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Phase 2a Flashcards > Haematology > Flashcards

Haematology Flashcards

1
Q

What are lymphomas? Accumulate where causing what? Histologically divided into what 2 types?

A

Malignant proliferations of lymphocytes
Lymph nodes–> lymphadenopathy, also in peripheral blood or infiltrate organs
Hodgkins- have characteristic cells with mirror-like image nuclei called Reed- Sternberg cells
Non-Hodgkins- do not have characteristic cells, low grade= follicular, high grade= diffuse large B cell, very high grade= Burkitt’s

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2
Q

Aetiology of lymphomas?

A

Primary immunodeficiency- ataxia telangiectasia, Wiscott- Aldrich syndrome
Secondary immunodeficiency- HIV, transplant recipients
Infection- EBV, human T-lymphotropic virus, helicobacter pylori
Autoimmune disorders e.g. SLE

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3
Q

Signs and symptoms of lymphomas? Investigations into lymphomas?

A

Stroke, ulcers, nodal/ extra nodal disease, compression syndrome (lump compressing structures,) systemic B symptoms- lump, loss of appetite, weight loss, sweat
FBC, DM, lymph node biopsy, immunophenotyping(antigens,) cytogenetics (karyotyping- chromosomal abnormalities, FISH- translocations,) molecular genetics (PCR- insertions/ deletions,) history and examination, CXR, ECHO, PFT, WHO performance status

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4
Q

Epidemiology and suggested role in pathogenesis? Further divided into what 2 types?

A

Male predominance, EBV suggested role
Peaks of incidence= teenagers and elderly
Classical (cHL)- hallmark= Reed-Sternberg cell with mirror- image nuclei, 90-95%
Nodular lymphocyte predominant HL (NLPHL)= Reed-Sternberg VARIANT, ‘popcorn cell’

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5
Q

Risk factors for HL? Clinical presentation?

A

Affected sibling, EBV, SLE, obese, post-transplantation
Painless groin lymphadenopathy, young women= cough due to mediastinal lymphadenopathy
B symptoms= weight loss, fever, night sweats
Emergency= infection, SVC obstruction with increased JVP, sensation of fullness in head, dyspnoea, blackouts, facial oedema

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6
Q

Diagnosis of HL?

A

CT/MRI of chest, abdomen and pelvis for staging (Ann Arbor), lymph node excision/ bone marrow biopsy- popcorn cells
Bloods: high ESR or low Hb- worse prognosis
Immunophenotyping, cytogenetics, PET scan

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7
Q

Staging of HL (Ann Arbor)? Each stage what or what?

A

I= confined to single lymph node region, II= two or more nodal areas on same side of diaphragm, III= nodes on both sides of diaphragm, IV= spread beyond lymph nodes e.g. liver/ bone marrow
A or B- A= no systemic symptoms other than pruritus (severe itching of skin,)
B= B symptoms- fever, weight loss and night sweats

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8
Q

Treatment of HL? Complications of radio and chemotherapy?

A

Combination chemotherapy- ABVD: A- adriamycin, B- bleomycin, V- vinblastine, D- dacarbazine
I-A to II-A= short course ABVD followed by RT
II-A to IV-B= longer course ABVD
Radio= increases risk of second malignancies- solid tumours esp in lung, breast, melanoma, stomach, sarcoma and thyroid, increased risk of IHD, hypothyroidism and lung fibrosis
Chemo= myelosuppression, nausea, alopecia and infection, infertility

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9
Q

Epidemiology and risk factor for N-HL?

A

All lymphomas without Reed-Sternberg cells, around 80%= B-cell origin, diffuse large B-cell= commonest, 20%= T-cell
More varied in terms of presentation, sub-types, treatments and outcomes, not all centre on nodes, strong link with EBV and Burkitts lymphoma
Family history= minor increase in risk

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10
Q

Presentation of N-HL?

A

Nodal disease (75%) e.g. superficial lymphadenopathy
Extranodal (25%): skin- esp T-cell lymphoma, oropharynx, gut, small bowel, bone, CNS and lungs
Systemic B symptoms
Pancocytopenia- anaemia, infection and bleeding

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11
Q

N-HL classified into what 2 grades? Presents and curable?

A

Low/indolent grade- e.g. follicular, slow growing, usually advanced at presentation, incurable, median survival= 9-11 years
High grade- e.g. diffuse large B-cell, usually nodal presentation, 1/3 cases have extra nodal involvement

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12
Q

Diagnosis of N-HL?

A

Raised lactose dehydrogenase= worse prognosis, lymph node excision/ bone marrow biopsy- not see R-S cells/ popcorn cells
Marrow and node biopsy for classification, CT/MRI of chest, abdomen and pelvis for staging, immunophenotyping, cytogenetics

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13
Q

Treatment of N-HL?

A 
R-CHOP regimen: 
R- rituximab (MAb- minimal side effects) 
C- cyclophosphamide 
H-hydroxy-daunorubicin 
O-vincristine (oncovin brand) 
P-prednisolone
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14
Q

Rituximab targets what? Treatment for grades of N-HL? What is Burkitts lymphoma?

A

MAb targeting CD20 expressed on B cell surface
Low grade= none may be needed, radio may be curative in localised disease
High grade= early- 3 months R-CHOP with radiotherapy, late= 6 months R-CHOP regimen with radiotherapy
Usually B cells with jaw lymphadenopathy in children

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15
Q

Epidemiology of myeloma?

A

Cancer of differentiated B lymphocytes known as plasma cells, accumulation of malignant plasma cells in bone marrow–> progressive bone marrow failure
Produce excess of one type of Ig= monoclonal paraprotein
IgG(55%), IgA(20%), rarely IgM and IgD
Others= low–> immunoparesis, bone disease, hypercalcaemia, renal failure
Peak age= 70 years, more common in Afro-Caribeeans than caucasians

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16
Q

Clinical presentation of myeloma? 2 things activate osteoclasts? Inhibiting osteoblasts?

A

OLD CRAB
Old age, calcium elevated, renal failure- nephrotic syndrome, Igs deposit in organs–> thirst from kidneys, anaemia- neutropenia or thrombocytopenia, bone lytic lesions- back pain= osteoclasts activated–> increased breakdown and lytic lesions
RANK ligand and IL-3
HGF and Dkk-1
Recurrent bacterial infections due to neutropenia, healthy= may cause high neutrophil count

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17
Q

Diagnosis of myeloma? Diagnosis requires?

A

Blood: normocytic normochromic anaemia, raised ESR, Rouleaux formation on blood film
U&Es= high calcium, high alkaline phosphatase, Bence-jones protein in urine
PLAIN X-ray- lytic ‘punched out lesions: pepper-pot skull, vertebral collapse
Serum and urine electrophoresis- B2- macro globulin present and prognostic, fractures and osteoporosis
Monoclonal protein band in serum or urine, increased plasma cells on bone marrow biopsy, hypercalcaemia/ renal failure/ anaemia, bone lesions on skeletal survey

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18
Q

Treatment of myeloma?

A

Bone pain= analgesia, avoid NSAIDs due to renal risk
Bisphosphonates e.g. zolendronate- reduce fracture rates and bone pain
Anaemia= RBC transfusion and erythropoietin
Rehydration of 3L/day
Renal dialysis- treat acute renal failure
Broad-spectrum antibiotics to treat infections quickly
Chemo= CTD- cyclophosphamide, thalidomide and dexamethasone, max 8 cycles for less fit people
VAD- in fitter people, max 6 cycles
Stem cell transplant

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19
Q

4 main subtypes of leukaemia? What is leukaemia?

A

Acute lymphoblastic, acute myeloid (AML,) chronic myeloid (CML,) and chronic lymphocytic (CLL)
Presence of rapidly proliferating immature blast blood cells (can be pre-cursors of RBCs, platelets or white cells) in bone marrow that are non functional i.e. defective

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20
Q

2 issues posed by leukaemia? Age of leukaemia in general?

A

Dividing rapidly but serve no function, so wasting energy, less available for making useful functional cells
Due to rapid replication, these cells take up lot of space within bone marrow, meaning little space and also food for other cells to grow
When no space in BM, leukaemia cells will be present in blood too
At any age, type varies with age, all mainly seen in childhood and CLL= elderly disease

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21
Q

Epidemiology of acute myeloid leukaemia (AML)?

A

Neoplastic proliferation of blast cells derived from marrow myeloid–> basophils, neutrophils and eosinophils
Progresses rapidly with death in 2 months if untreated, commonest acute leukaemia of adults
Associated with radiation and syndromes such as Down’s

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22
Q

Clinical presentation of acute myeloid leukaemia?

A

Marrow failure: anaemia- low Hb: breathlessness, fatigue, angina and claudication, pallor, cardiac flow murmur
Infection- low WCC–> infections, fever, mouth ulcers
Bleeding- low platelets–> bleeding and bruising
Hepatomegaly and splenomegaly due to infiltration, gum hypertrophy, DIC occurs in subtype of AML where release of thromboplastin

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23
Q

Diagnosis and complications of acute myeloid leukaemia?

A

WCC often raised, but can be normal/ low, may be few blast cells in peripheral blood so diagnosis depends on bone marrow biopsy, differentiation from all based on: immunophenotyping and molecular methods
Infection- alert to septicaemia, causes common organisms to present oddly, with few antibodies being made

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24
Q

Treatment for AML?

A

Blood and platelet transfusions, neutropenia= prophylactic antivirals, antibacterial and antifungals, allopurinol- prevents tumour lysis syndrome, IV fluids- Hickman line, chemotherapy, marrow transplantation

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25
Q

Epidemiology of chronic myeloid leukaemia (CML)?

A

Most exclusively disease of adults, uncontrolled clonal proliferation of myeloid cells, occurs most often between 40-60 years, slight male predominance, rare in childhood, more than 80%= Philadelphia chromosome forms fusion gene BCR/ ABL on chromosome 22= tyrosine kinase activity stimulating cell division

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26
Q

Clinical presentation of CML?

A

Symptomatic anaemia e.g. SOB, abdominal discomfort due to splenomegaly, weight loss, tiredness, pallor, fever and sweats in absence of infection, features of gout due to purine breakdown, bleeding due to platelet dysfunction

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27
Q

Diagnosis of CML? Treatment of CML?

A

Blood count: very high WCC- whole spectrum of myeloid cells i.e. increased; neutrophils, myelocytes, basophils and eosinophils, low Hb- normochromic and normocytic, low platelets or normal or raised, bone marrow aspirate: hyper cellular (increased cells)
Oral imatinib- specific BCR/ABL tyrosine kinase inhibitor, stem cell transplant

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28
Q

Epidemiology of chronic lymphocytic leukaemia (CLL)?

A

Most common leukaemia, occurs predominantly in later life, accumulation of mature B cells escaped programmed cell death and undergone cell-cycle arrest
Mutations, trisomies and deletions influence risk, pneumonia may be triggering event

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29
Q

Clinical presentation of CLL?

A

Often no symptoms, presenting as surprised on routine FBC, may be anaemic or infection prone, severe= weight loss, sweats and anorexia, hepatosplenomegaly, enlarged rubbery, non-tender nodes

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30
Q

Diagnosis and complications of CLL?

A

Normal or low Hb, raised WCC with very high lymphocytes, blood film: smudge cells may be seen in vitro
Autoimmune haemolysis- increased infection risk due to low IgG; bacterial and viral especially herpes zoster, marrow failure

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31
Q

Progression and prognosis of CLL? Tx?

A

Many stable for years and may even regress, death= often due to complication of infection, may transform–> aggressive lymphoma= Richter’s syndrome
1/3 will never progress, 1/3 progress slowly, 1/3 progress actively
Blood transfusions, human IV IGs, chemotherapy or radiotherapy, stem cell transplant

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32
Q

What is anaemia?

A

Decrease in Hb in the blood below reference level for age and sex of individual
Either due to low red cell mass (RCM) or increased plasma volume
May be due to reduced production from BM or increased loss of RBCs by spleen, liver, BM and blood loss and has many causes

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33
Q

What test is to whether bone marrow production is the issue? Reduction in plasma volume will lead to what? Various anaemia types classified by what? 3 major types?

A

Reticulocyte count- low= production issue, high= removal issue
Falsely high Hb- seen in dehydration
Mean corpuscular volume (MCV)- average volume of RBCs
Hypochromic microcytic- low MCV
Normochromic normocytic- normal MCV
Macrocytic- high MCV

34
Q

Consequences of anaemia? Pathological consequences?

A

Reduced O2 transport, tissue hypoxia, compensatory changes: increased tissue perfusion, increased O2 transfer to tissues, increased RBC production
Myocardial fatty change, fatty change in liver, aggravates angina and claudication, skin and nail atrophic changes, CNS cell death (cortex and basal ganglia)

35
Q

Non-specific symptoms of anaemia? Signs?

A

Fatigue, headaches, faintness, dyspnoea and breathlessness, angina- if pre-existing coronary disease, anorexia, intermittent claudication, palpitations

May be absent even in severe anaemia, pallor, tachycardia, systolic flow murmur, cardiac failure

36
Q

3 main causes of microcytic anaemia? Rarely from what? MCV value?

A

Iron deficiency anaemia- most common, anaemia of chronic disease, thalassaemia
Congenital sideroblastic anaemia, lead poisoning
<80

37
Q

Iron deficiency from what causes? Average daily intake? % absorbed in duodenum? How is it absorbed?

A

Diet, menorrhagia, hookworm
TB, RA, HF
15-20mg, AT into intestinal epithelial cells by HCP1, some into ferritin, not ferritin= to plasma protein transferrin, to BM into new erythrocytes, majority= Hb
Rest= in reticuloendothelial cells, hepatocytes and skeletal muscles cells either as ferritin/ haemosiderin

38
Q

Signs of iron deficiency anaemia? Diagnosis?

A

Brittle hair and nails, atrophic glossitis- tongue inflammation, kolionychia- spoon shaped nails, angular stomatitis- inflammation of corners of mouth
Variation in RBC shape and size
Low serum ferrite
Low serum iron, transferrin saturation< 19%, total iron-binding capacity (TIBC) rises compared to normal
Reticulocyte count low
Number of transferrin receptors increases
Further GI investigations

39
Q

Tx of iron deficiency anaemia? Side effects?

A

Find and treat cause, oral iron e.g. ferrous sulfate= nausea, abdominal discomfort, diarrhoea/ constipation and black stools
Ferrous gluconate if SEs bad
Parenteral iron e.g. IV iron/ deep intramuscular iron in extreme cases e.g. severe malabsorption

40
Q

Anaemia of chronic disease from what disease? Can cause what?

A

CKD, rheumatoid arthritis, lupus, cancer

Shortening of red blood cell life/ reducing red blood cell production

41
Q

Main causes of normocytic anaemia? MCV value?

A

Acute blood loss, anaemia of chronic disease, endocrine disorders such as hypopituitarism, hypothyroidism and hypoadrenalism, renal failure, pregnancy
80-100 = MCV

42
Q

Main causes of macrocytic anaemia? MCV value? What is megaloblastic anaemia? Non-megaloblastic anaemia?

A

B12 deficiency(pernicious,) folate deficiency, alcohol excess
MCV> 100
Inhibition of DNA synthesis i.e. B12 and folate deficiency anaemias
Where erythroblasts are normal i.e. normoblastic

43
Q

Causes of folate deficiency, absorbed where? Found in what foods? Diagnosis and Tx?

A

Poor folate diet, malabsorption, pregnancy, anti-folate drugs (methotrexate) , jejunum
Green veg, nuts, yeast and liver
Blood film= macrocytic, erythrocyte folate level
Tx= folic acid supplementation and treat underlying cause, consider supplementation during pregnancy

44
Q

Vitamin B12 absorbed where bound to what? Found in what foods? Causes of deficiency? Epidemiology? Specific sign?

A

Terminal ileum bound to intrinsic factor
Meat, fish, dairy products, but not plants
Atrophic gastritis, gastrectomy, Crohn’s, coeliac, pernicious anaemia= parietal cells of stomach attacked so intrinsic factor not produced
Common in elderly, fair-haired, blue-eyed and blood group A, more females than males
Neurological problems

45
Q

What is specific for pernicious diagnosis? Investigations for pernicious anaemia, Tx, complications?

A

Intrinsic factor antibodies
Blood film- macrocytic
Autoantibody screen- check for IF antibodies
Vitamin B12 tablets/ injections, not folic acid, dietary cause= oral B12, IM hydroxocobalamin
Heart failure, angina, neuropathy

46
Q

When does haemolytic anaemia occur? Main causes? Signs and symptoms? Investigations? Tx?

A

When RBCs are destroyed before 120 days
Membranopathies, enzymmopathies, haemoglobinopathies, autoimmune, infections, secondary to systemic disease
Gallstones, signs: jaundice, leg ulcers, splenomegaly, signs of underlying disease
FBC= reduced Hb, increased reticulocytes, Schistocytes on blood film
Tx= folate and iron supplementation, immunosuppressives, splenectomy

47
Q

What does bone marrow failure result in(aplastic anaemia)? Causes? Signs/ symptoms? Investigations? Tx?

A

Reduced number of pluripotent stem cells, lack of haemopoiesis
Congenital, acquired, cytotoxic drugs/ radiation, infections
Increased infection susceptibility, bruising, bleeding- nose and gums esp
FBC= pancytopenia, reticulocyte count- low, bone marrow biopsy- hepatocellular marrow with increased fat spaces
Removal of causative agent, blood/ platelet transfusion, bone marrow transplant, immunosuppressive therapy

48
Q

What is polycythaemia? Primary causes? Secondary causes?

A

Increased Hb, packed cell volume (PCV,) and RBCs
Increases bone marrow cells sensitivity to EPo= increased RBC production= polycthaemia rubra vera- genetic mutation in JAK2 gene, primary familial and congenital polycthaemia- mutation in EPOR gene
More RBCs due to more circulating EPO= chronic hypoxia, poor O2 delivery, abnormal RBC structure and tumours increased EPO levels

49
Q

Symptoms of polycythaemia? Investigations and Tx?

A

May present with no symptoms, easy bleeding/ bruising, fatigue, dizziness, headaches etc
FBC, bone marrow biopsy, genetic testing for JAK2 gene
Blood letting, aspirin- rubra vera
Secondary= treat the cause

50
Q

3 categories of red cell disorders?

A

Haemoglobinopathy- sickle cell disease, thalassemia
Membranopathy- spherocytosis, elliptocytosis
Enzymopathy- glucose 6 phosphate deficiency

51
Q

What is sickle cell anaemia? How do cells become sickled? Precipitated by what? Why can patients feel well despite being anaemic?

A

Autosomal recessive disease, commoner in Afro-Caribbean
Point mutation of B globin gene (valine to glutamine) resulting in HS variant
HbS= insoluble and polymerises when deoxygenated, become rigid, lose membrane flexibility–> shortened lifespan (haemolysis) and impaired passage through microcirculation
Infection, dehydration, cold, acidosis or hypoxia
HbS releases O2 to tissues more readily than normal

52
Q

Presentation of SCA in heterozygous state? In homozygous state?

A

Symptom free except in hypoxia- vasoconstriction-occlusive events may occur
Vaso-occlusive events- acute pain in hands and feet (dactylitis), long bones in adults, CNS infarction, acute chest syndrome, pulmonary hypertension, anaemia- acute drop due to: splenic sequestration, bone marrow aplasia

53
Q

Long-term issues of SCA?

A

Growth and development- short until adulthood, below normal weight, sexual maturation delayed
Avascular necrosis of hips and shoulders, compression of vertebrae and shortening of bones in hands and feet
Osteomyelitis- due to bacteria
Cardiac issues- cardiomegaly, arrhythmias, MI
Neurological- TIA, fits, cerebral infarction and coma
Chronic hepatomegaly, nephritis, retinopathy, spontaneous abortion

54
Q

Diagnosis and Tx of SCA?

A

Neonates= blood/ heel prick test, Hb and reticulocyte count, sickled erythrocytes on blood film, Hb electrophoresis for dx, HbSS present and absent HbA
Aggressive analgesia, treat cause, fluids, folic acid, transfuse with falling Hb
Transfusion, stem cell transplant, hydroxycarbamide
Hypsplenic= prophylactic acid Abx, pneumococcal and meningococcal vaccination

55
Q

What is thalassaemia? Precipitation of global chains in red cell precursors and in mature red cells causes what? 2 types? Common where?

A

Autosomal recessive disease of unbalance Hb synthesis within red cell precursors/ mature red cells, with under production/ no production of one global chain
Ineffective erythropoiesis and haemolysis
Alpha and Beta
From the Mediterranean to the Far East

56
Q

Epidemiology of Beta thalassaemia? Pathophysiology?

A

Little or no B chain production–> excess alpha chains= combine with whatever delta and gamma chains produced–> increased HbA2 (Hb delta) and HbF (Hb gamma), caused by over 200 genetic defects
Defects normally point mutations–> defects in transcription, RNA splicing and modification, translation via frame shifts and nonsense codons–> highly unstable B-globin cannot be utilised
Heterozygois= asymptomatic microcytosis with or without mild anaemia

57
Q

3 types of Beta thalassaemia?

A

B-thal minor- mild/absent anaemia, low MCV and MCH, iron stores and ferritin normal
B-thal intermedia- moderate anaemia, transfusions not required, splenomegaly, bone abnormalities, recurrent leg ulcers and gallstones
B-thal major= homozygous, presents in 1st year of life with severe anaemia, failure to thrive and chronic infections, bony abnormalities, hepatosplenomegaly

58
Q

Investigations and treatment for Beta thalassaemia?

A

FBC and film hypo chromic and microcytic anaemia, irregular and pale RBCs, increased reticulocytes and nucleated RBCs, diagnosis by Hb electrophoresis- shows increased HbF and absent/ low HbA

Blood transfusions= 2-4 weekly
Give iron chelation to decrease iron loading, ascorbic acid= increased urinary excretion of iron
Long term folic acid
Other= bone health, endocrine supplements and psychological support, promote fitness and healthy diet

59
Q

How many genes control production of alpha globin chains? Thalassaemia caused by what? 4,3,2 and 1 deletion causes what? Carriers protected from what?

A

4 genes control production, caused by gene deletions
4 deletions= no chain synthesis, Hb Barts cannot carry oxygen and is incompatible with life- infants= stillborn, pale, oedematous with huge livers and spleens
3 deletions= common in parts of Asia, low levels of HbA and Hb Barts, severe haemolytic anaemia and splenomegaly, sometimes transfusion dependent
2 deletions= asymptomatic with possible mild anaemia, 1 deletion= blood picture normal
Falciparum malaria

60
Q

What are membranopathies? Symptoms? Investigations and Tx?

A

Autosomal dominant condition–> deficiency in protein use to make red cell membrane–> deformed cells-> trapped in spleen (extravascular haemolysis)
Spherocytosis (vertical deformity,) elliptocytosis (horizontal deformity)
Neonatal jaundice and haemolytic anaemia exacerbated during infection (splenomegaly), excess bilirubin= gallstones
FBC and reticulocytes count, blood film; osmotic fragility tests (RBCs show fragility in hypotonic solutions)
Tx= folic acid and splenectomy

61
Q

Epidemiology of glucose-6-phosphate deficiency? Pathophysiology?

A

Heterogenous X-linked- more common in males, Africa, around the Mediterranean and in the Middle East and SE Asia
Presents with haemolytic anaemia- mutations mostly single amino acid substitutions
G6PD is vital for reaction necessary for RBCs, provide NADPH which is used with glutathione to protect RBC from oxidative damage from compounds like H2O2–> reduced RBC lifespan

62
Q

Presentation of G6PD deficiency, diagnosis and Tx?

A

Most asymptomatic, may get oxidative crisis due to reduction in glutathione production, precipitated by:
acute drug-induced haemolysis e.g. primaquine, sulphonamides, nitrofurantoin, quinolones, dapsone
In attacks= rapid anaemia, jaundice
Chronic haemolytic anaemia
Neonatal jaundice
Blood film= bite and blister cells, increased reticulocytes
Diagnosis= enzyme assay
G6PD levels= low
Tx= stop offending drugs, blood transfusion may be lifesaving, splenectomy= not usually helpful

63
Q

What are platelets regulated by produced by the liver? Binds to what receptors? Lifespan and normal count of platelets? Removed by what organ?

A

Thrombopoietin–> platelet and MK receptors
Decrease in platelets= less bound TPO= more TPO able to bind to MK= increased platelet production
7-10 days, normal count= 150-400 x10 9/L
Spleen

64
Q

Important platelet receptors?

A 
Thromboxane A2 (TXA2)- from arachidonic acid in platelets via cyclooxyrgenase (COX-1)--> platelet aggregation and vasoconstriction 
P2Y12- activated by ADP, amplifies platelet activation and helps activate glycoprotein IIb/ IIIa
Glycoprotein IIb/ IIIa= fibrinogen and von Willebrand Factor receptor, aids platelet adherence and aggregation
65
Q

Causes of platelet dysfunction?

A

Reduced platelet number: decrease in production, increase in destruction
Normal numbers but reduced function: congenital abnormality in platelet function, medication e.g. aspirin, von Willebrand disease, uraemia

66
Q

What is thrombocytopenia? What can cause decreased production? Increased destruction?

A

Deficiency of platelets in the blood
Congenital- malfunctioning platelets in BM
Infiltration of BM- leukaemia, metastatic malignancy, lymphoma, myeloma, myelofibrosis
Low B12/ folate/ reduced TPO, medication, toxins e.g. alcohol, infections e.g. HIV/ TB, aplastic anaemia
Myelodysplasia

Autoimmune- immune thrombocytopenia purpura (ITP); primary/ secondary
Hypersplenism- portal hypertension and splenomegaly, drug related immune destruction e.g. heparin induced thrombocytopenia
Platelet consumption- disseminated intravascular coagulopathy (DIC), thrombotic thrombocytopenia purport (TTP)

67
Q

Is ITP or TTP more common? Antibodies coating the platelets are removed by binding to what receptors on macrophages? Clinical features? Investigations? Tx?

A

ITP
Fc receptors on macrophages
Easy bruising, purpura, epistaxis/ menorrhagia
Reduced platelets so normal/ increased megakaryocytes, may have detection of platelet autoantibodies
Corticosteroids i.e. prednisolone, splenectomy, IV IG, anti D

68
Q

Physiology of thrombotic thrombocytopenia purpura? Clinical features? Investigation? Tx?

A

Microvascular clots form in small vessels in body, resulting in low platelet count and organ damage
Easy bruising, purpura, epistaxis/ menorrhagia
Reduced platelets so normal/ increased megakaryocytes
Tx= plasma exchange and immunosuppression, IV methylprednisolone, IV rituximab

69
Q

HbA made of what? HbF? HbA2? %s of each in an adult?

A 
HbA= haem + 2 alpha chains + 2 beta chains 
HbF(foetal)= haem + 2 alpha chains and 2 gamma chains 
Hb delta (HbA2)= haem + 2 alpha chains and 2 delta chains 
HbA= 97%, HbA2= 2%, HbF= 1%
70
Q

Patients that might be over anti coagulated? Why may this be? Symptoms? Assessing bleeding x2?

A

Vit K antagonists e.g. warfarin, NOACs e.g. apixiban
Bad pt compliance, artificial valves, new/ interacting drugs
Brusing, bleeding, melena, epistaxis, hematemesis, haemoptysis
APTT (activated partial thromboplastin time)= intrinsic pathway
PTT(prothrombin time)= extrinsic pathway

71
Q

What is disseminated intravascular coagulation? (DIC) Causes? Tx?

A

Generation of fibrin+ consumption of platelets/ coagulation factors causing sec activation of fibrinolysis
Malignancy, septicaemia, obstetric causes, trauma, infections, haemolytic transfusion reactions, liver disease
Tx= treat underlying cause- maintain blood volume and tissue perfusion, may need transfusions, activated protein C
Fresh frozen plasma (FFP) to replace coag factors
Cryoprecipitate to replace fibrinogen and some coagulation factors
Red cell transfusion in bleeding patients

72
Q

2 haemophilia types? Epidemiology? Symptoms? PTT and APTT times and pathways?

A 
A= factor 8 deficiency, Tx= IV factor 8
B= factor 9 deficiency 
X-linked recessive, A= more common 
Anything associated with bleeding
Normal PTT but prolonged APTT
PTT--> extrinsic pathway, APTT--> intrinsic pathway
73
Q

What is tumour lysis syndrome? At risk patients?

A

Malignant cells breakdown resulting in euro, cardio and renal complications
High uric acid, hyperkalaemia, hyperphoshatemia, hypocalcaemia
High tumour burden, high grade disease i.e. rapid cell turnover, pre-existing renal impairment, increasing age, drugs that increase uric acid formation e.g. alcohol
Tx= aggressive hydration, allopurinol or rasburicase= reduce uric acid production, monitor electrolytes, refer to dialysis if required

74
Q

What are counted as haematological emergencies? What is neutropenia defined as? At risk patients?

A

Febrile neutropenia, acute sickle cell crisis, chest crisis, spinal cord compression
Temp above 38 degrees, absolute neutrophil count< 1.0 x10 9/L
Chemo less than 6 weeks ago, stem cell trans/ high dose chemo within last year, aplastic anaemia, autoimmune disease, leukaemia
People on methotrexate, carbimazole and clozapine

75
Q

Presentation of neutropenia and management?

A

Pyrexia, malaise, sweats/ rigors, cough, sore throat, abode pain or diarrhoea, pain/ erythema around central venous catheter, tachycardia, hypotension and raised resp rate
Broad spec IV antibiotics without waiting for results within 1 hour of admission
Do not catheterise

76
Q

Patients at risk of malignant spinal cord compression? Clinical presentation?

A

Bone metastasis and vertebral collapse, local tumour extension, deposition of malignant cells within cord
Myeloma, lymphoma

Back pain, weakness/ numbness in legs, inability to control bladder/ bowel, saddle paraesthesia, uni/bilateral leg weakness, decreased perianal sensation and anal tone, acute= tone and reflexes reduced

77
Q

Management of malignant spinal cord compression?

A

Time= nerves, strict bed rest, high dose steroid e.g. oral dexamethasone, analgesia, urgent MRI of whole spine

78
Q

What is hyper viscosity syndrome? Usually due to high levels of what? Seen in what conditions? Can be due to what? Results in what?

A

Increase in blood viscosity (thickness,) high levels of Igs
Multiple myeloma and Waldenstrom macroglobulinaemia
Due to high cell numbers e.g. leukaemia or polycthaemia
–> vascular stasis and hypo perfusion

79
Q

Clinical presentation of hyperviscosity syndrome?

A

Mucosal bleeding, visual change due to hypo perfusion to retina, neurological disturbance due to brain hypo perfusion: vertigo, hearing loss, paraesthesia, ataxia, headache, seizure or stupor, SOB, fatigue, bruising/ bleeding, dilated retinal veins, ataxia/ nystagmus, evidence of volume overload

80
Q

Diagnosis and Tx of hyperviscosity syndrome?

A

Plasma viscosity level, CT head to exclude other causes of neurological signs, Ig levels, FBC
Keep hydrated, avoid transfusion= thicker, plasmapheresis- remove circulating Igs to decrease serum viscosity, symptoms will improve

Phase 2a Flashcards (14 decks)

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