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Phase 3B > Haematology > Flashcards

Haematology Flashcards

1
Q

What is multiple myeloma?

A

-Cancer of the bone marrow plasma cells (differentiated B lymphocytes)

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2
Q

What is the peak age that pts present with multiple myeloma?

A

-60-70 years

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3
Q

What is the mneumonic to remember the features of multiple myeloma?

A 
-CRAB
>HyperCalcaemia
>Renal impairment
>Anaemia
>Bone lesions/disease
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4
Q

What are some other features (other than CRAB) that multiple myeloma may cause?

A
  • Bone disease: bone pain, osteoporosis, pathological fractures
  • Lethargy
  • Infection
  • Paraprotein production
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5
Q

What are the symptoms of myeloma that pts present with?

A 
-Osteolytic bone lesions cause:
>back ache
>pathological fractures
-Hypercalcaemia
>moans, groans and bones
-Frequent infections
-Symptoms of anaemia
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6
Q

What should be checked in a pt >50 with back pain?

A
  • Serum protein elctrophoresis

- ESR

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7
Q

What investigations should be done for multiple myeloma?

A
  • FBC
  • Monoclonal proteins in serum and urine
  • Bone marrow biopsy
  • Blood film
  • Imaging: XR, CT/MRI
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8
Q

What would an FBC show in someone with multiple myeloma?

A

-Anaemia

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9
Q

Which immunoglobulins would be found in a serum monoclonal proteins in someone with multiple myeloma?

A
  • IgG

- IgA

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10
Q

What urine monoclonal proteins would be seen in someone with multiple myeloma?

A

-Bence Jones proteins

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11
Q

What would a bone marrow biopsy show in a pt with myeloma?

A

-Increase plasma cells within the bone marrow

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12
Q

What would a blood film show in a pt with myeloma?

A

-Roloeaux formation

>stacking of red blood cells

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13
Q

What would an xray show of someone with myeloma?

A

-Lytic ‘punched out’ lesions

>rain-drop skull

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14
Q

What are the diagnostic criteria for Myeloma?

A

-One major and one minor criteria

OR
-3 minor criteria
in a pt with features of myeloma

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15
Q

What are the major criteria for diagnosis of myeloma?

A
  • Plasmacytoma (demonstrated on biopsy specimen)
  • 30% plasma cells in bone marrow sample
  • Elevated levels of myoclonal protein in blood or urine
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16
Q

What are the minor criteria for myeloma diagnosis?

A
  • 10-30% plasma cells in bone marrow sample
  • Minor elevations in the level of monoclonal protein in blood or urine
  • Osteolytic lesions in imaigng
  • Low level of antibodies in blood
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17
Q

What are some poor prognostic features of 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 principles of management for myeloma?

A

-Treat symptomatic pts only

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19
Q

How are asymptomatic myeloma pts managed?

A

-regular monitoring

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20
Q

How is symptomatic myeloma managed?

A
  • Stem cell transplant

- Intensive drug treatment (chemo)

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21
Q

What chemo regimes are used to treat symptomatic myeloma?

A

-Thalidomaide or bortezomib with dexamethasone

> supportive treatment: analgesia, bisphosphonates, blood transfusions

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22
Q

Which pts are considered candidates for a stem cell transplant?

A
  • Pts under 65 of fit pts under 70
  • Assess general fitness, diagnosis, stage of disease
  • Younger and physically fitter people do better
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23
Q

What is monoclonaL gammopathy of undetermined significance?

A

-Also known as benign paraporteinaemia and monoclonal gammopathy
>10% develop myeloma at 5 years
>50% develop myeloma at 15 years

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24
Q

What are the features of MGUS?

A
  • Usually asymptomatic

- 10-30% of pts have demyelinating neuropathy

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25
Q

How can MGUS be differentiated from myeloma?

A
  • Normal immune function
  • Normal beta-2 macroglobulin levels
  • Lower level or paraproteinemia than myleoma
  • Stable level of paraproteinaemia
  • No clinical features of myeloma
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26
Q

What is lymphoma?

A

-Malignant proliferation of lymphocytes
>accumulate in lymph nodes and cause lymphadenopathy
>can be found in the peripheral blood or other organs (bone marrow, spleen)

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27
Q

What are the different type of lymphoma?

A
  • Hodgkin’s

- Non-Hodgkins

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28
Q

What are the causes of lymphoma?

A
  • Most cases unknown
  • Infection
  • Primary immunodeficiency
  • Secondary immune deficiency ie HIV, transplant pts
  • Autoimmune disorders
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29
Q

What are some examples of infections that cause lymphoma?

A
  • EBV
  • HTLV-1
  • Helicobacter pylori
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30
Q

What are the symptoms of Hodgkin’s lymphoma?

A
  • Enlarged, painless, non-tender, ‘rubbery’ superficial lymph nodes
  • Asymmetrical
  • Usually cervical but can be axillary or inguinal
  • May fluctuate in size
  • Alcohol induced lymph node pain
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31
Q

What are the constitutional B symptoms that present with Hodgkin’s lymphoma?

A
  • Pel-Ebstein fever (rises abruptly, stays high for a week and then drops for a week)
  • Weight loss
  • Night sweats
  • Pruritus
  • Lethargy
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32
Q

When does Hodgkin’s lymphoma typically present?

A

-3rd and 7th decade

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33
Q

What are the signs of Hodgkin’s lymphoma?

A
  • Painless lymphadenopathy
  • Normocytic anaemia, eosinophillia, raised LDH on blood tests
  • Hepatosplenomegaly
  • Cachexia
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34
Q

What investigations should be done for lymphoma?

A
  • Lymph node biopsy
  • Imaging (for staging)
  • Bloods: FBC, film, LFT, LDH
  • Immunophenotyping
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35
Q

What blood test results would indicated a poorer prognosis in someone with lymphoma?

A
  • Raised ESR
  • Low HB
  • Raised LDH due to increased cell turnover
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36
Q

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

A

-Reed-Sternberg cells

>mirror image nuclei appearance

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37
Q

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

A

-Excision of a complete Lymph node - submitted for detailed histiological evaluation

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38
Q

What staging system is used to classify lymphoma?

A

-Ann Arbor classification

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39
Q

What are the stages of Ann Arbor classification?

A

-Stage 1 = confined to one lymph node region
-Stage 2 = confined to 2 or more lymph node regions on the same side of the diaphragm
-Stage 3 = affecting lymph node regions on both sides of the diaphragm
-Stage 4 = spread beyond lymph nodes (e.g. liver, bone marrow)
*Each stage is then split further into
A – no systemic symptoms other than pruritis
B – B symptoms present - weight loss, fever, night sweats

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40
Q

What are the 4 subtypes of Hodgkin’s lymphoma?

A
  • Nodular sclerosing Hodgkin lymphoma (most common)
  • Mixed cellularity Hodgkin lymphoma
  • Lymphocyte depleted Hodgkin lymphoma (worst prognosis)
  • Lymphocyte rich classical hodgkin lymphoma (best prognosis)
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41
Q

What are poor prognostic factors for lymphoma?

A
  • Male sex
  • Increasing age
  • Stage 4 disease
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42
Q

How is Hodgkin’s lymphoma treated?

A
  • Stage 1-2A: short course combination chemo and radi

- Stage 2B: 4 combination chemo

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43
Q

What drugs are used for combination chemotherapy?

A 
-ABVD
>Adriamycin
-Bleomycin
-Vinblastine
-Decarbazine
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44
Q

What are some side effects of ABVD chemo?

A
  • Infertility
  • Doxorubicin: cardiomyopathy
  • Bleomycin: lung damage
  • Vinblastine: peripheral neuropathy
  • Secondary cancers
  • Psychological issues
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45
Q

What are some features of Non-Hodgkin’s lymphoma?

A
  • Median age: 55-60years
  • Painless widespread lymphadenopathy
  • Hepatosplenomegaly
  • Raised LDH
  • Paraproteinaemia
  • Autoimmune haemolytic anaemia
  • Extra-nodal disaese
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46
Q

What are the subtypes of NHL?

A
  • Indolent or low grade NHL (follicular)

- High grade or aggressive NHL (diffuse large B cell)

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47
Q

What are the features of indolent/low grade NHL?

A
  • Slow growing, usually advanced at presentation
  • Incurable
  • Median survival 9-11 years
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48
Q

What are the features of high grade or aggressive NHL?

A
  • Usually nodal presentation
  • 1/3 cases have extranodal involvement
  • Pt unwell, short history, poor prognosis
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49
Q

What are some extra-nodal features of NHL?

A
  • Skin > cutaneous T cell lymphomas
  • Oropharynx > Waldeyer’s ring lymphoma (sore throat/obstructed breathing)
  • Gut > gastric MALT, small bowel lymphomas
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50
Q

What is the classic treatment regime seen for NHL?

A 
-R-chop
>Rituximab
>Cyclophosphamide
>Hydroxydaunorubicin
>Oncovin (vincristine
>Prednisolone
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51
Q

What is the role of rituximab in NHL?

A
  • Monoclonal antibody

- Anti- CD20

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52
Q

How does anti-CD20 work in the context of rituximab and lymphoma?

A
  • Anti-CD20 targets CD20 on cell surgace of B cells and kills these cells by antibody-directed cytotoxicity
  • Sensitises the CHOP part of R-CHOP
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53
Q

What are some poor prognostic indicators for NHL?

A
  • Age >60
  • Systemic symptoms
  • Bulky disease is abdo mass >10cm
  • Raised LDH
  • Disseminated disease at presentation
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54
Q

What are some possible acute presentations of lymphoma?

A

-Infection
-SVC obstruction
>Sensation of ‘fullness in the head’
>Dyspnoea
>Black outs
>Facial oedema

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55
Q

What is leukaemia?

A

-Malignant proliferation of haemopoietic cells

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56
Q

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

A
  • Acute myeloid leukaemia (AML) - cancer of myeloblasts
  • Chronic myeloid leukaemia (CML) - cancer of basophils, neutrophils and eosinophils
  • Acute lymphoblastic leukaemia (ALL) - cancer of lymphoblasts (precursors to B and T cells)
  • Chronic lymphocytic leukaemia (CLL) - cancer of B lymphocytes
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57
Q

What is acute myeloid leukaemia?

A
  • Clonal expansion of myeloblasts

- Most common acute leukaemia in adults

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58
Q

What are risk factors for AML?

A
  • Down’s syndrome
  • Age
  • Previous chemo or exposure to radiation
  • Myelodysplasia
  • Myeloproliferative disorders
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59
Q

What are some examples of myeloproliferative disorders that can result in AML?

A
  • CML
  • Polycythaemia rubra vera
  • Essential thrombocythaemia
  • Myelofibrosis
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60
Q

What is myelofibrosis?

A

-Myeloproliferative disorder thought to be caused by hyperplasia of abnormal megakaryocytes
>release of platelet derived growth factor stimulates fibroblasts = haematopoiesis develops in the liver and spleen

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61
Q

What are the features of myelofibrosis?

A
  • Elderly person with symptoms of anaemia (usually fatigue)
  • Massive splenomegaly
  • Hypermetabolic symptoms ie weight loss, night sweats
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62
Q

What are the diagnostic findings of myelofibrosis?

A
  • Anaemia
  • High WBC and platelet count early in the disease
  • ‘Tear drop cells’ on blood film
  • Unobtainable marrow biosy
  • High urate and LDH
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63
Q

How does AML present?

A 
-Symptoms of bone marrow failure
>anaemia
>neutropenia
>thrombocytopenia
>infiltration (hepatosplenomegaly, gum hypertrophy)
>bone pain
>fever
>skin involvement
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64
Q

Why might WCC be high but someone with AML get frequent infections?

A

-There may be a high level of WCC, but number of functioning WC are low

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65
Q

What are some poor prognositc features of AML?

A
  • > 60 years
  • 20% blasts after 1st course of chemo
  • Cytogenetics: deletions of chromosome 5 and 7
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66
Q

How can AML be classified?

A

-French-American-British classification

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67
Q

What is acute pre-myelocytic leukaemia?

A
  • Associated with t(15;17)
  • Fusion of PML and RAR-alpha genes
  • Presents younger that other types of AML
  • Good prognosis
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68
Q

What type of cells are seen in someone with acute pre-myelocytic leukaemia?

A
  • Auer rods

- Seen with myeloperoxidase stain

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69
Q

How does pre-myelocytic leukaemia present?

A

-DIC/thrombocytopenia

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70
Q

How is AML treated?

A
  • Supportive therapy
  • Chemotherapy: daunorubicin, cytarbine
  • Allogenic bone marrow Transplantation (with cyclophosphamide to kill all leukaemic cells pre-transplant)
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71
Q

Which drugs are used to prevent graft vs host disease post transplant in someone with AML?

A

-Cyclosporin and methotrexate

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72
Q

What is chronic myeloid leukaemia?

A
  • Uncontrolled clonal proliferation of myeloid cells
  • Presents between 60-70 years
  • Slight male predominance
  • Slow onset
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73
Q

What causes CML?

A

-associated with the PHILADELPHIA CHROMOSOME
>t(9:22)
-Those without philadelphia chromosome have worse prognosis

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74
Q

How does CML present?

A 
-Mostly chronic and insidious. Some detected incidentally
>Weight loss
>Tiredness
>Fever and sweats
>Bleeding
>Abdo discomfort (splenomegaly)
>gout (purine breakdown)
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75
Q

What investigations should be done for CML?

A
  • Raised WCC
  • Decreased or normal Hb
  • Variable platelets
  • Urate increased
  • B12 increased
  • Bone marrow biopsy (hypercellular)
  • Cytogenic analysis of blood or bone marrow showing Ph Chromosome
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76
Q

How is CML treated?

A
  • IMATINIB (tyrosine kinase inhibitor)
  • Hydroxycarbamide
  • Interferon-alpha
  • Allogenic bone marrow transplant
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77
Q

What are the phases of CML?

A
  • Chronic: lasting months/years, few/no symptoms
  • Accelerated phase: increase in symptoms, spleen size, difficulty controlling blood cell counts
  • Blast transformation: features of acute leukaemia and death
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78
Q

What is the prognosis of CML?

A

-Survival >90% at 5 years

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79
Q

What is the epidemiology and aetiology of acute lymphoblastic leukaeamia?

A
  • Most common malignancy affecting children
  • Peak incidence 2-5 years
  • Boys affected> girls
  • Genetic susceptibility and environmental triggers
  • CNS involvement is common
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80
Q

What are the risk factors/assocaitions with ALL?

A
  • Down’s syndrome

- Ionising radiation ie xrays during pregnancy

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81
Q

What are the types of ALL?

A
  • Common ALL (75%) - CD10 present, pre-B phenotype
  • T cell (20%)
  • B-cell (5%)
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82
Q

What are the features of ALL?

A 
-Bone marrow failure features:
>anaemia
>neutropenia
>thrombocytopenia
-Other
>bone pain (2ndry to bone marrow infiltration)
>Hepatosplenomegaly
>Lymphadenopathy
>Testicular swelling
>Neurological (cranial nerve palsies, meningism)
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83
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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84
Q

What tests are done for ALL?

A
  • Blood film and bone marrow biopsy (shows blast cells)
  • WCC - v high
  • CRX/CT: mediatsinal and abdo LN involvement
  • LP: for CNS involvement
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85
Q

How is ALL treated?

A
  • Supportive
  • Infection management
  • Chemotherapy
  • Matched related allogenic marrow transplants
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86
Q

What kind of supportive therapy is given to someone with ALL?

A
  • Blood/platelet transufsion
  • IV fluids
  • Allopurinol (prevents tumour lysis syndrome)
  • Hickman line for IV access
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87
Q

What infection management is given to someone with ALL?

A
  • Immediate IV abx
  • Antifungals
  • Antivirals
  • Antifungals
  • Co-trimoxazole for PCP
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88
Q

What are some poor prognostic factors for ALL?

A
  • Age <2 or >10
  • WCC >20 at diagnosis
  • T or B cell surface markers
  • Non-Caucasians
  • Male sex
  • Philadelphia chromosome present
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89
Q

Which features in a person aged 0-24 should prompt a very urgent (within 48hrs) 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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90
Q

When should someone between 0-24 years be referred urgently for assessment for leukaemia?

A
  • Unexplained petechiae

- Hepatosplenomegaly

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91
Q

What is chronic lymphocytic leukaemia?

A
  • Most common leukaemia
  • Grandual accumulation of B lymhocytes in the blood, BM, spleen and LN
  • Often incidental finding on FBC, can be anaemic or infection prone
  • If severe: weight loss, sweats, anorexia
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92
Q

How is CLL more likely to affect?

A
  • Elderly

- Males

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93
Q

What are the features or CLL?

A
  • Often none
  • Constiutional: anorexa, weight loss
  • Bleeding + infections
  • Lymphadenopathy more marked than in CML
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94
Q

What is the clinical course of CLL?

A

-Variable
-Progressive lymphadenopthy and hepatosplenomegaly
-Autoimmune features ie haemolysis, ITP
-Bone marrow failure
>hypogammaglobulinaemia + infection
-Death often due to infection

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95
Q

Which infections are commonly associated with mortality in someone with CLL?

A
  • Pneumococcus
  • Haemophilus
  • Meningococcus
  • Candida
  • Aspergillus
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96
Q

What investgations are done to diagnose CLL?

A
  • Blood film: smudge cells

- Immunophenotyping

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97
Q

What are complications of CLL?

A
  • Hypogammaglobulinaemia > recurrent infections
  • Warm autoimmune haemolytic anaemia
  • Transformation to high-grade lymphoma (richter’s transformation)
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98
Q

What is Richter’s transformation?

A
  • Occurs when leukaemia cells enter the lymph node and change into high-grade, fast growing non-Hodgkin’s lymphoma
  • Pt suddenly becomes very unwell
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99
Q

What are the symptoms of Richter’s transformation?

A
  • Lymph node swelling
  • Fever without infection
  • Weight loss
  • Night sweats
  • Nausea
  • Abdominal pain
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100
Q

How is CLL staged?

A

-Binet staging
>A: lymphocytosis +/- 3 nodal areas
>B: 3 + nodal areas
>C: anaemia and/or thrombocytopenia

101
Q

How is CLL treated?

A
  • Do nothing
  • Chemotherapy
  • Monoclonal antibodies ie anti-CD20 rituximab
  • Targeted therapy
  • Bone marrow transplant
  • Supportive care ie transfusions, IVIG for recurrent infections
102
Q

What is the prognosis of CLL?

A
  • 1/3 never progress
  • 1/3 progress slowly
  • 1/3 progress actively
103
Q

What’s the difference between autologous and allogenic stem cell transplants?

A
  • Autologous: enables escalation of chemo with stem cell ‘rescue’
  • Allogenic: More toxic, stem cells attack residual tumour and recipient. - can cause graft vs host disease
104
Q

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

A

-Starry sky appearance

105
Q

What is burkitt lymphoma?

A
  • Rapidly proliferating B cell tumour
  • Can present in weird ways due to rapid proliferation ie nerve root compression
  • Ass. w/ EBV
106
Q

What is Waldernstrom’s macroglobulinaemia?

A
  • Uncommon condition seen in older men
  • Lympholpasmacytoid malignancy characterised by secretion of a monoclonal IgM paraprotein
  • Paraproteinaemia cause hyperviscosity of blood = ^ risk of ischaemic stroke
107
Q

What are the features of Waldenstom’s macroglobulinaemia?

A
  • Monoclonal IgM paraproteinaemia
  • Systemic upset
  • Hyperviscosity syndrome: visual disturbance, stroke, headahcehes, spont. bleeding from mucus membranes
  • Hepatosplenomegaly
  • Lymphadenopathy
  • Cyroglobulinaema (Raynaud’s)
108
Q

Define anaemia

A
  • Low hb concentration

- >either due to a low red cell mass or increased plasma volume ie in pregnancy

109
Q

What are the normal values of Hb in men and women?

A

-Males: 13.1-16.6g/dl
>low Hb <135g/L
-Females: 11-14.7g/dl
>Low Hb < 115g?L

110
Q

What are the symptoms of anaemia?

A
  • Fatigue
  • Dyspnoea
  • Faint
  • Palpitations
  • Headaches
  • Tinnitus
  • Anorexia
  • Angina
111
Q

What are the signs of anaemia?

A

-Pallor: conjunctiva, palmar creases
-Hyperdynamic circulation in severe anaemia:
>Tachycardia
>Flow murmur
>Cardiac enlargement
>retinal haemorrhage
>Heart failure (rare)

112
Q

What are the causes of microcytic anaemia?

A
  • Iron deficiency
  • Thalassaemia (reduced/faulty
  • Chronic disease (normally normocytic)
  • Lead poisoning
113
Q

What is iron deficiency anaemia?

A
  • Microcytic anaemia due to lack of iron
  • Common
  • Seen in 14% of menstruating women
114
Q

What ar ethe causes of iron deficiency anaemia?

A
  • Blood loss
  • Insufficient dietary intake
  • Malabsorption ie Coeliac disease
115
Q

What are the signs of Iron deficiency anaemia?

A
  • Kolionychia (spoon shaped nails)
  • Atrophic glossitis (big red tongue)
  • Angular stomatitis (sores/ulceration in corner of mouth)
  • Post-cricoid webs (Plummer vision syndrome)
116
Q

What is seen on a blood film for Iron deficiency anaemia?

A
  • Target cells
  • Pencil poikilocytes
  • If combines with B12/folate deficiency - dimorphic film occurs with mixed micro and macrocyctic cells
117
Q

How is iron deficiency anaemia treated?

A
  • Treat the cause: oral iron ie ferrous sulphate
  • Continue until Hb is normal + for at least 3/12 to replenish stores
  • Iron deficiency with no obvious source of bleeding = careful GI workup
118
Q

What are the side effects of ferrous sulphate?

A
  • Nausea
  • Abdominal pain
  • Diarrhoea or constipation
  • Black stools
119
Q

What is anaemia of chronic disease?

A 
-Most common cause of anaemia in hospital pts
>Chronic infection
>Vasculitis
>RA
>Malignancy
>Renal failure
120
Q

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

A
  • Normocytic anaemia
  • Ferritin normal or raised
  • Check haemoatinics as causes of chronic anaemia are usually multifactoral
121
Q

How is anaemia of chronic disease treated?

A
  • Treat underlying disaese vigorously - achieve optimum therapy
  • EPO: effective at raising Hb level and improves QoL in cancer pt
  • Iron (give parenterally)
  • Hepcidin inhibitors and inflammatory modulators
122
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 nucelus = sideroblast
  • Congenital or acquired
  • Consider when microcytic anaemia doesn’t respond to iron
123
Q

What are the causes of sideroblastic anaemia?

A

-Congenital
-Acquired:
>Myelodysplasia
->Alcohol
>Lead
>Anti-TB meds

124
Q

How is sideroblastic anaemia diagnosed?

A
  • Hypochromic microcytic anaemia

- Bone marrow: sideroblasts and increased iron stores

125
Q

How is sideroblastic anaemia managed?

A
  • Supportive
  • Treat any underlying cause
  • Pyridoxine (vit B6) may help
  • Transfusion for severe anaemia
126
Q

What are the causes of normocytic anaemia?

A
  • Bleeding
  • Chronic disease
  • Pregnancy
  • Sickle cell disease
  • CKD
  • Haemolytic anaemia
  • Aplastic anaemia
  • Bone marrow failure (suspect if low WCC and platelets
  • Combined haematinic deficiency
  • Hypothyroidism
127
Q

What are the causes of megalosblastic macrocytic anaemia?

A
  • B12 deficiency
  • Folate deficiency
  • Cytotoxics ie hydroxycarbaminde, phenytoin
128
Q

What are the causes of non-megaloblastic causes of macrocyctic anaemia?

A
  • Alcohol
  • Reticulocytosis
  • Liver disease
  • Hypothyroidism
  • Pregnancy
129
Q

What are some haematological disease causes of macrocytic anaemia?

A
  • Myelodysplasia
  • Myeloma
  • Myeloproliferative disorders
  • Aplastic anaemia
130
Q

What will blood tests show for macrocytic anaemia?

A

-Blood film: hypersegmented polymorphs in B12 and folate deficiency
-Bone marrow biopsy
>megalobastic RBC
>Normoblastic marrow (liver disease, hypothyroid)
>Abnormal erythropoiesis (sideroblastic, leukaemia, aplasia)
>Increased erythropoiesis ie haemolysis

131
Q

In what foods is folate found?

A
  • Green veg
  • Nuts
  • Yeast
  • Liver
132
Q

What does maternal folate deficiency cause in a fetus?

A

-Neural tube defects

133
Q

How is folate absorbed?

A
  • Duodenum

- Proximal jejunum

134
Q

What are the causes of folate deficiency?

A
  • Poor diet: poverty, alcoholics, elderly
  • Increased demand: pregnancy, tapeworm, increased cell turnover
  • Malabsorption: coeliac
  • Drugs: anti-epileptics, trimethooprim, methotrexate
  • Alcohol
135
Q

What drugs cause folate deficiency?

A
  • Anti-epileptics: phenytoin, valproate
  • Trimethoprim
  • Methotrexate
136
Q

What is the role of folate in a) heart disease and b) cognition?

A
  • a) folate lowers homocyteine levels = decreased risk of CVD
  • b) benefits cognition
137
Q

How is folate deficiency treated?

A

-Assess fro underlying cause
-Treat with oral folic acid (5mg/day)
-Prophylaxis in pregnancy (400mcg/day) from conception until at least 12/40
>high risk pregnany -> 5mg/day

138
Q

Which foods have B12 in?

A
  • Meat
  • Fish
  • Dairy products
139
Q

Where is B12 absorbed?

A

-Binds to intrinsic factor in the stomach and complex is absorbed in the terminal ileum.

140
Q

Why does B12 deficiency cause anaemia?

A

-Involved within in the synthesis of thymidine (hence DNA) = if impaired, RBC production is slow

141
Q

What are the causes of B12 deficiency?

A

-Dietary (vegans)
-Malabsorption
>stomach: lack of intrinsic factor ie post gastrectomy, pernicious anaemia
>Terinal ileum: resectio, Crohn’s, tapewrom
>Congenital metabolic errors

142
Q

What are teh features of B12 deficiency?

A

-Normal features of anaemia
-Neuropsychiatric:
>irritability
>depressino
>psychosis
>dementia
-Neurological:
>paraesthesia
>peripheral neuropathy
>subacute degeneration of spinal cord

143
Q

What are the features of subacute spinal cord degeneration?

A
  • Classic triad of:
    1. Extensor plantars (UMN)
    2. Absent knee jerks (LMN)
    3. Absent ankle jerks (LMN)
144
Q

What is pernicious anaemia?

A
  • Caused by an autoimmune atrophic gastritis leading to achlorhydria (absence of HCl) and lack of intrinsic factor secretion
  • Usually develops in middle-old age in females
145
Q

What are some associations with pernicious anaemia?

A
  • Thyroid disease
  • T1DM
  • Addison’s disease
  • RA
  • Vitiligo
  • Hypoparathyroidism
  • Predisposes to gastric carcinoma
146
Q

What are some features of pernicious anaemia?

A
  • Lethargy
  • Weakness
  • Dyspnoea
  • Paraesthesia
  • Mild jaundcie, diarrhoea, sore tongue
147
Q

What are some specific investigations for pernicious anaemia?

A
  • Parietal cell antibodies

- Intrinsic factor antibodies

148
Q

How is pernicious anaemia/B12 deficiency treated?

A
  • Treat underlying cause
  • B12 injections: hydroxycobalamin for malabsorption problems
  • Oral B12 if definitely dietary cause
149
Q

What is aplastic anaemia?

A

-Characterised by pancytopenia and hypoplastic bone marrow

150
Q

What are the features of aplastic anaemia?

A
  • Normochromic, normocytic anaemia
  • Leukopenia with lymphocytes relatively spared
  • Thrombocytopenia
  • Can be presenting feature of ALL or AML
  • Minority of pts develop paroxysmal nocturnal haemoaglobinuria/myelodysplasia
151
Q

What are the causes of aplastic anaemia?

A
  • Idiopathic
  • Congenital
  • Drugs
  • Toxins ie benzene
  • Infections ie parvovirus, hepatitis
  • Radiation
152
Q

What medications can cause aplastic anaemia?

A
  • Phenytoin
  • Cytotoxics
  • Chloramphenicol
  • Sulphonamides ie sulphonylureas, anticonvulsants,sulfasalazine
153
Q

What are the hereditary causes of haemolytic anaemia?

A
  • Membrane: hereditary spherocytosis/elliptocytocsis
  • Metabolism: G6PD deficiency
  • Haemoglobinopathies: sickle cell, thalassaemia
154
Q

What are the acquired causes of haemolytic anaemia? (Immune causes)

A

> autoimmune
alloimmune (transfusion reaction)
drugs: methyldopa, penicillin
ia?

155
Q

What are the acquired causes of haemolytic anaemia? (Non-immune causes)

A 
>microangopathic:
-TTP/HUS, DIC, malignancy
>prosthetic cardiac valves
>Paroxysmal nocturnal haemaglobinuria
>Infections ie malaria
.Drugs
156
Q

What is G6PD deficiency?

A

-Most common RBC enzyme defect?
>X-linked recessive (affecting males)
-Deficiency in G6PD causing anaemia
-Drugs, infections and fava beans can precipitate a crisis

157
Q

What are the features of G6PD deficiency?

A
  • Neonatal jaundice
  • Intravascular haemolysis
  • Gallstones
  • Splenomegaly
  • Heinz bodies on blood film
158
Q

Which drugs can precipitate haemolysis in G6PD deficiency?

A
  • Anti-malarials
  • Ciprofloxacin
  • Sulphonamides
  • Sulphalazine
  • Sulfonylureas
159
Q

How is G6PD deficiency diagnosed?

A

-G6PD enzyme assay

160
Q

What is hereditary spherocytosis?

A
  • Most common hereditary anaemia
  • Autosoma dominant
  • Normal biconcave disc shape of FBC replaced by sphere-shaped one
  • RBC survival reduced as destroyed by spleen
161
Q

How does hereditary spherocytosis present?

A
  • Failure to thrive
  • Jaundice, gallstones
  • Splenomegaly
  • Aplastic crisis precipitated by parvovirus infection
  • Spherocytes on blood film
162
Q

How is herditary spherocytosis diagnosed?

A
  • Osmotic fragility test

- Spherocytes on blood film - round, lack of central pallor

163
Q

How is hereditary spherocytosis treated?

A
  • Folate replacement

- Splenectomy

164
Q

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

A
  • Transfusion threshold in pts without ACS - 70g/L

- Transfusion threshold in pts with ACS - 80g/L

165
Q

Why is the transfusion threshold lower in ACS?

A

-Anaemia can exacerbate ACS because there is less Hb to carry oxygen - heart has to work harder

166
Q

What are some complications of blood transfusions?

A
  • Immunological: acute haemolysis, allergy/anaphylaxis
  • Infections
  • Transfusion-related lung injury
  • Fluid overload
  • Hyperkalaemia
  • Iron overload
  • Clotting
167
Q

What causes an acute haemolytic transfusion reaction?

A
  • Mismatch of ABO blood group
  • Causes massive intravascular haemolysis
  • Symptoms begin minutes after transfusion starts: fever, abdo and chest pain, agitation and hypotension
168
Q

What is the treatment for acute a hemolytic transfusion reaction? What are the complications?

A
  • Immediate transfusion termination, generous fluid resus, inform lab
  • Complications: DIC, renal failure
169
Q

What causes a non-haemolytic febrile transfusion reaction?

A
  • White blood cell HLA antibodies

- Often the result of sensitisation by previous pregnancies/transfusions

170
Q

What causes an allergic/anaphylactic blood transfusion reaction?

A

-Hypersensitivity reaction to components within the transfusion
-Symptoms arise within minutes and vary in severity:
>urticaria, anaphylaxis, hypotension, wheezing, dyspnoea, stridor

171
Q

How is allergic/anaphylactic blood transfusion reaction treated?

A
  • Simple urticaria: discontinue transfusion + antihistamines. Transfusion can continue when symptoms resolve
  • More severe reaction: treat urgently with antihistamine, corticosteroids and bronchodilators. Permanently stop transfusion
172
Q

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

A

-Bacterial infections

>due to platelets being stored in room temp

173
Q

What is the universal donor blood group for a) rbc b) FFP?

A
  • RBC: O Rh -ve

- FFP: AB Rh -ve

174
Q

> 2 units of blood can result in fluid overload, How can this be avoided?

A

-Prescribe frusemide in between transfusion of each unit

175
Q

What are the features of post-thrombotic syndrome?

A
  • Painful, heavy calves
  • Pruritus
  • Swelling
  • Varicose veins
  • Venous ulceration
  • Management: compression stockings
176
Q

What is the most common a) clotting disorder and b) bleeding disorder?

A

a) factor V leiden (activated protein C deficiency)

b) Von Willebrand disease

177
Q

How long should someone be on warfarin following a DVT for?

A
  • Provoked DVT ie recent surgery: 3 months

- Unprovoked: 6 months

178
Q

What is the MOA for the NOACs, heparin and warfarin?

A
  • Rivaroxiban and apixaban: direct factor Xa inhibitor
  • Dabigatran: direct thrombin inhibitor (factor 2a)
  • Heparin: activates antithrombin III
  • Warfarin: inhibits 10,9,7,2
179
Q

What antibiotic is recommended in an episode of neutropenic sepsis?

A

-Piperacillin with Tazobactam (tazocin)

180
Q

What are the 2 main classifications of polycythaemia?

A
  • Primary/proliferative: ie polycythaemia rubra vera

- Secondary: reactive

181
Q

What are some causes of secondary polycythaemia?

A

-Altitude
-COPD/other lung disease
-Smoking
-Cyanotic heart disease
-Obstructive sleep apnoea
-EPO/androgen excess
>cerebellar haemangioma
>hypernephroma
>hepatoma
>doping

182
Q

What is polycythaemia rubra vera?

A
  • Myeloporoliferative disorder (overactive bone marrow)
  • Caused by clonal proliferation of marrow stem cells leading to an increased in red cell volume, often accompanied by overproduction of neutrophils and platelets
  • JAK2 MUTATION
183
Q

Which mutation is associated with polycythaemia rubra vera?

184
Q

What are the features of polycythaemia vera?

A
  • Hyperviscosity
  • Thrombosis
  • Pruritus (esp. after hot bath)
  • Splenomegaly
  • Haemorrhage
  • Plethoric appearance
  • Hypertension
  • Gouty arthritis
185
Q

How is polycythaemia vera investigated?

A
  • FBC: raised haematocrit
  • JAK2 mutation test
  • Serum ferritin
  • Renal and liver function tests
186
Q

How is polycythaemia verta treated?

A
  • Aspirin
  • Venesection
  • Bone marrow suppression (hydroxycarbamide)
187
Q

What is sickle cell anaemia?

A
  • Abnormal recessive disorder causing production of abnormal globin chains
  • HbS produced
  • Carriers are protected against Falciparum malaria
  • Periods of good health with intervening crises
188
Q

How is chronic sickle cell disease managed?

A
  • Hydroxycarbamide to suppress bone marrow if frequent crises
  • Abx and immunisation as prophylaxis for splenic infarct
  • Febrile sickle cell chicken > high risk for sepsis. Give ceftriaxone
189
Q

What is thalassaemia?

A

-Genetic disease of unbalanced Hb synthesis
(with underproduction or no production of one globin chain)
-Unmatched globins precipitate and damage RBC membranes causing haemolysis whilts they are still in the marrow

190
Q

What is beta thalassaemia?

A
  • Point mutation in the beta globin genes on chromosome 11

- Causes decreased beta chain production (B+) or absence (Bo)

191
Q

What investigations are done for beta thalassaemia?

A
  • FBC + mcv (microcytic anaemia)
  • Blood film
  • Iron
  • HbA2 and HbF raised
  • Hb electrophoresis
192
Q

How is beta thalassaemia managed?

A
  • Promotes fitness and healthy diet
  • Folate supplements
  • Regular (every 2-4 weeks) transfusion to keep Hb >90
  • Iron chelators to prevent iron overload (oral deferiprone)
  • Large doses of ascorbic acid to increase urinary iron excretion
  • Splenectomy if hypersplenism persists
  • Hormonal replacement
  • Genetic counselling
193
Q

What is alpha thalassaemia?

A

-2 separate alpha globin genes on chromosome 16
-4 genes termed aa/aa
-Gene deletions:
>(–/–) - inutero death
>(–/-a) - moderate anaemia and features of haemolysis
>(–/aa) or (-a/-a) - asymptomatic carrier
>1 deleted: clinical state is normal

194
Q

What are the clinical classifications of thalassaemia?

A
  1. Thalassaemia major - transfusion dependent
  2. Thalassaemia intermedia - less severe anaemia can survive without regular blood transfusions
  3. Thalassaemia carrier/heterozygote - asymptomatic
195
Q

What is beta thalassaemia trait?

A
  • AR
  • Mild hypochromic, microcytic anaemia:
  • HbA2 raised (>3.5%)
  • Usually aymptomatic
196
Q

What is haemophilia?

A
  • X linked recessive disorder or coagulation
  • Up to 30% of pts have no hx of condition
  • Haemophillia A - factor VIII deficiency
  • Haemophillia B (christmas disease) - factor IX deficiency
197
Q

What are the features of haemophillia?

A
  • Haemoarthroses
  • Haematomas (particular in the muscles)
  • Prolonged bleeding after surgery or trauma
198
Q

What would appear on blood tests in someone with haemophillia?

A
  • Prolonged APTT

- Normal bleeding time, thrombin time and prothrombin time

199
Q

How is haemophillia treated?

A
  • A: factor VIII replacement

- B: factor IX replacement

200
Q

How are platelets produced?

A
  • Anucleate cell fragments from megakaryocytes

- Thrombopoietin stimulates production of platelets by megakaryocytes (mainly produced in the liver)

201
Q

What are some causes of thrombocytosis? (too many platelets)

A
  • Reactive: platelets are an acute phase reactant
  • Malignancy
  • Essential thrombocytosis
  • Hyposplenism (spleen breaks down platelets, so less breakdown= more platelets)
202
Q

What is essential thrombocytosis?

A
  • Myeloproliferative disorders
  • Overlaps with CML, PRV, myelofibrosis
  • Megakaryocyte proliferation results in overproduction of platelets
203
Q

What are the features of essential thrombocytosis?

A
  • Platelet count >600
  • Thrombosis
  • Haemorrhage
  • Burning sensation in the hands (characteristic)
  • JAK2 mutation
204
Q

How is essential thrombocytosis managed?

A
  • Hydroxyurea (hydroxycarbamide)
  • Interferon alpha
  • Low dose aspirin to reduce thrombotic risk
205
Q

What are some causes of severe thrombocytopenia?

A
  • ITP
  • DIC
  • TTP
  • Haematological malignancy (marrow failure)
206
Q

What are some causes of moderate thrombocytopenia?

A
  • Heparin induced thrombocytopenia
  • Drug induced
  • Alcohol
  • Liver disease
  • Hypersplenism
  • Viral infection (EBV, HIV, hepatitis)
  • Pregnancy
  • SLE/antiphospholipid syndrome
  • Vit B12 deficiency
207
Q

What is ITP?

A
  • Immune or idiopathic thrombocytopenic purpura
  • An immune mediated reduction in platelet count
  • Antibodies are directed against the glycoprotein 2b/3a or Ib-V-IX complex
  • Acute and chronic
  • Primary and secondary
208
Q

What are the clinical features of ITP?

A

-Low platelet count on bloods
-Isolated thrombocytopenia
-Non-blanching purpruirc rash
-Easy bruising
-Evidence of mucosal bleeding
>menorrhagaia
>nose bleeds
>gum bleeding when brushing teeth

209
Q

What drugs can cause moderate thrombocytopenia?

A
  • Aspirin
  • Thiazides
  • Quinine
  • Diuretics
  • Sulphonamides
210
Q

What are the features of acute ITP?

A
  • More common in children
  • May follow infection or vaccination
  • Usually self limiting over 1-2 weeks
211
Q

What are the features of chronic ITP?

A
  • More common in young/middle aged women
  • Relapsing remitting course
  • History of autoimmune disease
212
Q

How is ITP managed?

A
  • General: stop any NSAIDs or aspirin and observe platelet counts
  • Prednisolone
  • IVIG
  • Rituximab (2nd line)
213
Q

What is Evan’s syndrome?

A

-ITP in association with autoimmune haemolytic anaemia

214
Q

What is thrombotic thrombocytopenic purprua?

A
  • Medical emergency
  • Rare form of thrombotic microangiopathy
  • Abnormal larege and sticking multimers of VWF cause platelet clumps in vessels
215
Q

WHat are risk factors for thrombotic thombocytopenic purpura?

A
  • Pregnancy/post-partum
  • HIV
  • Autoimmune disease
  • Cancer
216
Q

What are the features of TTP?

A
  • Anaemia and haemolyis
  • Thrombocytopenia
  • Fever
  • Purpura
  • Cerebral dysfunction: confusion, headache, paresis, dysarthria, visual problems
217
Q

How is TTP managed?

A
  • No abx (can worsen outcome)
  • Plasma exchange
  • Steroids
  • Immune suppressants
  • Vincristine
218
Q

What are the indications for a platelet transfusion?

A
  • Platelet count <30 with clinically significant bleeding ie haematemesis, malaena, prolonged epistaxis
  • Platelet count <100 for pts who have more severe bleeding classification or bleeding at critical sites ie CNS
219
Q

What are some contra-indications for platelet transfusion?

A
  • Chronic bone marrow failure
  • Autoimmune thrombocytopenia
  • Heparin induced thrombocytopenia
  • Thrombotic thrombocytopenia purpura
220
Q

What is DIC?

A

-Cytokine release in response to SIRS
-Causes a systemic activations of clotting cascade = microvascular thrombosis
= consumption of platelets and clotting factors which causes bleeding
-Can lead to organ failure

221
Q

What are some causes/risk factors for DIC?

A
  • Sepsis
  • Malignancy (esp. leukaemias)
  • Major trauma
  • Complications of pregnancy ie abruption, pre-eclampsia)
  • Incompatible blood transfusion
  • Transplant rejection
  • Severe liver disease
  • Pancreatitis
  • Recreational drugs
  • Snake bites
  • Connective tissue disorders
222
Q

How is DIC investigated?

A
  • Look for underlying cause
  • Clotting profile
  • D dimer: raised
  • +/- evidence of organ failure
223
Q

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

A
  • Low platelets
  • Prolonged PT
  • Prolonged APTT
  • Prolonged bleeding time
224
Q

How is DIC managed?

A

-Treat underlying cause
-Supportive provision of
>platelets
>FFP
>Cyroprecipitate

225
Q

What is Von Willebrand’s disease?

A

-Most commonly inherited bleeding disorders
-Majority of cases are AD
-Behaves like a platelet disorder
>mucosal bleeding: epistaxis, menorrhagia
>unlikely to have large joint bleeds and muscle haematomas

226
Q

What would blood investigation results show for VWD?

A
  • Prolonged bleeding time
  • APTT may be prolonged
  • Factor VIII levels - moderately reduced
  • Defective platelet aggregation with ristocetin (substance that aims to force platelet aggregation)
227
Q

How is VWD managed?

A
  • Transexaminc acid for mild bleeding
  • Desmopressin (raised levels of vWF)
  • Factor VIII concentrate
228
Q

What is heparin induced thrombocytopenia?

A
  • Development of an IgG antibody against a complex formed between platelets and heparin
  • IgG/PF4/heparin complexes bind to and activate platelets = platelet consumption increased, thrombosis, skin necrosis
229
Q

When are pts most at risk of heparin induced thrombocytopenia?

A

-After cardiac bypass surgery with large amounts of unfractioned heparin treatment

230
Q

How does heparin induced thrombocytopenia present?

A

-Sharp fall in platelets 5-10 days after starting heparin

231
Q

How is heparin induced thrombocytopenia managed?

A
  • Life threatening: stop heparin immediately

- Use alternative antioagulation even if platelets are low

232
Q

What are the main haematological emergencies?

A
  1. Neutropenic sepsis
  2. Hyperviscosity syndrome
  3. Tumour lysis syndrome
  4. Acute sickle chest syndrome
233
Q

What is tumour lysis syndrome?

A
  • Chemo causes tumour lsis syndrome due to rapid death of cells when they are responding to treatment.
  • IV rasburicase or IV allopurinol can be given pre-chemo to reduce risk
234
Q

What are the diagnostic criteria for tuour lysis syndrome?

A 
-Abnormality in >2 of the following:
>uric acid ^475 micromol
> K+ >6mmol or 25%^
>Phosphate >1.125 or 25%^
>Calcium >1.75 or 25%^
-Lab tumour lysis syndrome +:
>Increased serum creatining
>Cardiac arrhythmias or sudden death
>Seizure
235
Q

What are hte complcications of tumour lysis syndrome?

A
  • Hyperkalaemia
  • Hyperphosphataemia
  • Hypocalcaemia
  • Hyperuricaemia
  • Acute renal failure
236
Q

How is tumour lysis syndrome treated?

A
  • Aggressive IV fluid resuscitation
  • Allopurinol or rasbicurase
  • Electrolyte control
  • Early dialysis referral
237
Q

What is the definition of neutropenic sepsis?

A
  • Neutrophil count of <0.5
  • In a pt who is having anti-cancer treatment
  • Temp >38 or other signs/symptoms consistent with clinical sepsis
238
Q

What are the types of recgonised crises in sickle cell disease?

A
  • Thrombotic, vaso-occlusive, painful crises
  • Sequestration
  • Acute chest syndrome
  • Aplastic
  • Haemolytic
239
Q

What are the features of a thrombotic/painful crisis?

A

-Precipitated by: infection, dehydration, hypoxia
-Microvascular occlusion causing severe pain
-Infarcts occur in various organs:
>bones (avascular necrosis of the hip)
>Hand-foot syndrome
-Lungs
-Spleen
-Brain

240
Q

What are the features of a sequestration crisis?

A
  • Sickling within organs such as the spleen or liver
  • Causes pooling of blood with worsening anaemia
  • Mainly affects children as the spleen has not yet undergone atrophy
  • Splenomegaly, hepatomegaly
  • Severe anaemia and shock
  • Required uregnt transfusion
241
Q

What are the features of acute chest syndrome?

A
  • Dyspnoea
  • Chest pain
  • Pulmonary infiltration
  • Low p)2
242
Q

What are the features of aplastic crisis?

A
  • Occurs due to infection with parvovirus B19 (slapped cheek)
  • Sudden reduction in marrow production (esp. RBCs)
  • Sudden fall in Hb
  • Usually self-limiting within 2/52
  • May need transfusion
243
Q

What are the features of haemolytic crisis?

A
  • Rare

- Fall in Hb due to ^ rate of haemolysis

244
Q

How is acute sickle cell crisis managed?

A
  • ABCDE
  • Prompt, generous analgesia (IV opioids)
  • Crossmatch blood, FBC, reticulocytes, cultures, CXR
  • Rehydrate with IVI and keep warm
  • 02
  • Ceftriaxone if T>38
  • Transfusion if Hb falls sharply
245
Q

What is hypervisocisty synrome?

A
  • An increase in whole blood viscosity due to raised immunoglobulins produced by malignant clones of plasma cells (myeloma)
  • Sludging and lack of perfusion through microvasculature
246
Q

What are the causes of hyperviscosity syndrome?

A
  • Due to increased antibodies: myeloma, Waldenstrom’s macroglobulinaemia
  • Due to increased WBCs: CML, AML, ALL
  • Due to increased RBCs: polycythaemia
247
Q

What is the clinical presentation of hyperviscosity syndrome?

A
  • Lethargy
  • Headaches
  • Confusion
  • Cranial nerve defects
  • Ataxia
  • Retinal haemorrhages
  • Dyspnoea and cough
  • Mottling of the skin
248
Q

How is hyperviscosity diagnsed?

A
  • Clincical diagnosis
  • Plasma viscosity level
  • CT head to exclude other neuro causes of signs
  • Globulin levels
  • FBC and Ig levels
249
Q

How is hyperviscosity managed?

A
  • Plasmapharesis (if raised Ig levels)
  • Leucophoresis (if raised WBCs)
  • Avoid blood transfusions
  • Start appropriate chemo

Phase 3B (14 decks)

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