Haematology Flashcards

1
Q

3 classic Sx of anaemia?

A

fatigue, dyspnoea, faintness

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2
Q

When is anaemia severe? name 2 signs ?

A

< 8g/dL

Tachycardia
Flow murmur
Cardiac enlargement
Increased cardiac output

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3
Q

what transports iron?

A

transferrin

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4
Q

where is iron stored

A

ferritin, haemosiderin

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5
Q

Name 3 things on presentation with Fe deficiency anaemia

A

Pallor
Nail changes - Koilonychia (spoon), brittleness
Hair loss
Mouth changes - Angular stomatitis, atrophic glossitis

Classic 3
Fatigue
Faintness
Dyspnoea

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6
Q

What are the general causes of iron deficiency

A

Inadequate intake
Poor absorption
Excessive loss
Excessive iron requirement

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7
Q

Name 3 Ix in Iron deficiency anaemia and 2 things seen on bloods

A

Hb <13g/dL (men), <12g/dL (women)
MCV - microcytic
Peripheral blood smear
Iron studies - Low iron / ferritin

Ix for cause
Coeliac (malabsorption), H. pylori (basically eats iron), endoscopy (bleed or Malab)

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8
Q

Why might ferritin be unexpectedly high in iron deficiency

A

It is an acute phase protein so will increase if inflammation, infection and malignancy

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9
Q

2 key DDx for iron deficiency anaemia

A

chronic disease
sideroblastic anaemia (bone marrow produces sideroblasts rather than normal erythrocytes)

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10
Q

mx of iron deficiency

A

oral replacement - ferrous sulphate

consider transfusion

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11
Q

SE of ferrous sulphate

A

constipation, black stools, vomit

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12
Q

When would you think this pt has sideroblastic anaemia

A

microcytic hypochromic anaemia NOT responding to iron

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13
Q

Pathology and What causes sideroblastic anaemia

A

Ineffective erythropoesis - cant incorporate iron into Hb

Congenital - inherited XLSA (x-linked)

Acquired - MDS (myelodysplastic syndrome), myeloma, PRV, pyridoxine (B6) deficiency

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14
Q

name 3 Ix in sideroblastic anaemia. Make sure you know the last one

A

Hb - low
MCV - microcytic
Peripheral blood smear
Iron studies - Iron / ferritin high

Marrow aspirate - Perinuclear ring of iron granules with Prussian Blue

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15
Q

Mx of sideroblastic

A

iron chelation (gets rid of excess iron in body) - desferrioxamine
Avoid alcohol / vit c (increase iron absorption)

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16
Q

What happens in b thalassaemia

A

Inherited microcytic anaemia caused by mutation in beta-globin gene

-ineffective erythropoesis
Erythroid hyperplasia

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17
Q

2 Effects of erythroid hyperplasia in b thalassaemia?

A

Bony changes - skull bossing, vertebral….

hepatosplenomegaly

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18
Q

Name 3 Ix in b thalassaemia

A

FBC
Peripheral blood smear
Hb anaylsis
LFT
Xray skull for skull bossing
Abdo USS for hepatosplenomegaly

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19
Q

b thal mx points

A

genetic councelling
transfusion
Iron chelation

splenectomy
Bone marrow transplant

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20
Q

name 3 complications of thalassaemia

A

thrombotic
iron overload - heart arrhythmia, pituitary, pancreas

transfusion reactions

transfusion infections

splenectomy comps

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21
Q

Reticuloendothelial causes of haemolysis

A

Macrophages of liver

Spleen

Accelerated red cell destruction due to immune targeting by antibodies

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22
Q

Name 3 hereditary causes of haemolytic anaemia

A

Glucose 6 phosphate dehydrogenase deficiency

Hereditary spherocytosis,

Sickle cell anaemia, thalassaemia

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23
Q

Test for immune mediated haemolytic anaemia

A

direct antiglobulin +ve (Coombs’ positive)

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24
Q

Name 3 causes of non immune mediated haemolytic anaemia

A

Infection (e.g. malaria), trauma,

microangiopathic haemolytic anaemia (DIC, TTP, HUS, HELLP)

hypersplenism, liver disease

Paroxysmal nocturnal haemoglobinuria

trauma - eg mechanical heart valve

complement mediated lysis

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25
Dx of Paroxysmal nocturnal haemoglobinuria
urinary haemosiderin People with this condition have paroxysmal episodes of haemolytic due to things like infection
26
3 Ix in haemolytic anaemia
FBC - low Hb Unconjugated bilirubin Urinanalysis - haemaglobinuria Peripheral blood film Direct coombs test = test antibodies ON red blood cells
27
In haemolytic anaemia there is increased breakdown - what do you see in blood (and one other place) as a result of this?
Increased bilirubin, increased urobilinogen [not blood obvs], increased LDH
28
In haemolytic anaemia there is increased production - what do you see as a result of this?
Increased MCV as there are increased reticulocytes (bigger) marrow hyperplasia
29
How might you differentiate between Extravascular or intravascular haemolytic anaemia
Intravascular - increased free plasma Hb and decreased haptoglobin, increased haemoglobinuria (no blood cells) Extravascular - splenomegaly
30
What supplement is usually given in haemolytic anaemia
folic acid
31
How does G6PDD present? Triggers?
Presents with prolonged neonatal jaundice In older: Jaundice Pallor Dark urine (suggests intravascular haemolysis) Nausea, vomiting, dehydration, AKI (haemoglobin precip) Infection Broad beans Certain drugs (causing oxidative stress) e.g. sulphonamides, *cephalosporins
32
mx of G6PDD
Fluid intake folic acid blood transfusion if severe anaemia renal support if needed
33
what is anaemia of chronic disease
Anaemia and evidence of immune system activation - decreased RBC production + decreased survival
34
Whats seen on lab studies of anaemia of chronic disease?
Normocytic normochromic/microcytic hypochromic Low reticulocyte count Low serum iron Low TIBC Low transferrin saturation Elevated ferritin
35
1 test that can differentiate anaemia of chronic disease and iron deficiency
ferritin - increased in ACD
36
How does aplastic anaemia present? What is it?
Autoimmune destruction of RBCs. Neutropenia - infections Anaemia - fatigue, pallor, dyspnoea, faintness Thrombocytopenia - bleeding, bruising
37
Name 2 associations with aplastic anaemia
pregnancy, coeliac, SLE, paroxysmal nocturnal haemoglobinuria
38
Seen on bone marrow biopsy of aplastic anaemia
Hypocellular marrow with no abnormal cell population
39
2 parts of Non pharma mx of aplastic anaemia
MASSC risk assessment if NP< 0.5 [risk of febrile neutropenia] Full barrier nursing Avoid IM injections Look for infection Check bloods and cultures Vitals 4 hourly
40
Medical Mx of aplastic
allogeneic BM transplant + RBC and Pt transfusion + ABX
41
2 issues with crescent shaped Hb in sickle cell
Disrupt blood flow and break (haemolysis) - Painful crises -Organ damage -Increased vulnerability to infection Cause varying degrees anaemia
42
what triggers sickle cell crisis?
CHIDS: Cold Hypoxia (extreme exercise) Infection Dehydration Stress
43
3 areas affected in exam land by sickle cell crisis?
Acute chest syndrome: pneumonia like syndrome (due to sickling in pulmonary vasculature) - chest pain, fever, dyspnoea, tachypnoea Bone infarction and avascular necrosis femoral head Acute abdomen - mesenteric ischaemia
44
When is sickle cell usually Dx? Mx at this point?
newborn screening - blood spot pneumococcal vaccine and penicillin prophylactically genetic counselling parental education
45
mx of sickle cell crisis
cross match analgesia O2 IV vluids Abx -broad Blood if needed
46
Chronic Mx of sickle cell
Supportive -Pain -Pneumococcal vaccination and penicillin prophylaxis -Trigger avoidance -Folic acid if severe haemolysis Hydroxyurea - Increases fetal Hb production Repeat transfusions if Hb <10g/dL
47
Name 3 comps of sickle cell
crisis anaemia liver - jaundice / gallstones iron overload from transfusion leg ulcers
48
What causes megaloblastic anaemia?
B12/folate deficiency + drugs (hydroxyurea) This causes defective DNA synthesis which also leads to leukopenia and thrombocytopenia
49
Where is folate found naturally
green veg nuts liver
50
4 causes of folate deficiency
Poor diet - eg alcoholic , poverty Increased demand - eg pregnancy renal disease Malabsorption - coeliac/ tropical spure Drugs, alcohol and methotrexate
51
signs of folate deficiency
Headache - hallmark of megaloblastic Anaemia Pallor, fatigue, dyspnoea, faintness, tachycardia, heart murmur Gi Loss of appetite/wt loss Skin glossitis, exfoliative dermatitis
52
Mx of folate deficiency
oral folic acid
53
What do you get with b12 deficiency
macrocytic anaemia with peripheral neuropathy and neuropsych complaints
54
foods rich in b12
meat, fish, dairy [vegans always lacking]
55
which cell and what do they produce is needed for b12 absorption
parietal - intrinsic factor
56
neuro sx of b12 deficiency
Subacute degeneration of spinal cord [peripheral neuropathy, demetia, ataxia, paraethesia]
57
triad of signs Subacute degeneration of SC?
Upgoing plantars Loss knee jerk Loss ankle jerk
58
mouth signs b12 deficiency
Glossitis (painful), angular cheilitis
59
mx of b12 deficiency if severe (pancytopenia + anaemia + neurological)
IM hydroxycobalamin (cobalamin = b12) oral folic acid blood transfusion admit neuro / haem
60
Mx of b12 deficiency if no neuro o
IM hydroxycobalamin
61
What is pernicious anaemia
Autoimmune atrophic gastritis -> Atrophy of gastric mucosa with failure of IF and acid production due to autoantibodies to IF
62
Association wit pernicious anaemia? increased risk of ?
AI diseases - thyroid, DM, vitiligo.... Gastric Ca
63
Specific Ix for pernicious
IF antibody antiparietal antibody Schilling test (radio b12)
64
What is leukaemia
Excess of abnormal white cells in peripheral blood - myeloid or lymphoid
65
Acute leukaemia 3 Sx categories?
Tumour related: Bone pain, fever, lethargy, night sweats, wt loss BM sx Anaemia thrombocytopenia neutropenia Circulating cell sx headache, lyphadenopathy, hepatosplenomegally
66
Dx of AML
Bone marrow blasts > 20% or peripheral blood [may be complication of chemotherapy] [?most common in old age]
67
Ix in AML name 3
FBC - leukocytosis, neutropenia Peripheral blood film - blasts + auer rods Bone marrow biopsy - blasts >20% CXR - pulm infiltrates Coagulation UE, LFT, Coagulation
68
name 2 DDx of AML
ALL myelodysplastic syndrome aplastic anaemia
69
What is tumour lysis syndrome? electrolytes?
Electrolyte and metabolic disturbance due to breakdown of large number leukaemic cells (hyperuricaemia, hyperphosphataemia, hyperkalaemia, hypocalcaemia, renal impairment)
70
Mx of tumour lysis syndrome?
IV fluids allopurinol [haemodyalysis if needed]
71
Drug for leukoreduction in AML?
Hydroxycarbamide
72
2 parts Mx AML ?
Chemo - [Cytarabine and daunorubicin (don't think need to know)] Allogenic transplant at first remission
73
Dx of ALL
Bone marrow blasts > 20%, blood smear leukaemic lymphoblasts
74
Chromosomal association with ALL
Philadelphia chromosome - translocation (9,22
75
ALL affects CNS. How present?
CNS infiltration by leukaemoid cells presents as papilloedema, nuchal rigidity and meningismus May also have a focal neurology (CN 3, 4, 6, 7)
76
ALL ix
FBC - peripheral blood film coagulation pannel bone marrow biopsy CXR , LP UE, LFT, renal
77
seen on CXR of ALL
Mediastinal widening
78
Name 3 Comps of ALL
Tumour lysis syndrome Neutropenic sepsis Pancytopenia Chemotherapy side effects
79
Name 3 parts of acute Mx for ALL
Supportive care: -Hydration - [electrolyte abnormalities] -Allopurinol - [for acute tumour lysis syndrome (increased urate)] -Prophylactic antimicrobials -Think bacterial/viral/fungal (aciclovir + fluconazole + ciprofloxacin) Transfusions - RBC + platelets (if pt<10 x 10^9) Induction chemotherapy (kill leukaemic cells) +- tyrosine kinase inhibitor (imatinib) Intrathecal methotrexate (for CNS disease)
80
CML genetics
Philadelphia chromosome/ BCR-ABL fusion gene Confirms Dx
81
mx of CML
Tyrosine kinase inhibitor (imatinib / dasatinib) allogeneic haematopoeitic stem cell transplant high-dose induction chemotherapy
82
Cell in CLL? what happens?
B lymphocytes avoid apoptosis -> invade liver spleen and bone marrow -> Lymphadenopathy, hepatosplenomegaly, BM suppression
83
most common leukaemia of old age? trigger?
CLL Pneumonia
84
Sx of CLL
Anaemia SOB / fatigue... Infiltration - Lymphadenopathy , splenomegaly B sx Fever Night sweats Weight loss [Fatigue, Chills]
85
name 3 Ix in CLL
FBC - lymphocytosis , low hB, low pt Peripheral blood film - smudged cells (damage to lymphocytes in prep) Flow cytometry CD5, CD19, CD23 positive CT Hepatosplenomegaly, retroperitoneal, mediastinal lymph nodes
86
Mx of CLL
chemotherapy: [rituximab + cyclophosphamide + fludarabide] +/-stem cell transplant
87
2 comps and their Mx in CLL
Hypogammaglobulinaemia - cant produce antibodies -> give monthly IVIg Autoimmune haemolytic anaemia -> pred
88
Difference between lymphomas and leukaemia
Lymphomas are solid, leukaemias are circulating
89
Most common type of non-hodgkins ? what can it produce?
is diffuse large B-cell lymphoma (mainly from B cell lines) May produce immunoglobulins. [T cells can travel to extranodal sites e.g. skin and CNS]
90
Associations with NHL
AI disorders (Sjogren’s, RA, SLE, coeliac), immunodeficiency
91
How does NHL present?
Lymphadenopathy Dry cough - mediastinal mass / pneumonia Bone marrow spenomegaly GI B Sx Night sweats Fever Weight loss
92
3 Ix in NHL
FBC - Thrombocytopenia (liver or BM), or pancytopenia (BM) Lymph node biopsy - (for flow cytometry - tumour surface markers and cytogenetics) LDH - high CT/MRI - staging
93
NHL how might you provide CNS prophylaxis? Antimicrobial?
intrathecal methotrexate ciprofloxacin + aciclovir + fluconazole
94
HL from what cells? most common presentation? what cells are seen?
Arises from mature B cells Most commonly presents with cervical or supraclavicular lymphadenopathy (painless) -B symptoms in 30% of patients Presence of Reed-Sternberg cells (large, multinucleated
95
HL aetiology?
Immunodeficiency EBV AI - SLE
96
Often get mediastinal adenopathy in HL -> name 3 sx?
Dry cough Dyspnoea Chest pain Superior vena cava syndrome (facial and upper limb oedema, dilated vessels)
97
What is multiple myeloma? how is it therefore diagnoses?
Clonal proliferation of plasma cells in the bone marrow associated with monoclonal element (Ig or Ig fragment) in serum or urine -> Diagnosis by serum and urine protein electrophoresis
98
Most common presentation of MM
Bone pain (esp back pain!) Anaemia [Fatigue Infections Hypercalcaemia Renal impairment]
99
Key protein in MM
M-protein - monoclonal component
100
Which organs / features involved in MM
CRAB Calcium elevation Renal insufficiency Anaemia (Hb < 10g/dL Bone disease - lytic or osteopenic
101
2 Ix you should do in all over 50 with back pain?
serum protein electrophoresis ESR
102
Sx groups in MM
Osteolytic lesions (backache / fractures) Anaemia, neutropenia, thrombocytopenia from BM infiltration Recurrent infections Renal impairment
103
What causes renal impairment in MM
Deposition of Tamm-Horsfall protein in loop of henle
104
gold standard Ix in MM
Serum/urine electrophoresis
105
bar Serum/urine electrophoresis, what other Ix might you do MM? name 3
Skeletal survey BM aspiriate Blood film FBC, ESR, UE, Ca
106
MM mx of comps... Bone disease? anaemia? spinal cord compression? hyperviscosity -> reduced cognition / blurred vision? AKI? Infection?
Bone disease: Hypercalcaemia + pain - bisphosphonates + analgesics Anaemia - blood transfusion/EPO Spinal cord compression - dexamethasone Hyperviscosity (reduced cognition/blurred vision) - plasmapheresis AKI - rehydration/ preserve good hydration Infection - antibiotics + pneumonia/flu vaccine
107
What is polycythaemia? 2 cause categories
increased proportion of haemoglobin in the blood Relative - decreased plasma volume e.g dehydration, smoking, obesity Absolute - increased RBC Eg polycythaemia vera, altitude, chronic lung disease
108
PRV -> More RBCs (also WBC and pt a bit). What comps?
Hyperviscosity -> thrombosis, haemorrhage progress -> myelofibrosis and acute leukaemia
109
genetics of PRV
JAK2
110
Sx categories of PRV
Hyper-viscous - headache, visual change, dizzy, red/full apperance Thrombosis /bleeding Bath Pruritus after hot bath, erythromelalgia (pain) + redness of fingers, palms, heels toes
111
3 key Ix in PRV
FBC Raised haemoglobin, raised haematocrit, raised WBC, raised Pt JAK2 gene mutation screen (e.g. PCR) BM biopsy
112
in Polycythaemia Vera treatment aims to keep a low haematocrit and reduce the risk of thrombosis... What drug? what other mx?
hydroxycarbamide [Reduces number of blood cells produced by bone marrow - So also used in CML] (+aspirin) Management of CV risk factors (DM, hyperlipidaemia, HTN, smoking)
113
Differentiate PV and CML
PV has no philadelphia chromosome Neutrophil alkaline phosphatase is raised in PV but decreased in CML
114
3 characteristics of myelodysplastic syndrome?
dysplastic changes in one or more cell lineages ineffective hematopoiesis likely to develop AML
115
How does Myelodysplastic syndrome present
anaemia neutropenia thrombocytopenia
116
Name 3 Ix in Myelodysplastic syndrome
FBC Blood film - diamorphic blood cells Ferritin, B12, renal function, LFT, CXR ECG BM biopsy
117
What anaemia described ... [Congenital dysmorphic features (what?) Pancytopenic bone marrow failure Susceptibility to cancer ] Age?
faconi [Sounds Italian - think of darker skin, italian hand shape which has a weird thuumb and in shape of triangle and could be a odd shaped heart - DIS SHIT TENUOUS] Triangular faces Cafe au lait + hyperpigmented skin Cardiac and renal malformations Abnormal thumbs <7 - similar to aplastic/AML/Myelodysplastic
118
what regulated production of patelets?
thrombopoeitin (from liver)
119
When does ITP usually occur?
children with a preceding viral illness
120
3 Ix in ITP
FBC Peripheral smear - Rule out other cause BM biopsy
121
how can ITP be differentiated from Thrombocytopenia due to liver disease? TTP? DIC?
Thrombocytopenia due to liver disease or alcohol - raised GGT, alk phos TTP - may have neurological changes or fever + anaemia DIC - prologed PT and aPTT
122
Mx of severe bleeding in itp
IVIG Pred pt transfusion
123
Mx of chronic ITP
rituximab + splenectomy
124
Thrombotic thrombocytopenic purpura is a medical emergency (95% fatal) What are the pentad of Sx ?
[Fuck Renal HTN] Fever Renal failure Haemolytic anaemia Thrombocytopenia Neurological change
125
What causes TTP
Absence of VWF cleaving protein (ADAMTS-13) [platelet aggregation in microvasculature (brain, kidney, heart) +Haemolysis]
126
seen on peripheral blood smear of TTP
Microangiopathic blood film with schistocytes (RBC fragments)
127
Mx of TTP ? what not?
Urgent plasma exchange + prednisolone + aspirin DO NOT GIVE PLATELETS
128
Which drug commonly used to target antibpdy production
rituximab
129
Pres of DIC
Symptoms/signs of systemic collapse: oliguria, hypotension, tachycardia Bleeding: bruising, purupura
130
2 things you transfuse in DIC
Platelet transfusion FFP - replace coagulation factors
131
can girls get haemophilia? Where is bleeding commonly \/ age of presentation?
no - x linked recessive- muscles and joints toddlers
132
When does WVD present? how?
teens Bleed into mucus membranes and skin
133
Haemophilia Avs B
A = F8 deficient B = F9 deficient
134
What should you avoid with haemophilia
IM injections, aspirin and NSAIDS
135
What can be given in mild haem A ?
desmopressin (IV) -> stimulates endogenous release F8 + VWF
136
genetic VWD
AD chromosome 12 Presentation - menorrhagia, telangiectasia, bruising and bleeding, gum bleeding
137
mx VWD
IV demopressin severe - + F8
138
HHT is? other name?
hereditary haemorrhagic telangiectasia Osler-Weber-Rendu
139
Vit K needed for synthesis of what factors
1972
140
What is prolonged in liver disease
PT time
141
What happens in the coagulation cascade
Series of proteolytic enzymes that circulate in inactive state which are sequentially activated Generate thrombin that cleaves fibrinogen creating fibrin = clot
142
In endothelium damage what do platelets adhere to?
collagen and VWF and GP1b
143
2 drugs affecting platelet function ? 2 for coagulation
Aspirin and clopidogrel affect platelet function Heparin and warfarin affect coagulation cascade
144
Aspirin mech?
Irreversibly inhibits COX1
145
Clopidogrel mech?
Irreversible P2Y12 antagonist
146
warfarin Mech
a vitamin K antagonist - prolongs PT
147
Eg of noac and mech?
FXa inhibitors - apixaban, rivaroxiban
148
Name some RFs of DVT
Antiphospholipid syndrome Cancer Slow flow - sickle cell, PRV Nephrotic syndrome Obesity Pregnancy Sedentry Protein C/S deficiency
149
what 2 Ix before transfusion
Group and screen Cross match
150
Name 3 comps of transfusion
infection Acute Acute haemolytic reaction Febrile, non-haemolytic reaction Allergic/anaphylactic reactio Delayed Iron overload Delayed haemolytic reaction Transfusion-associated graft vs host disease
151
2 main comps of splenectomy
thrombocytosis - platelets peak at 7-10 days - could give aspirin prophylactic infection
152
What prophylax in splenectomy
ABx phenoxymethylpenicillin + or macrolides (azithromycin/clarithromycin Pneumococcal vaccine and influenza vaccine
153
blood test to differentiate ALL and AML
nuclear staining for TdT - only in Lymphoblasts [not in lymphocytes or myleoblasts] myeloperoxidase in myeloblasts [seen as auer rods]
154
Key features of subtype monoblast AML
infiltrates gums often no myeloperoxidase
155
Key features of megakaryoblast AML
often no myeloperoxidase associated with downs
156
What is myelodysplastic syndrome ? usual cause of death?
blast buildup in bone marrow but not >20% ->can progress to AML Cytopenia -> infection + bleeding
157
Key difference in cells between acute and chronic leukemia
Chronic - partial maturation acute - no maturation
158
Seen on blood smear of CML vs CLL For Dx?
CML - increased granulocytes and monocytes CLL - smudge cells - due to immature B cells being damaged Dx: phildelphia chromosome = CML Chromosomal defects in CLL
159
How long for effects of change / initiation in warfarin? What should you co prescribe when initiating warfarin?
3 days [If initiating give 3 days of LMWH]
160
What is beriplex also called
prothrombin complex concentrate
161
Staging tool for lymphoma
Ann Arbor scale
162
Sickle cell crises
Veno-occlusive - Eg Long bones, GI, priaprism Acute chest Aplastic - preceded by parvovirus b19 Sequestration - massive spleen, decreased Hb, Hypovolaemic shock
163
HL Vs NHL in age ?
HL - younger - 20-34 50% EBV positive
164
Key associations with CML ?
Gout - purine breakdown Abdo distension
165
If you were going to give 1 drug for CML what would it be?
Imatinib
166
Which leukaemia has rule of 3rds?
CLL
167
DKA 5 aspects of Mx
fluids insulin detrose LMWH Kcl if needed