation by previous pregnancies or transfusions

206
Q

What causes an allergic/anaphylactic blood transfusion reaction?

A

Hypersensitivity reaction to components within the transfusion

207
Q

How long after a transfusion may an allergic/anaphylactic reaction occur?

A

Within minutes - can be varying severity

208
Q

What symptoms/signs would a patient experiencing an allergic/anaphylactic reaction from transfusion present with?

A 
Urticaria
Hypotension
Wheezing
Dyspnoea
Stridor
Angioedema
209
Q

Which type of infection is most likely to occur from a platelet transfusion? Why?

A

Bacterial infections

Because platelets are stored at room temp, so provide a favourable environment for bacterial contamination

210
Q

What is the universal donor blood group for:

1) Red cells
2) Fresh frozen plasma?

A

1) O Rh-ve

2) AB Rh-ve

211
Q

What is leucodepletion?

A

Process of removing white cells from a blood product to reduce risk of white-cell borne infection (including vCJD transmission and GVHD)

212
Q

What does the ABO system refer to?

A

Various forms of the ‘H’ antigen on the surface of red cells

213
Q

What is the ABO group if the H antigen is unmodified?

A

O

214
Q

What are the five Rh antigens?

A

C, c, D, E, e

215
Q

Presence of which allele confers Rh positive status?

A

D

216
Q

Why does ABO incompatibility between mother and fetus not cause any problems, whilst Rh incompatibility does?

A

Antibodies against Rh D are IgG - small enough to cross placenta

Antibodies against ABO are IgM - large, bulky, cannot pass placenta

217
Q

What is a ‘sensitising event’?

A

Event leading to development of anti-D IgG (antibodies) in Rh-ve females

May result in haemolytic disease of the fetus/newborn

218
Q

Give 5 examples of sensitising events

A

1) Normal pregnancy (small amounts of fatal blood enter maternal circulation)
2) Maternal trauma during pregnancy
3) Ectopic pregnancy
4) Abortion
5) Amniocentesis/chorionic villous sampling

219
Q

How is Anti-D immunoglobulin delivered to Rh-ve females?

How does it work?

A

IM injection
Coats fetal red cell D antigens that have entered maternal circulation, before maternal anti-D antibodies can be generated

220
Q

What is haemolytic disease of the fetus (HDF)?

A

IgG anti-D antibodies cross placenta and lead to immune haemolysis and hydrops fetalis (prenatal heart failure in utero)

221
Q

2+ units of blood can result in fluid overload. How can this be avoided?

A

Prescribe frusemide in between transfusions of each unit

222
Q

In addition to red cell transfusions, what are some other transfusion products?

A 
Platelets
Granulocytes
FFP
Immunoglobulins 
Human albumin solution (HAS)
Cryoprecipitate
Clotting factor concentrates (single or combined)
223
Q

What transfusion product is used to reverse warfarin in bleeding?

A

Prothrombin complex concentrate (a combined clotting factor concentrate)

224
Q

What is Octaplex, and what is it used for? Why is it used in preference to FFP?

A

A PCC (prothrombin complex concentrate)

Reduced risk of TACO (transfusion-associated circulatory overload)

225
Q

What is Octoplas?

A

Detergent-treated FFP

226
Q

Give 5 acute (within 24hr) life-threatening complications that may occur following transfusion

A

1) AHTR
2) Bacterial contamination
3) Anaphylaxis
4) TACO
5) TRALI

227
Q

Give 2 acute (within 24hr) mild complications that may occur following tranfusion

A

1) Febrile non-haemolytic transfusion reaction (FNHTR)

2) Urticaria

228
Q

Give 3 delayed (after 24hr) complications that may occur following transfusion

A

1) DHTR
2) Post-transfusion purpura (PTP)
3) taGVHD (transfusion-associated GVHD)

229
Q

Give 2 chronic/late complications that may occur following transfusion

A

1) post-transfusion viral infection

2) iron overload

230
Q

Management of anaphylaxis from transfusion?

A 
Stop transfusion
Detach giving set
Flush cannula with saline
IM adrenaline (0.5mg)
Lie flat and elevate legs
IV crystalloids

CALL ANAESTHETIST - airway

IV hydrocortisone + chlorphenamine

231
Q

What are the features of post-thrombotic syndrome?

How may these symptoms be managed?

A 
Painful, heavy calves
Pruritus 
Swelling
Varicose veins
Venous ulceration

Manage with:

  • Compression stockings
  • Leg elevation
232
Q

What is the most common:

1) Clotting disorder (thrombophilia)
2) Bleeding disorder ?

A

1) Factor V Leiden (aka activated protein C deficiency)

2) Von Willebrand disease

233
Q

What is the usual lifespan of platelets?

A

7-10 days

234
Q

How are platelets made (thrombopoiesis)?

A

TPO (made by liver) stimulates synthesis from megakaryocytes in bone marrow

235
Q

ITP in children usually follows a viral infection and resolves spontaneously. In adults, it usually needs treatment. How is it managed?

A

1) Immunosuppression (steroids, ciclosporin, or rituximab)
2) IVIg/anti-D Ig given to saturate splenic macrophages
3) TPO mimetics to increase platelet count

236
Q

Give 2 examples of TPO mimetics

A

Romiplostim

Eltromboplag

237
Q

What is the classic pentad seen in TTP?

A

1) Fever
2) Transient focal neurological deficits
3) AKI
4) MAHA (microangiopathic haemolytic anaemia)
5) Thrombocytopenia

238
Q

What is the name of the protease enzyme that cleaves large multimers of vWF?

A

ADAMTS-13

239
Q

What is the usual underlying mechanism for TTP?

A

Acquired deficiency of ADAMTS-13 enzyme, resulting in persistence of large vWF multimers
This leads to inappropriate haemostasis

240
Q

TTP is a medical emergency and can be fatal if untreated. How is it managed?

A 
Urgent plasmapheresis 
(removes anti-ADAMTS-13 antibodies and replaces deficient ADAMTS-13)
241
Q

What is the most common cause of AKI in children?

A

Haemolytic uraemic syndrome (HUS)

242
Q

What is the diagnostic triad of HUS?

A

AKI
Low platelets
Microangiopathic haemolytic anaemia (MAHA)

243
Q

What usually causes HUS?

A

Infection with shiga-toxin producing E. coli (O157:H7 strain)
Shiga-toxin inhibits ADAMTS-13 enzyme
Microthrombi form in glomeruli of kidney

244
Q

How does hypocalcaemia affect the coagulation cascade?

A

Calcium is required for activation of vitamin-K dependant factors (2, 7, 9, 10)

245
Q

How does citrate in blood test tubes prevent clotting? How does this differ from EDTA?

A

It de-ionises calcium - this can be reversed by adding calcium

(unlike EDTA, which irreversibly chelates the calcium ions)

246
Q

What are the vitamin K dependent clotting factors?

A

2, 7, 9, 10

247
Q

How does warfarin work?

A

Inhibits the enzyme vitamin K epoxide reductase - prevents regeneration of active form of vitamin K

248
Q

Give some important questions to ask about in a bleeding history

A

1) Any mucocutaneous bleeding? (from gums, nose, rectum, easy bruising, etc)
2) Bleeding into joints?
3) Previous surgical complications? Excessive bleeding after eg dental extraction?

249
Q

What are the 3 components of a clotting screen?

A

Prothrombin time
Activated partial thromboplastin time
Fibrinogen

250
Q

Which part of the clotting screen represents the:
1) intrinsic pathway
2) extrinsic pathway
How long are they?

A

1) APTT (usually 30-40s)

2) PT (usually 10-14s)

251
Q

Which blood test is most affected by warfarin overdose?

A

PT (prolonged) - also INR

252
Q

What are proteins C and S?

Which vitamin are they dependent on?

A

Protein C = serine protease - destroys activated Va and VIIIa

Protein S = cofactor for protein C

Both are vitamin K-dependent

253
Q

Give 4 intrinsic methods for anti-coagulation

Which does heparin potentiate?

A

1) Tissue-factor pathway inhibitor (TFPI) - prevents extrinsic pathway of coagulation
2) Proteins C and S
3) Thrombomodulin (binds thrombin)
4) Antithrombin (inhibits some clotting factors) - potentiated by heparin

254
Q

What is the name for the process that enables clot breakdown?

A

Fibrinolysis

255
Q

What is plasmin?

What is it activated by?

A

Serine protease that breaks down fibrin networks, degrading clots

Activated by tPA (tissue plasminogen activator) or urokinase

256
Q

What are D-dimers?

A

Small fibrin fragments

257
Q

Which factor does rivaroxaban inhibit?

How will PT and APTT be affected?

A

Xa

Both prolonged

258
Q

Which factor does dabigatran inhibit?

How will PT and APTT be affected?

A

Thrombin (IIa)

Both prolonged

259
Q

Give 4 examples where fibrinogen may be high?

A

Pregnancy
Acute/chronic inflammation
Trauma
Certain cancers, eg Hodgkin lymphoma

260
Q

What is the difference between a thrombus and a thromboembolism?

A

Clot remaining at site of formation = thrombus

If dislodged, thrombus = thromboembolism (pathologic)

261
Q

What is Virchow’s triad?

A

Haemodynamic disruption
Endothelial injury/dysfunction
Hypercoagulability

262
Q

How long should someone be on Warfarin following a DVT?

A

Provoked (ie recent surgery) = 3 months

Unprovoked = 6 months

263
Q

What is the MOA for for:

1) Rivaroxaban
2) Apixaban
3) Dabigatran
4) Heparin
5) Warfarin

A

1) and 2) - direct factor Xa inhibitor
3) Direct thrombin (IIa) inhibitor
4) Activates antithrombin III
5) Inhibits factors 10, 9, 7, 2

264
Q

Give 3 ways advanced liver disease increase bleeding risk?

A

1) All clotting factors (except vWF) synthesised by liver
2) Cirrhosis -> Portal HTN -> Splenomegaly -> increased platelet sequestration
3) Reduced TPO synthesis by liver reduced platelet production

265
Q

Which antibiotic is recommended in an episode of neutropenic sepsis?

A

Piperacillin with tazobactam (Tazocin)

266
Q

What are the 2 main classifications of polycythaemia (too many RBCs)?

A

1) Primary/proliferative - a true polycythaemia, e.g. a myeloproliferative disorder, polycythaemia rubra vera
2) Secondary (more common) - physiological response to tissue hypoxia

267
Q

Give 6 causes of secondary polycythaemia

A 
Altitude
COPD/lung disease
Smoking
Cyanotic heart disease
Obstructive sleep apnoea
EPO/androgen excess
268
Q

Give 4 causes of EPO/androgen excess which may cause secondary polycythaemia

A

Cerebellar haemangioma
Hypernephroma
Hepatoma
Doping

269
Q

What is polycythaemia rubra vera?

A

Myeloproliferative disorder - ‘overactive bone marrow’

Caused by clonal proliferation of marrow stem cells leading to an increase in red cell volume, often accompanied by over-production of neutrophils and platelets

270
Q

What mutation is seen in 95% polycythaemia rubra vera?

A

JAK2 mutation

271
Q

At what age is the peak incidence of polycythaemia rubra vera?

A

50s

272
Q

Give 9 features of polycythaemia rubra vera

A 
Hyperviscosity
Thrombosis
Pruritus, typically after hot bath
Splenomegaly
Haemorrhage (secondary to abnormal platelet function)
Plethoric appearance
HTN in 1/3
Gouty arthritis in 1/5
Abnormal FBC
273
Q

How is polycythaemia rubra vera investigated?

A

FBC (raised haematocrit)
JAK2 mutation screening
Serum ferritin
Renal and liver function tests

274
Q

How is polycythaemia rubra vera treated?

A

Aspirin
Venesection
Bone marrow suppression (hydroxycarbamide)

275
Q

What is sickle cell anaemia?

A

Autosomal recessive disorder causing production of abnormal beta globin chains
HbS rather than HbA produced

276
Q

In which group of people is sickle cell anaemia most common?

A

African origin (due to protection against plasmodium falciparum in heterozygous carriers)

277
Q

What is the amino acid substitution seen in sickle cell?

A

Valine for glutamic acid

278
Q

What happens to the HbS at low pO2?

What happens on reoxygenation?

A

Polymerises into long intracellular fibres - deforms RBC and causes capillary occlusion

Initially reforms on reoxygenation, but will eventually become irreversibly sickled

279
Q

What is the term for an acute episode of sickle cell disease?

A

Sickle cell crisis

280
Q

Give 9 main complications of sickle cell disease

A

1) Chronic haemolytic anaemia
2) Haemolytic crises
3) Painful infarction crises
4) Aplastic crises
5) Splenic complications
6) Infection susceptibility
7) Acute chest syndrome
8) Cerebrovascular ischaemia
9) Priapism, Skin ulceration, Proliferative retinopathy

281
Q

Give 2 possible triggers of aplastic crises in sickle cell disease

How can it be differentiated from acute haemolytic crises?

A

1) Parvovirus B19 infection
2) Folate deficiency

No reticulocytes present

282
Q

Why does sickle cell disease cause chronic haemolytic anaemia?

A

Deformed RBCs are removed from the circulation more rapidly, reducing the lifespan from 120d to 10-20d

283
Q

How can sickle cell disease cause splenic complications?

A

1) Repeated micro-infarctions result in hyposplenism/asplenism
2) Acute splenic sequestration may occur (trapped blood in spleen -> abdomen pain, splenomegaly, drop in Hb)

284
Q

What are the 4 key diagnostic features of acute chest syndrome in sickle cell disease?

A

Dyspnoea
Drop in arterial pO2
Chest pain
Pulmonary oedema on xray

285
Q

What type of infections are people with sickle cell disease at particularly increased risk of?

A

Encapsulated organisms

286
Q

How may sickle cell disease cause TIA/strokes?

A

Micro-infarctions

287
Q

What is the most common reason for hospital admission in patients with sickle cell anaemia?

A

Painful infarction crises

288
Q

How is chronic sickle cell disease managed?

A

Input from haematology

Hydroxycarbamide to suppress bone marrow if frequent crises

Antibiotics and immunisation as prophylaxis for splenic infarct

Febrile sickle cell children high risk for sepsis - give ceftriaxone

289
Q

What is thalassaemia?

A

Genetic disease of unbalanced Hb synthesis, with deficiency/complete lack of one globin chain

Unmatched globin chains precipitate, damaging RBC membranes, causing haemolysis whilst still in bone marrow

290
Q

In which areas is thalassaemia common?

A

Mediterranean to Far East

291
Q

What is beta thalasaemia?

A

Decreased (beta thalassaemia minor) or absent (beta thalassaemia major) production of beta chains

292
Q

What is the most common cause of beta thalassaemia?

A

Point mutations in the beta globin genes on chromosome 11

293
Q

What investigations are done for beta thalassaemia?

A 
FBC + MCV (microcytic anaemia)
Blood film
Iron
HbA2 and HbF raised
Hb electrophoresis
294
Q

How is beta thalassaemia managed?

A

Promote fitness and healthy diet

Folate supplements

Transfusions (every 2-4 wk) - keep Hb >90

Iron chelators to prevent iron overload (oral deferiprone, SC desferroxamine)

Ascorbic acid (increase urinary iron excretion)

Splenectomy if hypersplenism persists

Hormonal replacement/treatment for endocrine complications

Histocompatible marrow transplant can offer cure

Genetic counselling/antenatal diagnosis can prevent

295
Q

What is alpha thalassaemia?

A

2 separate alpha globin genes on each chromosome 16

4 genes termed aa/aa

Alpha thalassaemias mainly caused by gene deletions

If all 4 deleted (–/–) = in utero death (hydrops fetalis)
If 3 genes deleted (–/-a) = moderate anaemia and features of haemolysis (splenomegaly, leg ulcers, jaundice)
If 2 genes deleted (–/aa) or (-a/-a) = asymptomatic carrier state with decreased MCV
With just one deleted, clinical state is normal

296
Q

What are the clinical classifications of thalassaemia?

A

Thalassaemia major = transfusion dependent

Thalassaemia intermedia = less severe anaemia, can survive without regular blood transfusions

Thalassaemia carrier/heterozygote = asymptomatic

297
Q

What is beta thalassaemia trait?

A

Autosomal recessive
Mild hypochromic, microcytic anaemia – microcytosis is characteristically disproportionate to the anaemia
HbA2 raised (>3.5%)
Usually asymptomatic

298
Q

What are the 3 types of autoimmune haemolytic anaemia (AIHA)?

A

1) Warm AIHA (haemolysis occurs at body temp)
2) Cold AIHA (haemolysis occurs below core body temp)
3) Paroxysmal cold haemoglobinuria

299
Q

What test is used to detect AIHA?

A

Coomb’s test (direct antigen test)

300
Q

What are the four types of globin chains may make up a haemoglobin molecule?

A

Alpha
Beta
Delta
Gamma

301
Q

What are the following composed of:

1) HbF (foetal)
2) HbA (main adult)
3) HbA2

A

1) alpha2, gamma2
2) alpha2, beta2
3) alpha2, delta2

302
Q

At what age do symptoms of beta-thalassaemia develop?

A

3-6 months old (when HbA takes over from HbF)

303
Q

How can hydroxyurea help some patients with sickle cell crisis?

A

Stimulated production of fetal haemoglobin

304
Q

What is haemophilia?

A

X-linked recessive disorder of coagulation

305
Q

What are the 2 types of haemophilia?

A

A (factor 8 deficiency)

B (factor 9 deficiency) - Christmas disease

306
Q

How does haemophilia present?

A

Haemarthroses (bleeding in joints)

Haematomas (esp in muscle)

Prolonged bleeding after surgery or trauma

307
Q

What would appear on blood tests in someone with haemophilia?

A

Prolonged APTT

Normal bleeding time, thrombin time and prothrombin time

308
Q

How is haemophilia treated?

A

A: factor 8 replacement
B: factor 9 replacement

Up to 1-15% of patients with haemophilia A develop antibodies to factor VIII treatment

309
Q

How are platelets produced?

What is their lifespan?

A

Anucleate cell fragments from megakaryocytes

Thrombopoietin (TPO) - stimulates production of platelets by megakaryocytes, produced mainly by liver

Lifespan 7-10 days

310
Q

What are some causes of thrombocytosis?

A

Reactive (platelets are an acute phase reactant, so can increase in response to stress such as severe infection or surgery)

Malignancy

Essential thrombocytosis

Hyposplenism (spleen breaks down old platelets so if its function is reduced, more platelets can circulate)

311
Q

What is essential thrombocytosis?

A

A myeloproliferative disorder

Overlaps with CML, PRV, myelofibrosis

Megakaryocytic proliferation results in overproduction of platelets

312
Q

What are some features of essential thrombocytosis?

A

Platelet count >600
Thrombosis (venous or arterial)
Haemorrhage
Characteristic symptom = burning sensation in hands

313
Q

What mutation is seen in 50% essential thrombocytosis?

A

JAK2 mutation

314
Q

How is essential thrombocytosis managed?

A

Hydroxyurea (hydroxycarbamide) - widely used to reduce platelet count

Interferon alpha

Low-dose aspirin to reduce thrombotic risk

315
Q

What are some causes of severe thrombocytopenia?

A

ITP
DIC
TTP
Haematological malignancy (bone marrow failure)

316
Q

What are some causes of moderate thrombocytopenia?

A 
Heparin-induced thrombocytopenia (HIT)
Drug-induced (quinine, diuretics, sulphonamides, aspirin, thiazides)
Alcohol/Liver disease
Hypersplenism
Viral infection (EBV, HIV, hepatitis)
Pregnancy
SLE/antiphospholipid syndrome
Vitamin B12 deficiency
317
Q

What is ITP?

A

Immune thrombocytopenic purpura

Immune-mediated reduction in platelet count - antibodies are directed against glycoprotein 2b/3a or Ib-V-IX complex

318
Q

What are the symptoms/signs of ITP?

A

Low platelet counts on bloods

Isolated thrombocytopenia - rest of blood count is normal

Non-blanching purpuric rash

Easy bruising

Evidence of mucosal bleeding - menorrhagia, nose bleeds, gum bleeding when brushing teeth

319
Q

What are the features of acute ITP?

A

More common in children

May follow infection or vaccination - antibody-viral antigen complexes bind to platelets, resulting in removal of platelets by reticuloendothelial system

Usually self-resolves within 1-2 weeks

320
Q

What are the features of chronic ITP?

A

More common in young/middle aged women

Tends to run a relapsing-remitting course

Often Hx autoimmune disease

321
Q

How is ITP managed?

A

General:

  • Stop NSAID/aspirin
  • Observe platelet count

Immunosuppression:

  • Steroid (Prednisolone)
  • Rituximab (second line)

Reduce platelet destruction:
-IVIG (saturates splenic macrophages)

Increase platelet production
-TPO mimetics, e.g. romiplostim, eltrombopag

322
Q

What is Evan’s syndrome?

A

ITP in association with autoimmune haemolytic anaemia

323
Q

What is thrombotic thrombocytopenic purpura?

A

Medical emergency!

Rare form of thrombotic microangiopathy

324
Q

What are some RFs for TTP?

A

Pregnancy/post-partum (up to 25% cases)
HIV
Autoimmune disease
Cancer

325
Q

What is the pathogenesis of TTP?

A

Abnormally large and sticking multimers of vWF (due to ADAMTS-13 deficiency) cause platelets to clump within vessels

Overlaps with HUS

326
Q

Features of TTP?

A 
Classic pentad:
Fever
Cerebral dysfunction (confusion, headache, paresis, dysarthria, visual problems)
Haemolytic anaemia
Thrombocytopenia
AKI
327
Q

What are the two features of TTP required to make a diagnosis?

A

AIHA

Thrombocytopenia

328
Q

How is TTP managed?

A

Urgent plasmapheresis
Steroids
Immune suppressants
Vincristine

329
Q

What are the 3 indications for a platelet transfusion?

A

Platelet count <30 with clinically significant bleeding (WHO bleeding grade 2 - haematemesis, melaena, prolonged epistaxis)

Higher transfusion thresholds up to <100 (for patients with WHO bleeding grades 3/4 or bleeding at critical sites - e.g. CNS)

Pre-invasive procedure (prophylactic)

330
Q

Give 4 contraindications for platelet transfusion?

A

Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin-induced thrombocytopenia
Thrombotic thrombocytopenic purpura

331
Q

What is disseminated intravascular coagulation?

A

Dangerous condition due to widespread activation of coagulation cascades.

Triggered by sepsis, trauma, burns, malignancy, massive blood loss etc

332
Q

What is the underlying pathophysiology of DIC?

A

1) Systemic activation of clotting cascade ->
2) Microvascular thrombosis
AND
1) Consumption of platelets and clotting factors ->
2) Bleeding

Microvascular thrombosis can lead to organ failure

333
Q

Which malignancy in particular can be a RF for DIC?

A

Leukaemias

334
Q

Give some causes/RFs for DIC

A 
Sepsis
Malignancy - especially leukaemias
Major trauma – crush syndrome, burns
Complications of pregnancy – abruption, HELLP, pre-eclampsia
Incompatible blood transfusion
Transplant rejection
Severe liver disease
Pancreatitis 
Recreational drugs
Snake bites
Connective tissue disorders – incl. antiphospholipid syndrome
335
Q

How is DIC investigated?

A

Look for underlying cause
Clotting profile
D-dimer (raised)
+/- evidence organ failure

336
Q

What would be seen on the blood tests for someone with DIC?

A

Low platelets
Prolonged PT
Prolonged APTT
Prolonged bleeding time

337
Q

How is DIC managed?

A 
Treat underlying cause
Supportive:
-Platelets
-FFP
-Cryoprecipitate
338
Q

What is the most common inherited bleeding disorder?

A

von Willebrand disease

339
Q

How is von Willebrand disease usually inherited?

A

Autosomal dominant

340
Q

How many von Willebrand disease present?

A 
Mucosal bleeding (epistaxis, menorrhagia)
Unlikely to have large haemarthroses/haematomas
341
Q

What would blood investigation results show in vWD?

A

Prolonged bleeding time
APTT may be prolonged
Factor 8 levels may be reduced
Defective platelet aggregation with ristocetin (substance that aims to force platelet aggregation)

342
Q

How is vWD managed?

A

1) Tranexamic acid for mild bleeding
2) DDAVP (Desmopressin) - raises levels of vWF
3) Factor VIII concentrate

343
Q

Where is vWF produced?

A

Endothelial cells

344
Q

What is the most common type of VWD?

A

Type 1 (partial deficiency - if mild, may not show on clotting screen)

345
Q

What are the 3 types of VWD?

A 
Type 1 - partial deficiency
Type 2 (A, B, M, N) - functional issue with protein 
Type 3 (absolute deficiency - may look like a haemophilia)
346
Q

Why may factor VIII levels be low in someone with VWD?

A

Factor VIII has a short half-life

VWF binds to it and lengthens its half life

347
Q

What is heparin-induced thrombocytopenia?

A

Development of IgG antibody against platelet-heparin complexes

IgG-PF4-Heparin complexes bind to and activate platelets

  • > Platelet consumption increased
  • > Thrombosis (arterial or venous)
  • > Skin necrosis
348
Q

Who is most at risk of heparin-induced thrombocytopenia?

A

Patients following cardiac bypass surgery with large amounts of unfractionated heparin treatment

349
Q

How does heparin-induced thrombocytopenia present?

A

Sharp fall in platelets 5-10 days after starting heparin

350
Q

How is heparin-induced thrombocytopenia managed?

A

Life-threatening: STOP HEPARIN IMMEDIATELY

Use alternative anticoagulation (even if platelets low)

Never re-expose patient to heparin

351
Q

What are the 4 main haematological emergencies?

A

1) Neutropenic sepsis
2) Hyperviscosity syndrome
3) Tumour lysis syndrome
4) Acute sickle chest syndrome

352
Q

Haematological malignancies are a common cause of tumour lysis syndrome. What causes this and how can it be prevented?

A

Chemotherapy (often) - due to rapid death of cells

IV rasburicase or IV allopurinol can be given before chemo to reduce risk

353
Q

What are the diagnostic criteria for tumour lysis syndrome?

A

Abnormality in 2+ of the following within 3 days before and 7 days after chemo:

1) Uric acid
2) K+
3) Phosphate
4) Calcium

Lab TLS +

1) ^serum creatinine
2) cardiac arrhythmia/sudden death
3) seizure

354
Q

Give 5 complications of tumour lysis syndrome

A 
Hyperkalaemia
Hyperphosphataemia
Hypocalcaemia
Hyperuricaemia
Acute renal failure
355
Q

How is tumour lysis syndrome treated?

A 
Aggressive IV fluid resus
Allopurinol, rasburicase
Control of electrolytes
-BM monitoring in at-risk patients
-Treat hyperkalaemia
Early referral for dialysis if needed
356
Q

What is the definition of neutropenic sepsis?

A

Neut <0.5
In a patient having anti-cancer treatment
AND
T >38
or other Sx consistent with clinically significant sepsis

357
Q

Give the 5 types of recognised crises in sickle cell disease

A 
Thrombotic, vaso-occlusive 'painful crises'
Sequestration
Acute chest syndrome
Aplastic
Haemolytic
358
Q

Why might a patient being treated for leukaemia develop hip pain?

A

Steroids can cause avascular necrosis, in this case of the femoral head

359
Q

What are the three main triggers of thrombotic/painful crises?

A

Infection
Dehydration
Hypoxia

360
Q

What are the features of a thrombotic/painful crisis?

A

Microvascular occlusion causes severe pain

Infarcts occur in various organs:

  • Bones (eg avascular necrosis of hip - Perthe’s)
  • Hand-foot syndrome (children)
  • Lungs
  • Spleen
  • Brain
361
Q

What are the features of a sequestration crisis?
Who is most affected?
How is it managed?

A

Sickling within organs (eg spleen, liver)
Pooling of blood + worsening anaemia
Mainly affects children as spleen has not yet undergone atrophy
Splenomegaly, hepatomegaly
Severe anaemia and shock
Requires urgent transfusion

362
Q

What are the key clinical features of acute chest syndrome?

A

Dyspnoea
Chest pain
Pulmonary infiltrations
Low pO2

363
Q

What are the features of aplastic crisis?

A

Triggered by parvovirus B19 (slapped cheek)

Sudden reduction in marrow production, esp RBCs

Sudden drop in Hb

Usually self-limiting (2 weeks) but may need transfusion

364
Q

How is an acute sickle cell crisis managed?

A

ABCDE
Analgesia (eg IV opioids)
X-match blood, FBC, retics, cultures, CXR if febrile/chest Sx
Rehydrate with IVI and keep warm
HiFlow O2
Ceftriaxone if T >38
Transfusion if Hb falls sharply (aim HbS <30%)

365
Q

What is hyperviscosity syndrome?

A

Increase in blood viscosity due to raised Ig produced by malignant clones of plasma cells (eg myeloma)

OR (rare): heavy white cell loads in leukaemia

366
Q

What are the causes of hyperviscosity syndrome?

A

Increased antibodies

1) Myeloma
2) Waldenstrom’s macroglobulinaemia

Increased WBCs

1) CML
2) AML
3) ALL

Increased RBCs
1) Polycythaemia

367
Q

Clinical presentation of hyperviscosity syndrome?

A 
Lethargy
Headaches
Confusion
Cranial nerve defects
Ataxia
Retinal haemorrhages
Dyspnoea and cough (pulmonary leukostasis - infiltrates on CXR)
Mottling of skin
368
Q

How is hyperviscosity diagnosed?

A 
Clinical diagnosis
Plasma viscosity level
CT head to exclude other causes of neurological signs
Globulin level
FBC + Ig levels
369
Q

How is hyperviscosity managed?

A

1) Plasmapheresis (if raised Ig level)
2) Leucophoresis (if raised WBCs)

Avoid blood transfusions
Urgently start appropriate chemo

Medicine - Finals (13 decks)

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