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Metabolic and Endocrine > Haematology > Flashcards

Haematology Flashcards

1
Q

what causes RBC production?

A

Tissue hypoxia -> EPO kidneys -> bone marrow ->RBC production

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2
Q

describe staining of erythrocyte?

A

has mesangial mRNA-stain blue (loses this within 2-3 days)

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3
Q

what is myelofibrosis?

A

scarring of bone marrow-splenomegaly as hematopoiesis reverts back to spleen

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4
Q

where does prenatal hematopoiesis take place?

A

yolk sac
liver
spleen
bone marrow

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5
Q

describe erythropoiesis

A

in bone marrow: pluripotenti hematopoietic stem cell -> proerythoblast ->erythroblast ->reticulocyte
in blood: reticulocyte-> erythrocyte

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6
Q

describe the symptoms of tissue hypoxia?

A

Brain-sleepy, fatigue
Heart – decreased work capacity, tachycardia, pump failure
Lung-dyspnea, respiratory reserve declines
Skeletal muscle-weakness, fatigue

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7
Q

what are the signs and symptoms of anaemia?

A

headache, dizziness, fatigue, chest pain, SOB, pallor, tachycardia, murmurs

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8
Q

what are the potential causes of anaemia?

A 
Hematuria
Prregnancy
Nutritional deficiency
GI pathologies
Menorrhagia
Haemorrhoids 
Hook worms
Iron deficiency
Red cell maturation disorders
Hemolytic anaemia
Acute bleeding 
Marrow damage
Inflammation
Neoplasia
Chronic disease
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9
Q

how is a diagnosis of anaemia made?

A

History
Examination
Laboratory (Depends on history-family history, clinical findings, ethnicity, morbidity, previous treatments, base line laboratory results)
Interpret clinical findings

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10
Q

describe microcytic anaemia?

A

Low MCV

Ferritin (either deficient- establish cause or ferritin normal-anaemia of chronic disease or hemoglobinopathy)

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11
Q

describe normocytic anaemia?

A

Normal MCV
Reticulocyte count
-High-hemolysis or blood loss
-Low-anemia of chronic disease, renal failure, marrow failure

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12
Q

describe macrocytic anaemia?

A

High MCV

Measure B12 and folate

  • Normal-obvious cause or cause not obvious so consider bone marrow
  • Low-establish cause
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13
Q

what are the dietary sources of iron?

A

red meat, certain vegetables (spinach, brocholi, iron fortified cereals.
Haem iron in meat
Non haem iron in dairy products, eggs, legumes

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14
Q

describe iron absorption?

A

Can only lose iron from bleeding
Gastric acid keeps iron soluble and iin ferrous state
Duodenum
Mucosal cells regulate quantitiy of iron absorbed

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15
Q

what are the issues with iron excess?

A

injury to organs

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16
Q

what are the signs and symptoms of iron deficiency?

A 
Hair loss
Depressed
SOB
Tired/fatigue
Restless legs syndrome
Headaches
Cold
brittle nails
Need to give iron treatment for 4-6 months
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17
Q 
describe the levels of 
1. Hb
2. other Fe functions
3. stores (SF)
in mild deficiency
A
  1. normal
  2. normal/low
  3. normal/low
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18
Q 
describe the levels of 
1. Hb
2. other Fe functions
3. stores (SF)
in marginal deficiency
A
  1. normal
  2. low
  3. low
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19
Q 
describe the levels of 
1. Hb
2. other Fe functions
3. stores (SF)
in severe deficiency
A
  1. low
  2. low
  3. low
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20
Q

describe the progressive depletion of iron stores?

A

normal-> depletion of iron stores -> iron deficiency erythropoiesis ->iron deficiency anaemia

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21
Q

how are iron levels measured?

A

Serum iron level (transferrin bound ferric iron)
Total iron binding capacity (measure of transferrin protein)
Percentage transferring saturation
Serum ferritin
Bone marrow iron stores
Plasma transferrin receptor

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22
Q

what are the causes of iron deficiency?

A 
Increased physiological demand
Blood loss
Malabsorption
Dietary deficiency
Increased demand or decreased intake/absorption
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23
Q

what are the risk factors for iron deficiency?

A

Dietary factors
Demographic factors
Social/physical factors

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24
Q

describe how a diagnosis of iron deficiency anaemia is made?

A

Microcytic hypochromic anaemia
Serum iron decreased
TIBC increased
Percentage transferrin saturation decreased
Serum ferritin decreased
Absent bone marrow haemosiderin
Reduced Hb, MCV, MCH, MCHC, serum ferritin, serum iron, TSA.blood film,

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25
Q

how can a source of blood loss be found?

A 
FOB
Upper GI endoscopy
Colonoscopy
Small bowel enema
Capsular entroscopy (if other investigations negative)
Gynae referral 
Blood loss from urinary tract?
No value for barium meal/enema studies to ascertain cause of GI blood loss-NOT ROUTINE
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26
Q

what is the management for iron deficiency?

A 
Diet
Personal care
Manage blood loss 
Oral iron
parenteral iron
blood transfusion
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27
Q

describe the use or oral iron?

A

Non enteric coated (preferable)
Replace iron deficit in total, correct anaemia and MCV, replenish iron stores
Failure: poor compliance, excessive loss, poor absorption, coeliac disease, underlying inflammation, malignancy, combined deficiency, wrong diagnosis

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28
Q

describe the use of parenteral iron?

A

Iron dextran –cosmofer
Iron sucrose – venofer
Ferric carboxymaltose – ferinject
Iron isomaltoside 1000 – monofer (PREFFERED)
Indications: oral iron intolerance, GI upset eg crohns or UC, extensive small bowel resection, severe IDA 3rd trimester pregnancy, pre EPO in CRF

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29
Q

what are the causes of macrocytic anaemia?

A

B12 deficiency, folate deficiency, liver disease, alcohol excess, hypothyroidism, drugs

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30
Q

what are the types of macrocytic anaemia?

A

megaloblastic

non megaloblastic

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31
Q

what are the causes of megaloblastic macrocytic anaemia?

A

Vitamin B12 deficiency
Folate deficiency
Drugs-methotrexate, zidovudine, metformin (decreases B12 absorption)

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32
Q

what are the causes of non-megaloblastic macrocytic anaemia?

A

Alcohol abuse
Liver disease
Myelodysplasia
hypothyroidism

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33
Q

what are the investigations for macrocytic anaemia?

A
  • Endoscopy-Gastric biopsy (b12), buodenal biopsy (folate)
  • Bone marrow-Mds, aplastic anaemia, myeloma
  • Folate deficiency-Autoantibody screen for coeliac
  • B12 deficiency-Auto antibodies (GPC, IF), diacopac, gastrin assay
  • Macrocytosis-B12, folate, LFT, TFT, reticulocyte count, EP
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34
Q

what are the sources of b12?

A

Basic structure can only be synthesized by bacteria and human body convert to various forms
Sources-everything that walks, swims or flies but nothing that grows out of the ground

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35
Q

why is b12 important?

A

Key to normal functions of brain, CNS, and blood
Involved in DNA synthesis
Sub acute combined degeneration: patchy loss of myelin in posterior and lateral columns, weakness, glove and stocking paresthesia, vision disturbances, mental disturbances, bilateral spastic paresis.

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36
Q

what are the causes of b12 deficiency?

A 
Diet
Malabsorption (gastric, intestinal)
Drugs (H2 receptor antagonists, PPI, metformin
Vegan, poor diet
PA gastrectomy
Crohns 
Stagnant loop
Ileal resection
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37
Q

what is needed for b12 absorption?

A 
intrinsic factor (parietal cells)
intrinsic factor is a glycoprotein. vitamin b12 combines with intrinsic factor to form a complex that can resist GIT enymes and is absorbed in the terminal ileum by pinocytosis. it is transported to the liver where it is stored
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38
Q

what is the treatment for B12 deficiency if there are no neurological symptoms?

A

Initially 1mg 3x a week for 2 weeks then 1mg every 3 months

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39
Q

what is the treatment for B12 deficiency if there are neurological symptoms?

A

Initially 1mg on alternate days until no further improvement then 1mg every 2 months

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40
Q

what are the side effects of B12 injections (hydroxocobalamin)?

A

Nausea, headache, dizziness, fever, hypersensitivity reactions (rash and pruritis), injection site pain, hypokalaemia during initial treatment

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41
Q

what is the role of folic acid?

A 
Water soluble (B9, Bc)
DNA synthesis, DNA repair, cofactor in biological reactions, rapid cell growth
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42
Q

describe the features of folic acid deficiency/

A 
Macrocytic anaemia
Diarrhoeas
Fatigue and tiredness
Bald, swollen tongue
Mental onfusion, forgetfulness, cognitive disability
Neural tubular defects in embryos
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43
Q

what are the causes of folate deficiency?

A 
Diet 
Malabsorption
Increased demand eg pregnancy
Increased loss
Drugs (anticonvulsants, sulphasalazine, metformin, methotrexate, triamterene, barbiturates)
Increased requirements
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44
Q

what is the treatment of folate deficiency?

A

Oral folic acid
5mg daily
Prophylaxis in pregnancy

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45
Q

describe the features of heamoglobin s?

A

Participation in molecular polymerization of deoxy-Hb
Erythrocytes of heterozygous (sickle cell trait) individuals have been shown to resist invasion by malarial parasites which improves protection against plasmodium falciparum

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46
Q

describe sickle cell crisis/

A 
More likely to have a sickle crisis during anaesthesia 
Vaso-occlusive crisis
Painful crisis
Sequestration crisis
Infections
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47
Q

what is the treatment for sickle cell crisis?

A

Hydration
Analgesia
Antibiotics
Blood transfusion

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48
Q

what are the classifications of thalassemia?

A 
Thalssemia major
Thalassemia trait
Hb-H disease
Hydrops fetalis
Silent carrier
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49
Q

what is thalassemia?

A

Hereditary anaemias due to mutations affecting synthesis of Hb
In beta thalassaemia there is a deficient synthesis of beta globin
Alpha thalassaemia-deficienct synthesis of alpha globin
Reduced synthesis of one of two globin polypeptides leads to deficient haemoglobin accumulation, resulting in hypochromic and microcytic red cells

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50
Q

what is thalassemia trait?

A

mild and clinically significant anaemia that apparently protects individuals from malaria, and therefore through natural selection it has become extremely common in some parts of the world

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51
Q

how is a laboratory diagnosis of thalassemia made?

A 
FBC- low MCV, high RBC-test for HBA2 and HBF
Ante natal screening
Partner screening for ante natal clinic
FBC on parents following positive newborn screening 
Family screening
Iron status check
Hb electrophoresis 
Hb column chromatography
Newborn blood screening at 5-8 days
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52
Q

what are the types of haematological malignancies?

A
  • Lymphoid-derived from neoplastic lymphocytes
  • Myeloid-derived from neoplastic neutrophil precursors (in bone marrow)
  • Both types present with similar symptoms, non specific
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53
Q

give examples of myeloid malignancies?

A 
Acute myeloid leukaemias 
Myelodysplastic disorders
Chronic myeloid leukaemia 
Juvenile chronic myeloid leukaemia
Polycythemia rubra vera
Essential thrombocythaemia 
chronic myelomonocytic leukaemia 
Myelofibrosis 
Myeloproliferative neoplasm unspecified 
Hypereosinophilic syndromes
Systemic mastocytosis 
Paroxysmal haemoglobinuria
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54
Q

give examples of lymphoid malignancies?

A 
Diffuse large B cell lymphoma
Follicular lymphoma
Hodgkin lymphoma
Mantle cell lymphoma
Burkitts lymphoma 
Acute lymphoblastic leukemia 
Chronic lymphocytic leukemia 
Hairy cell leukaemia 
Waldenstroms macroglobulinaemia 
Large granular lymphocytic leukaemia 
Marginal zone lymphomas
Myeloma 
Other plasma dyscrasias 
AL amyloidosis 
Heavy and light chain deposition diseases
Peripheral T cell lymphoma 
Anaplastic large cell lymphoma
Mycosis fungiodes 
Sezary syndrome
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55
Q

describe the presentation of lymphoma?

A

Incidental finding of raised WCC
Lymphadenopathy (usually painless, lump in neck or groin, slowly getting bigger)
Sweats (pathological=drenching sweats often at night to point where they have to change clothes)
Pruritis (itching)-hodgin lymphoma
Weight loss (pathological=loss of 10% or more unintentionally)
splenomegaly

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56
Q

describe the presentation of myeloid disorders?

A

Weight loss
Fatigue
Splenomegaly
Intermittent spiking fevers

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57
Q

what blood results are typically seen in bone marrow failure?

A 
Hb- anaemia
MCV raised
Neutropenia
Thrombocytopenia
Abnormal white cells –blood films
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58
Q

describe infections seen in patients with haematological maignancies?

A

Increased risk for patients with haematological malignancies
Direct infiltration of marrow- neutropenia and increase risk of bacterial and fungal infections
Atypical infection risks increased
Myeloma-cause neutropenia and failure to produce normal IG causing hypogabagloblinaemia
Multiple myeloma-herpes zoster
Pneumococcal sepsis- bone marrow failure
Varicella zoster-chronic lymphocytic leukaemia

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59
Q

what is the cause of bone pain in myeloma?

A

osteolytic lesions due to increased bone turnover, inhibition of osteoblast

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60
Q

describe the relationship between gout and haematological malignancy?

A

Increased cell turnover
Hyperuricaemia
Myelofibrosis associated

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61
Q

describe the relationship between neurological problems and haeatological malignancy?

A

Focal neurology
Spinal cord compression
Present with spinal cord compression due to malignancy
Peripheral neuropathy-myeloma, amyloidosis

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62
Q

describe the common presentation of haematological malignancies?

A 
Incidental blood count
Symptomatic anaemia
Bleeding-minor/major
Infection
Lymphadenopathy-unresolved for 4-6 weeks
Pain-bone, abdominal
Medical/surgeical emergency
Unexplained weight loss, sweating, dyspnea
Non-specific symptoms of tiredness and lethargy 
Medical co-morbidity-VTE, TIA, CVA
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63
Q

what information is it important to give to patients with haematological malignancies?

A 
Diagnostic information
Explanation of diagnosis
Confirming malignancy
Explain results of investigations
Discuss management options
Discuss prognosis 
Explain chemotherapy
Look for special needs
Written information about diagnosis and treatment 
National/local self help groups
Offer internet sites
Discuss fertility
Wig
Work
Attending for chemptherapy
Role of GP and other services
Family and financial support
Care in community
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64
Q

what tests would be of use in patient with acute leakemia?

A 
bloods
PB immunophenotyping
bone marrow
BM karotyp
X-rays
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65
Q

what tests would be of use in a patient with chronic leakemia?

A 
bloods
PB immunophenotyping
bone marrow
BM karotyp
X-rays
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66
Q

what tests would be of use in a patient with MPD?

A 
bloods
PB immunophenotyping
bone marrow
BM karotyp
X-rays
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67
Q

what tests would be of use in a patient with MDS?

A 
bloods
PB immunophenotyping
bone marrow
BM karotyp
X-rays
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68
Q

what tests would be of use in a patient with lymphoma?

A 
bloods
LN biopsy
bone marrow
X-rays
CT scan
PET
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69
Q

what tests would be of use in a patient with myeloma?

A 
bloods
bone marrow
X-rays
MRI
PET
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70
Q

describe myeloid maturation?

A 
myeloblast
promyelocyte
myelocyte
metamyelocyte
band
neutrophil
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71
Q

what are lymphomas?

A

Neoplasms of lymphoid origin, typically causing lymphadenopathy
Leukaemia vs lymphoma
Lymphomas as clonal expansions of cells at certain developmental stages

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72
Q

describe biologically rational classification for lymphoma?

A

Diseases that have distinct morphology, immunophenotype, genetic features, clinical features

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73
Q

describe clinically useful classification for lymphoma?

A

Diseases that have distinct clinical features, natural history, prognosis and treatment

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74
Q

what are the types of B cell neoplasms?

A

Precursor B cell neoplasms
Mature B cell neoplasms
B cell proliferation of uncertain malignant potential

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75
Q

give examples of T cell and NK cell neoplasms?

A

Precursor T cell neoplasms
Mature T cell and NK cell neoplasms
T cell proliferation of uncertain malignant potential

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76
Q

what are the types of hodgkin lymphoma?

A

Classical Hodgkin lymphomas

Nodular lymphocyte predominant hodgkin lymphoma

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77
Q

what are the mechanisms of lymphomagnesis?

A

Genetic alterations
Infections
Antigen stimulation
Immunosuppression

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78
Q

describe the epidemiology of lymphomas?

A

5th most frequently diagnosed cancer overall for men and women
Hodgkin lymphoma bimodal age groups rise in 20s and 70s
Males>female
Incidence
-NHL increasing over time
-Hodgkin lymphoma stable
-9750 cases of NHL diagnosed in UK each year and 4450 deaths
-1350 diagnosed with HL in UK each year and 300 deaths

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79
Q

describe outcomes ofhodgkin lymphoma?

A

variable survival if untreated
mostly curable
should be treated

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80
Q

describe outcomes of indolent non hodgkin lymphoma?

A

survivial for years in untreated
generally not curable
Defer treatment if asymptomatic

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81
Q

describe outcomes for aggresive no hodgkin lymphoma?

A

survival for months if untreated
curable in some cases
should be treated

82
Q

describe outcomes for very aggresive non hodgkin lymphoma?

A

survivial for weeks if untreated
curable in some
should treat

83
Q

what are the risk factors for non hodgkin lymphoma?

A 
Immunosuppression or immunodeficiency
Connective tissue disease
Family history of lymphoma
Infectious agents 
Ionizing radiation
84
Q

describe the clinical manifestations of non hodgkin lymphoma?

A

Variable

  • Severity – asymptomatic to extremely ill
  • Time course-evolution over weeks, months or years

Systemic manifestations
-Fever, night sweats, weight loss, anorexia, pruritis

Local manifestations

  • Lymphadenopathy, splenomegaly most common
  • Any tissue potentially infiltrated
85
Q

what are the potential complications of lymphoma?

A 
Bone marrow failure
CNS infiltration
Immune hemolysis or thrombocytopenia
Compression of structures (spinal cord, ureters) y bulky disease
Pleural/pericardial effusions, ascites
86
Q

how is a diagnosis of lymphoma made?

A

Biopsy proven before initiating treatment

Need enough tissue to assess cells

87
Q

what are the types of non hodgkin lymphoma?

A

Follicular lymphoma
Diffuse large B cell lymphoma
other NHL

88
Q

what are the stages of lymphoma?

A
  1. neck lymph
  2. neck + axilla
  3. neck+axilla+groin +abdo lymph
  4. all of above +liver +pelvis
89
Q

describe follicular lymphoma?

A 
Most common type of ‘indolent’ lymphoma
Usually widespread at presentation
Often asymptomatic 
Not curable
Associated with BCL-2 gene rearrangement [t(14;18)]
Cell or origin-germinal center B cell
Watch and wait if asymptomatic 
Chemotherapy is symptomatic 
Median survival – years
Transformation to aggressive lymphoma can occur
90
Q

what is the treatment for follicular lymphoma?

A 
Rituximab based chemotherapy 
Relapse
-2nd line chemotherapy followed by ASCT
-Maintenance rituximab for 2 years
-Zevlin (yattrium labelled anti CD20 antibody
Commonly used in uk
-RCHOP
-RCVP 
-Trial shows RCHOP is better than RCVP or RMCP
91
Q

describe diffuse large B cell lymphoma?

A 
Most common type of aggressive lymphoma 
Usually symptomatic 
Extranodal involvement is common 
Cell of origin-germinal center B cell
Treatment should be offered
Curable 40%
92
Q

describe the management of diffuse large B cell lymphoma?

A

Localised
-RCHOP X4 cycles followed by involved field RT (IFRT)
Stage IIB-IV
-RCHOP X minimum 6 maximum 8 cycles
Relapse following chemotherapy
-2nd line chemotherapy (various all approximately equally effective)
-ASCT

93
Q

describe hodgkin lymphoma?

A

Cell of origin – germinal centre B cell
Reed-Sternberg cell (or RS variants) in the affected tissues
Most cells in affected lymph node are not polyclonal reactive lymphoid cells, not neoplastic cells
Epideiology – less frequent than non Hodgkin lymphoma / M>F / peak incidence in 3rd decade

94
Q

what are the associated factors with hodgkin lymphoma?

A

EBV infection
Smaller family size
Higher socio-economic status
Caucasian>non caucasian

95
Q

what is the possible mode of pathogenesisfor hodgkin lymphoma?

A

Germinal centre B cell - > transforming event such as EBV -> RS cell leading to loss of apoptosis and cytokines leading to inflammatory response

96
Q

what are the histological subtypes of classical hodgkin lymphoma?

A

Nodular sclerosis (most common)
Mixed cellularity
Lymphocyte-rich
Lymphocyte depleted

97
Q

what are the clinical manifestations of hodgkin lymphoma?

A

Lymphadenopathy
Contagious spread
Extranodal sites relatively uncommon except in advanced disease
‘B’ symptoms

98
Q

describe treatment and survival for stage I or II hodgkin lymphoma?

A

ABVD x4 and radiation
70-80% failure free survival
80-90% overall 5 yr survival

99
Q

describe treatment and outcome for stage III, IV hodgkin lymphoma?

A

ABVD X6
60-70% failure free survival
70-80% 5 yr survival

100
Q

what are the long term complications of treatment for hodgkin lymphoma?

A

Infertility
MOPP >ABVD / males>females
Sperm banking should be discussed
Premature menopause

Secondary malignancy
Skin, AML, lung, MDS, NHL, thyroid, breast

Cardiac disease

101
Q

describe myeloproliferative disease?

A

Neoplastic disorder of hemopoietic stem cells
Over production of all cell lines, with usually one line in particular
Fibrosis is a secondary event
Acute myeloid leukemia may occur

102
Q

what are the types of myeloproliferative disease?

A

Polycythemia (rubra) vera (PRV, PV)
Essential (primary) thromocythemia
Myelofibrosis (with myeloid metaplasia), agnognic myeloid metaplasia (MF, MMM, AMM)

103
Q

describe normal platelet reguation?

A

thrombopoietin
Constitutive production of thrombopoietin by liver
Bound by platelets
Excess stimulates megakaryopoiesis

104
Q

describe issues with platelet regulation in primary/essential thrombocytosis?

A

Neoplastic stem cell disorder causing dysregulated production of large numbers of abnormal platelets
Some cases non-clonal (esp young women)
Abnormal platelets aggregate in vivo causing thrombosis
Abnormal platelets also cause bleeding

105
Q

what are the clinical features of essential thrombocytosis?

A 
None
Erythromelagia
Peripheral vascular occlusion
Transient ischaemic attack 
Stroke 
bleeding
106
Q

describe making a diagnosis of essential thrombocytosis?

A

Distinguish from reactive thrombocytosis and chronic myeloid leukemia
Secondary causes to be excluded-haemorrhage, infection, trauma, recent surgery, recent chemotherapy, drug induced
Clinical setting, blood film, bone marrow and cytogenetics help

107
Q

describe the management of essential thrombocytosis?

A

Low risk – (age <40 ,platelet count upto 600/aspirin 75mg/d

Intermediate risk (age 40-60, platelets 600-1000) hydroxycarbamide / anagrallide / aspirin 75mg/d)

High risk –(age >60, platelets>1000, other risks) hydroxycarbamide/ aspirin 75mg/d)

108
Q

what is polycythaemia rubra vera?

A

A neoplastic (clonal JAK-2 mutation) stem cell disorder, which leads to excessive production of all myeloid cell lines, but predominantly red cells. The increase in whole blood viscosity causes vascular occlusion and ischemia, compounded by increase in platelets

109
Q

what are the secondary causes of polycythemia?

A

Smoking, COPD, coal miners, high altitude, haepatoma, renal diseases, uterine fibroids

110
Q

what are the clinical features of polycythaemia rubra vera?

A 
Headaches
Aqua pruritus 
Vascular occlusion
Thrombosis
TIA, stroke
Splenomegaly
111
Q

how is a diagnosis of polycythaemia rubra vera made?

A

Exclude secondary polycythemia

Look for features of primary polycythemia

112
Q

what is the management for polycythaemia rubra vera?

A

Phlebotomy treatment of choice reduce haematocrit
Low dose aspirin 75mg/day
Hydroxycarbamide –if v/s unsuccessful or associated leukocytosis or thrombocytosis
Do not treat with iron

113
Q

what is myelofibrosis?

A

Hemopoietic stem cell disorder
Distinguish from secondary marrow fibrosis
Bone marrow failure
Myeloid metaplasia (neoplastic (clonal) extra-medullary hemopoiesis)

114
Q

what is the management for myelofibrosis?

A

Supportive care
Regular blood transfusion
Treat iron overload due to blood transfusion
Thalidomide may be useful to reduce splenomegaly
Splenectomy in selected patients
Allo BMT for younger patients

115
Q

what is the presentation of myelofibrosis?

A

Normocytic anaemia
Anaemia transfusion dependent
Abnormal blood film-lecoerythroblastic with tear drop RBCs
Splenomegaly (one of the causes of massive splenomegaly
Splenic infarts

116
Q

how do you make a diagnosis of myelofibrosis?

A 
Typical blood picture
Splenomegaly
Bone marrow aspirate-dry tap
Marrow fibrosis on trephine biopsy
Absence of other cause of splenomegaly and marrow fibrosis
117
Q

how do lymphomas usually present?

A 
Asymptomatic lump
Incidental finding
‘B’ symptoms
-Lumps
-Sweats
-Weight loss
118
Q

describe the presentation of lymphomas due to complications?

A

Infection – bacterial / atypical / viral
Immune – AIHA / ITP / vasculitis
Chemistry – hypercalcaemia / renal failure
Coagulopathy
Marrow failure
Neurology – neuropathy / cerebral / spinal deposits
Obstruction – SVC / biliary / bowel

119
Q

describe how a diagnosis of lymphoma is made?

A 
Tissue sample
Cytochemistry with makers
Cytology + immunophenotyping
Conventional cytogenetics
Fluorescent In-Situ Hybridisation (FISH)
Molecular studies
120
Q

describe the use of PET scans in diagnosing lymphomas?

A 
Injection of 18-Fluorodeoxyglucose (FDG)
Glucose analogue labelled with Fluorine 18
Metabolically active areas highlighted
Baseline assessment
Response assessment
121
Q

what is diffuse large B cell lymphoma?

A

The histologic features of all three components (large immunoblasts and centroblasts, small lymphocytes and histiocytes) of this NHL are most evident in this view. In addition scattered eosinophils can also be seen. A diagnosis of diffuse large B-cell lymphoma, T-cell/histiocyte rich variant was confirmed on flow cytometric analysis and immunohistochemistry.

122
Q

what is the revised international prognostic index (R-IPI)?

A 
used for large B cell lymphoma
Score 1 point for
Age >60 		
Stage III/IV
>1 extranodal site
Performance status >1
Raised LDH

Three groups:-
Very good PFS 94%
Good PFS 80%
Poor PFS 53%

123
Q

describe the treatment for diffuse large B cell lymphoma?

A 
Depends on clinical stage. 
Patient specific (Fertility, Side effects, Age / co-morbidities / fitness)
Clinical trials when available
Patient education and support
Chemotherapy
124
Q

describe the use of chemotherapy in diffuse large B cell lymphoma treatment?

A

Localised disease, stage I/II
-Rituximab, cyclophosphamide, vincristine, doxorubicin, prednisolone (RCHOP) x 3, followed by radiotherapy to the involved sites.

Bulky (>10cm) stage I/II disease, stage III/IV disease
-RCHOP x 6

125
Q

describe follicular lymphoma?

A 
Variable presentation and course.
Asymptomatic in many.
Treatment only for symptomatic / progressive disease.
Risk of high grade transformation.
Incurable
126
Q

what is used to assess prognosis of follicular lymphoma?

A

FLIPI to assess prognosis:

Based on age <>60, number of nodal sites <>3, LDH normal/elevated, haemoglobin <>120g/L, Stage I/II vs III/IV

127
Q

describe treatment of follicular lymphoma?

A

Watch and wait.
Radiotherapy for localised stage I/II disease.
Rituximab-based chemotherapy for symptomatic generalised disease.
Rituximab monotherapy
Rituximab chlorambucil.
Rituximab cyclophosphamide vincristine prednisolone.
Rituximab fludarabine cyclophosphamide.
BCR inhibitors idelalisib or ibrutinib.
Stem cell transplantation for relapsed disease.

128
Q

describe staging designations for lymphoma?

A

Ann Arbor staging I/II/III/IV, extranodal (E)
A - no symptoms
B – Temp > 38oC, night sweats, 10% wt loss over <6 months
Pruritis not B symptom
International prognostic score for advanced stage

129
Q

describe chemotherapy used to treat hodgkin lymphoma?

A

Anthracycline based adriamycin, bleomycin, vinblastine and dacarbazine (ABVD) – Gold standard.
Alkylator based chlorambucil, vinblastine, procarbazine, prednisolone (ChlVPP).
Dose intensified alkylator Bleomycin, adriamycin, cyclophosphamide, vincristine, procarbazine, prednislone BEACOPP

130
Q

describe the role of radiotherapy post chemotherapy in treating hodgkin lymphoma?

A

No evidence of benefit if in CR.
Patients with PR post chemotherapy receiving DXT to residual mass similar outcome to those in CR.
Late toxicity, especially breast Ca.
Mantle DXT < 15 years, 136x increase in risk of breast Ca.
Baseline risk if irradiated > 30 years.

131
Q

describe the presentation of myeloma?

A 
Pain from osteolytic bone lesions
Renal issues
Anaemia
Bone problems
Infections – immunepariesis
132
Q

what are the laboratory features of myeloma?

A 
Routine biochemistry (Raised calcium, Raised urea &amp; creatinine, Raised total protein)
Beta 2 microglobulin - prognostic
ESR / PV is usually markedly elevated
Bone marrow increased plasma cells
M band in serum
M band in urine (BJP)
Serum free light chains
Blood film (Anaemia, Rouleaux, Background staining)
133
Q

describe the course and prognosis of myeloma?

A 
Death
-aggressive tumour growth
-bone marrow failure
-Infection
-skeletal complications
-renal failure.
Median survival was 3 years
Modern therapy >6 years or more
134
Q

what are the predisposing factors for acute myeloblastic leukaemia?

A

Radiation, benzene, previous chemo clonal haematological states
Inherited bone marrow failure conditions, downs

135
Q

what are the clinical features of acute myeloblastic leukaemia?

A

Bone marrow failure
Extramedullary involvement, usually monocytic subtypes.
Granulocytic sarcomas.
Hyperleukostasis, WCC > 100 x 109/l

136
Q

describe the WHO classification of acute myeloblastic leukaemia?

A

AML with recurring cytogenetic abnormalities (15:17, inv16, 8:21, 11q MLL rearrangements).
Therapy related AML
AML with multilineage dysplasia
AML not otherwise classified

137
Q

what subgroups have a good prognosis from acute myeloblastic leukaemia?

A

Defined by cytogenetics
8:21.
15:17, PML - RAR, responds to ATRA, associated with hyperfibrinolysis.
Inv 16

138
Q

describe acute myelocytic leukaemia?

A

Associated with 15:17 PML:RARa.
Good prognosis, 70% 5 year overall survival.
Coagulopathy can be fatal.
Sensitive to ATRA and arsenic trioxide.

139
Q

describe the treatment of non-APL AML?

A

Remission induction using anthracycline / nucleoside + cytosine.
2-3 subsequent courses of consolidation chemotherapy.
Allo SCT for high risk patients (adverse cytogenetics, relapsed patients).
Good risk genetics– 65-70% 5year survival.
Standard risk genetics– 55 % 5 year survival
Poor risk genetics– 20-30% 5 year survival.
Elderly >65years 10% 5 year survival

140
Q

describe the treatment of acute lymphoblastic leukaemia?

A

Combination chemotherapy with intrathecal methotrexate as induction.
Consolidation therapy using high dose methotrexate for CNS prophylaxis.
Allo SCT for high risk cases (poor risk genetics, relapse).
Maintenance chemotherapy for up to 2 years, not required for B cell ALL.

141
Q

what is the prognosis for acute lymphoblastic leaukaemia?

A

50-60% response rate in adults
20-40% 5 year survival.
Better results in children

142
Q

describe the features of chronic lymphocytic leukaemia?

A 
Indolent B lymphoproliferative disorder
Commonest adult leukaemia in West.
Males>females.
Increases with age (Median age 65)
Lymphocytosis, clonal mature B cells
Lymphadenopathy
Hepatosplenomegaly
143
Q

how is a diagnosis of chronic lymphocytic leukaemia made?

A

Peripheral blood B cell lymphocytosis, 5×109/L (NCI)
Bone marrow infiltration (>30%)
Characteristic immunophenotype: sIg (weak), CD5+, CD23+, FMC7­, CD79b+ (weak), CD19+/CD5+

144
Q

describe the treatment of chronic lymphocytic leukaemia?

A 
Only for symptoms or progressive disease
No benefit for early treatment 
Chlorambucil
Bendamustine +/- rituximab
Fludarabine, Cyclophosphamide, Rituximab
Campath (anti CD52)
Novel targeted therapies.
Solitary plasmacytomas radiotherapy
MGUS and asymptomatic Myeloma do not require therapy
Patients less than 70 years
Thalidomide/lenalidomide/bortezomib containing combination therapy 
autologous stem cell transplant with high dose Melphalan
Older patients attenuated regimens
Bisphosphonate for myeloma-bone disease.
145
Q

what are the indications of therapy for chronic lymphocytic leukaemia?

A 
Constitutional symptoms referable to CLL
Progressive marrow failure
Anaemia and/or thrombocytopenia 
Massive or progressive splenomegaly
Massive or progressive lymphadenopathy
Progressive lymphocytosis
146
Q

describe the features of CML?

A

Rare
Diagnosed incidentally or following development of systemic symptoms, splenomegaly or gout.
Characterised by Philladelphia chromosome (t9:22), producing constitutive activation of Abl kinase.
Risk of transformation to acute leukaemia.

147
Q

what is the treatment for CML?

A 
Tyrosine kinase inhibitors:-
Imatinib
Nilotinib
Dasatanib
Posnatinib
Bosutinib
Allogeneic transplantation for refractory/resistant/transformed cases.
148
Q

what is myeloma?

A 
Malignant plasma cells (terminally differentiated b cells that produce IgG,  iGA (IgD)- identifiable ammonut of monoclonal immunoglobulin 9m band)
Monoclonal immunoglobulin (m band)
149
Q

how is myeloma usually diagnosed?

A 
Incidentally or symptomatically 
Patients with renal failure should be screened for myeloma
Average age presentation in 70s
At least one of 
-Renal dysfunction, hypercalcaemia, bone damage, anaemia
->1 bone lesion on whole body MRI/CT/PET
-Free light chain ratio >100
->60% plasma cells in bone marrow
150
Q

describe the presentation of myeloma?

A

Pain from osteolytic bone lesions
Renal issues
Anaemia
Bone problems
Amyloidsis –displation of amyloid protein
Infections –immuneparesis eg due to neutropenia

151
Q

what are the laboratory features of myeloma?

A

Blood film–anaeia or rouleaux (sticky chain of red cells on blood film this isn’t specific for myeloma also in acute phase response or waldenstrome macroglobinemia)
Raised calcium, urea, creatinine, protein
Beta 2 macroglobulin-prognostic
ESR/PV elevated
Bone marrow increased plasma cells
M (monoclonal) band in urine or serum
Serum free light chaing
Electrophoresis-identify type and level of M protein, may show marked immune paresis
60% IgG, 20% IgA, 20% light chain only, rarely IgD, E,M or non secretory

152
Q

describe the course and prognosis of myeloma?

A

Determined by international staging system and cytogenetics
Stage1 beta 2 macroglobulin <3.5mg/l, albumin >35g/l – 62 months
Stage 2 beta 2 macroglobulin 3.5mg/l and/or albumin <35g/l – 44 months
Stage 3 beta 3 macroglobulin >5.5mg/l -29 months

153
Q

what are the other investigation findings found in a patient with myeloma?

A

Bone marrow aspirate – if more than 10% WC are plasma cells this is dianostic of myeloma
Enlarged plasma cell with enlarged golgi
Poor prognosis – immunoglobulin G rearrangements on chromosome 14 (2-3 yr survival), 17p chromosome (p53 protein)
Pathological fractures / spinal cord compression (steroid and radiotherapy)
Punched out lesions in skull (pepperpot skull)

154
Q

what is the therapy given for treatment of myeloma?

A

Asymptomatic – observe 10% risk of progression to symptomatic per year
Solitary plasmacytoas radiotheraoy
MGUS and asymptomatic myeloma don’t require therapy
Patients less tha 70
Thalidomide/lenalidomide / bortezomib containing combination therapy
Autologous stem cell transplant with high dose melphalan
Older patients attenuated regimens
Bisphosphonate for myeloma bone disease

155
Q

what is sickle cell anaemia?

A

Haemaglobinopathy
Heamaglobin –iron haem complex, 2 beta and 2 alpha chaing
Occur when there is mutation to alpha or beta change

156
Q

what does thalassemia mean?

A

reduction in haemaglobin

157
Q

what does haemaglobinopathy mean?

A

structural change in Hb

158
Q

describe the structure of habmoglobin in sickle cell anaemia?

A

Mutation in beta globin change
Glutamine for valine at position 6
Production of haemoglobin S (2 alpha and 2 abnormal beta)
Genetics-Autosomal recessive
Hb S has a tendency to polymerise when deoxygenated and produces long insoluble chains, abnormal shape, cant pass through capillary vessels

159
Q

how is a diagnosis of sickle cell anaemia made?

A

family history, anaemic, blood film analysis (some sickle shape, some normal), detect abnormal Hb using electrophoresis, HBAC, mass spec

160
Q

what are the clincial features of sickle cell anaemia in children?

A

Children – delayed growth, dactylitis, splenic infarct, aplastic crisis, cardiac, respiratory, renal problem

161
Q

what are the clinical features of sickle cell anaemia in adults?

A

Adults-acute resp failure, cardiac failure, liver failure, renal problem, vascular necrosis of hip, chronic ulcer, increase stroke risk

162
Q

where is there a high rate of inheritance of sickle cell anaemia?

A

Inheritence associated with areas where malaria is prevalent due to protection. Middle east, sub sahara
Confers survival advantage

163
Q

describe the risks associated with sickle cell anaemia?

A

Sepsis leading cause of mortality particulary in developing countries
Underlying immune dysfunction related to hyposplenism
Mainly encapsulated bacteria
Highest risk under age of 5
Pneumococcal vaccination and prophylactic penicillin needed

164
Q

what are the clinical issues with sickle cell anaemia and how are they resolved?

A

Painful vaso-occlusive crisis – bone bain, in children-phalanges. Management with rest and rehydration, analgesia
Acute chest syndrome - increasing pain in ribs with associated increasing SOB, hypoxaemic. Manage pain, red cell exchange to suppress Hb S <30%. Significant mortality in developed countries
Leg ulcers-vaso-occlusion over medial and lateral malleolus, supportive management, persist for months or years
Stroke – thrombtic stroke, stenosis of internal carotid artery due to intimal hyperplasia. Hypertransfusion (regular), screen children for carotid stenosis
Anaemic crisis – aplastic criss, parovirus, splenic sequestration
Hydroxycarbomide (chemotherapy) for patients with recurrent problems, OD

165
Q

what are the causes of microcytic anaemia?

A 
SIT
Sideroblastic 
Iron deficiency 
Thalassemia 
Anaemia of chronic disease
166
Q

what are the causes of normocytic anaemia?

A

Acute blood loss
Haemolytic anaemia
Sickle cell
Anaemia of chronic disease

167
Q

what are the causes of megaloblastic anaemia?

A

Megaloblastic:
-b12/folate deficiency

Non megaloblastic

  • alcohol
  • reticulocytosis
  • liver disease
  • pregnancy

Hypothyroidism
Bone marrow failure (aplastic anaemia, myelodysplasia, leukaemia, myelofibrosis)

168
Q

what does high neutrophil count suggest?

A 
bacterial infection
inflammation
necrosis
corticosteroids
malignancy
stress
169
Q

what does low neutrophil level suggest?

A 
post chemotherapy
agranulocytosis causing drugs (4C's-carbamazepine, clozapine, colchicine, carbimazole)
viral infection
hypersplenism
bone marrow failure
felty's syndrome
170
Q

what does a high lymphocyte count suggest?

A

viral infection
chronic infection
CLL/lymphoma

171
Q

what does a low lymphocyte count suggest?

A 
viral infection
HIV
post chemo
bone marrow failure
whole body radiation
172
Q

what does a high monocyte count suggest?

A

bacterial infection
autoimmune disease
leukaemias/hodgkins

173
Q

what does a low monocyte count suggest?

A

acute infections
corticosteroids
leukaemias

174
Q

what does a high eosinophil count suggest?

A 
allergy
parasite infection
drug raactions
hypereosinophilic syndrome
skin diseases
malignancy (hodgkins)
175
Q

what does a high basophil count suggest?

A 
some leukaemias
IgE mediated hypersensitivity
inflammatory disorders
myeloproliferative disorders 
viral infection
176
Q

what factors can cause physiological variations in the total WBC count?

A 
Age
Sex
Individual variation
Diurnal variation
Exercise
Smoking
Emotion
Pregnancy
Racial variation
Drugs
Elderly people receiving the flu vaccination
177
Q

describe the causes of neutropenia?

A 
Impaired production
Excessive consumption
Ethnic
Stable vs progressive 
Main risk is infections
Risk of sepsis increases with neuts < 1.0 x109/L. Neuts > 1 x109/L no increase in infections.
178
Q

what should you considerin a patient with lymphopenia?

A 
HIV
Steroids/immunosuppression
Exotic infections
SLE/autoimmunity
Malignancy/Hodgkins/Aplastic anaemia
Rare immunodeficiency states
179
Q

what are the primary causes of lymphocytosis?

A

Monoclonal B lymphocytosis / LPD

180
Q

what are the reactive causes of lymphocytosis?

A 
Chronic inflammation
Viral infections
Autoimmunity
Smoking
Hyposplenism
Physiological stress – sepsis, cardiac, surgery
Drug induced
181
Q

what is bone marrow aspiration?

A

Examination of bone marrow to further investigate abnormalities in peripheral blood
Aspiration – provides film which can be examined for morphology of development of haemopoietic cells

182
Q

what is trephine?

A

obtaining bone marrow
Trephine provides core of bone which is processed as histological specimen and allows view of bone marrow architecture, cellularity and presence/absence of abnormal infiltrates

183
Q

descrbe the process of bone marrow aspiration?

A 
iliac crest
local anaesthetic
bone marrow needle
aspirate marrow
make smear with glass slide
stain with romanowsky technique or perls' reaction for iron
184
Q

what are the indications for trephine to obtain bone marrow?

A

dry tap obtained with aspiration
better assessment of cellularity eg aplastic anaemia
better assessment of presence of infiltration or fibrosis

185
Q

what is the technique for trephine?

A

posterior iliac crest
local anaesthetic
special needle (longer and wider than aspiration)
obtain core of bone
fix informalin-decalcify
stain with haematoxylin and eosin or reticulin stain

186
Q

how is a diagnosis of anaemia made?

A 
Blood film
Ferritin
Iron Studies
B12 -may be low in 20% of normal pregnancies – and will return to normal rapidly post delivery
Folate
Reticulocyte count
Direct antigen test
Haemoglobinopathy screen
187
Q

what blood result will be seen if a patient is responding appropriately to their anaemai?

A

high reticulocyte count

188
Q

describe a patient driven diagnosis of anaemia/

A

Red cell production
Iron, b12, folate
Control – anaemia of chronic disease / renal problems
Structural – sickle, thalassaemia, enzymes
Bone marrow – haematological malignancies
Infiltration
toxicity
Red cell loss
Red cell loss
Bleeding
Haemolysis

189
Q

what is the use of measuring ferritin level in diagnosing anaemia?

A

High MW protein ~ 20% iron
Low level → iron deficiency
Acute phase protein, normal level does NOT rule out IDA
Check alongside a CRP

190
Q

in iron deficiency anemia what will the following levels be like

  1. ferritin
  2. iron
  3. transferrin
  4. transferrin saturation
A
  1. low
  2. low
  3. high
  4. low
191
Q

in ihaemochromatosis what will the following levels be like

  1. ferritin
  2. iron
  3. transferrin
  4. transferrin saturation
A
  1. high
  2. high
  3. low
  4. high
192
Q

in anaemia of chronic disease what will the following levels be like

  1. ferritin
  2. iron
  3. transferrin
  4. transferrin saturation
A
  1. normal/high
  2. low
  3. low
  4. low
193
Q

in haemolytic anemia what will the following levels be like

  1. ferritin
  2. iron
  3. transferrin
  4. transferrin saturation
A
  1. high
  2. high
  3. normal/low
  4. low
194
Q

describe the features of anaemia of chronic disease?

A

20% of anaemias over 65 years of age.
Hb 7-10g/L. MCV normal or low.
Rest of count normal.
Non-progressive anaemia.
Often associated with chronic disease.
B12/Folate/Ferritin normal.
Iron/transferrin/transferrin saturation low.
Diagnosis of exclusion.
Main differential MDS.
Defect of iron incorporation into erythron.
No role for oral iron.
Erythropoietin +/- IV iron effective at elevating Hb, but unlicensed, risk of thrombosis and cost £500-£1000.
Refer if progressive anaemia or other abnormalities in blood count or other evidence of haematological disease

195
Q

describe the features of megaloblastic anaemia?

A 
B12 and folate deficiency.
Macrocytic anaemia, thrombocytopenia.
Characteristic morphological features.
Raised bilirubin and lactate dehydrogenase (LDH) due to ineffective erythropoiesis.
Lab assays for B12 and folate imperfect
196
Q

what is pernicious anaemia?

A

autoimmune disorder in which there is atrophic gastritis with loss of parietal cells in gastric mucosa with failure of intrinsic factor production and b12 malabsorption

197
Q

what is pernicious anaemia associated with?

A

Common in elderly
Blood group A
More common in women
Associated with other autoimmune diseases
Parietal cell antibodies in 90% of ptients
Insidious onset, progressively increasing symptoms
Neurological changes can become irreversible if untreated – poynueropathy progressively involveing peripheral neres and posterior and lateral columns of the spinal cord

198
Q

what are the investigations for pernicious anaemia?

A

Haematological findings – features of megaloblastic anaemia
Bone marrow – show megaloblastic erythropoiesis
Serum bilirubin –may be raised due to ineffective EPO
LDH
Serum methylmalonic acid and homocysteine –raised in b12 deficiency
Serum vitamin B12 – usually below 160ng/l
Serum folate level – normal or high and red cell folate is normal or reduced

199
Q

describe the investigation findings in megaloblastic anaemia

A 
Hb - low
MCV - raised
erythrocyte - low
reticulocyte - low
leukocyte - low/normal
platelet - low/normal
serum ferritin - raised
plasma lactate dehydrogenalse- high
200
Q

what is seen on blood film in megaloblastic anaemia?

A

oval macrocytosis
poikilocytosis
red cell fragmentation
neutrophil hypersegmentation

201
Q

what factors can impact the likelihood of deveoping anaemia?

A

IDA causes – think about patient age, wealth and geography. Ethnic effect – diet may differ and chapatti flour has poor iron bioavailability
IDA number one worldwide is hookworm – parasitic infection. SLIDE
UK elderly - malignancies
Young women – high risk of IDA - menorrhagia
Western diet contains 15mg iron / day – 10 % can be absorbed – approx 1.5 mg day
Daily baseline loss 1mg
Menstrual loss 20 mg / month
Pregnancy takes 1g iron mother to fetus usage not spread evenly across the pregnancy
Iron replacement
Vit C promotes iron absorption so can co-administer with iron replacement, tea and coffee impair (polyphenols)

Metabolic and Endocrine (12 decks)

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