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MBBS / MBChB finals > Hematology > Flashcards

Hematology Flashcards

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1
Q

Risk factors for VTE

A 
Age >60
Surgery >30 mins
Immobilisation 
Trauma
Obesity
COCP
previous VTE
Congenital / acquired hypercoagulable states
Pregnancy
HF
Malignancy
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2
Q

Management of urticarial reaction to a blood transfusion

A

Slow / temporarily stop blood transfusion

IV / oral chlorphenamine

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3
Q

Symptoms of haemolytic transfusion reaction

A 
Burning at infusion site
Headache
Nausea
Chills
Fever
Chest tightness
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4
Q

In what condition howel jolly-bodies occur?

A

Hyposplenism

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5
Q

When do Burr cells occur?

A

Uraemia

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6
Q

When do Heinz bodies occur?

A

Glucose-6-phosphate dehydrogenase deficiency

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7
Q

When may reticulocytosis occur?

A

Bleeding, haemolysis

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8
Q

When may target cells occur?

A

Liver disease,

Iron deficiency anaemia

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9
Q

What causes macrocytic anaemia with glossitis and peripheral neuropathy?

A

B12 deficiency

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10
Q

What drugs may cause folate deficiency?

A 
Phenytoin
Trimethoprim
Methotrexate
Sulfasalazine
Oral contraceptive pill
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11
Q

Causes of a macrocytic anaemia?

A 
B12deficiency, 
folate deficiency, 
hypothyroidism
Alcohol excess
Reticulocytosis
Multiple myeloma
Myeloproliferative disorders
Aplastic anaemia
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12
Q

Causes of a Microcytic anaemia?

A

Iron deficiency
Thalassaemia
Sideroblastic anaemia

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13
Q

Causes of a Normocytic anaemia?

A

Chronic disease (E.g. malignancy, rheumatoid arthritis)
Haemolytic anaemia
Acute blood loss
Marrow infiltration

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14
Q

Signs and symptoms of sickle-cell anaemia?

A 
Anaemia
Shortness of breath
Dizziness
Headaches
Cold hands and feet
Pain
Splenic crisis
Infections
Priapism 
Stroke
Gallstones
Leg ulcers
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15
Q

What causes dark urine in the morning and a positive hams test?

A

Paroxysmal nocturnal haemalobinuria

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16
Q

Weightloss plus anaemia and dysphagia suggests what ?

A

Oesophageal carcinoma

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17
Q

Weightloss plus anaemia and painless obstructive jaundice suggests what ?

A

Carcinoma of the head of the pancreas

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18
Q

In what condition do reed Sternberg cells occur?

A

Hodgkin’s lymphoma

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19
Q

In what condition do auer Rods occur?

A

Acute myeloid leukaemia

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20
Q

In what condition does the Philadelphia chromosome occur?

A

Chronic myeloid leukaemia

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21
Q

Features of myeloma?

A 
Bone pain
Fractures
Nerve compression
Hypercalcaemia
Bone marrow failure --> Anaemia, Clotting problem, Infection
Hyperviscosity --> retinal haemorrhage 
Renal failure
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22
Q

What is myeloma?

A

malignant proliferation of plasma cells

Leading to bone marrow infiltration and bone destruction and bone marrow failure

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23
Q

Causes of pancytopenia

A

Aplastic anaemia,
Bone tumours,
hypersplenism,
sepsis,

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24
Q

Causes of excess erythropoietin?

A 
Polycystic kidney disease
Renal cell carcinoma,
adrenal tumour,  
hepatocellular carcinoma
Cerebellar haemangioblastoma
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25
Common symptoms of antiphospholipid syndrome
``` DVT Stroke Multiple miscarriage Placental infarction Migraine Livedo reticularis ```
26
Syx of anaemia
``` Fatigue Dizziness Palpitations Pallor Cold skin SOB Muscle weakness ```
27
Signs of anaemia
Low BP Tachycardia Splenomegally Pallor
28
What is thrombopoiesis
Platelet formation in the bone marrow | Formed from megakaryocytes
29
When are basophils raised
Allergy | Parasitic infection
30
When are eosinophils raised
Allergy
31
When are neutrophils raised
Acute inflammation
32
What are the primary lymphoid tissues?
Bone marrow | Thymus
33
What are the secondary lymphoid tissues
Lymph nodes Spleen Mucosal associated lymphoid tissue
34
What is phlebitis
Inflammation of a vein
35
What is thrombophlebitis
Inflammation of a vein associated with a clot
36
Risk factors for peripheral vascular disease
``` Male History CVD Increased age Family history DM Hypercholesterolaemia Hypertension ```
37
Symptoms of idiopathic thrombocytopenia purpura
Easy bruising Mucosal bleeding Menorrhagia Epistaxis
38
Features of thrombotic thrombocytopenic purpura (TTP)
``` Thrombocytopenia Microangiopathic haemolytic anaemia Neurological signs Renal impairment Fever ```
39
Features of hereditary haemorrhagic telangiectasia (HHT)
Telangiectasias On skin, lips and mucosal surfaces Small aneurysms on fingertips, face, nasal passage, tongue, lung, GIT. AV malformations Recurrent bleeding (E.g. Epistaxis)
40
Causes of thrombocytopenia
``` Leukaemia myeloma bone marrow cancer myelofibrosis B12 deficiency folate deficiency Marrow hypoplasia DIC ITP TTP EBV Gram negative septicaemia Hypersplenism Liver disease SLE ```
41
What is the most common congenital bleeding disorder?
Haemophilia A
42
What is the inheritance of haemophilia A?
X linked recessive
43
Features of haemophilia A?
Bruising Excess bleeding Spontaneous haemarthrosis Muscle haematoma
44
What is Christmas disease?
Haemophilia B
45
What is von Willebrand disease.
``` Common Mild Bleeding disorder Autosomal dominant Onset in adolescence ```
46
Features of von Willebrand disease
``` Bruising epistaxis menorrhagia GI haemorrhage gum bleeding ```
47
What may initiate DIC?
Infections Cancers Obstetric complications
48
Signs of DIC
Acutely ill patient Shock Bruising, purpura Initial thrombosis then bleeding e.g From mouth,nose, vene puncture sites Prolonged PT, APTT, TT Low fibrinogen End organ ischaemia --> Multiorgan failure
49
Causes of secondary polycythaemia
``` Chronic hypoxia (incl COPD) Renal cancer renal cyst hydronephrosis Hepatocellular carcinoma Cerebellar haemangioma ```
50
What is Fanconi anaemia
Inherited aplastic anaemia
51
Causes of haemolytic anaemia
``` Membrane defects Sepsis Sickle cell Thalassaemia Autoimmune Transfusion reaction Haemolytic disease of the newborn HUS DIC Malignant hypertension Mechanical valves Penicillin Malaria ```
52
What may precipitate sickling in sickle cell anaemia
Infection DeHydration Acidosis Hypoxia
53
What type of thalassaemia is most common?
Beta thalassaemia
54
What is wergener's granulomatosis
Necrotising small vessel vasculitis Multi system disorder Commonly involves lungs and kidneys Associated with cANCA
55
What is polycythaemia rubra vera
High Hb due to myelo-proliferation | Potential to transform into acute myeloid leukaemia
56
What does the prothrombin time best represent?
The intrinsic pathway | The effect of heparins
57
Causes of iron deficiency anaemia
Menorhagia Blood loss. E.g. GI Hookworm Malabsorption (coeliac)
58
Signs of iron deficiency anaemia
Koilonychia Atrophic glossitis Angular cheilosis Post-cricoid webs
59
Where is folate absorbed
Duodenum and proximal jejunum
60
Causes of folate deficiency
``` Poor diet Pregnancy Coeliac Alcohol Phenytoin Sodium valproate Methotrexate Trimethoprim ```
61
Causes of b12 deficiency
Decreased dietary intake (vegan) | Decreased absorption - pernicious anaemia, gastrectomy, crohn's, tropical sprue, ileal resection
62
Where is b12 absorbed
``` Terminal ileum (Bound to intrinsic factor) ```
63
Features of b12 deficiency
``` Pallor Mild jaundice Glossitis Red beefy tongue Subacute combined degeneration of the cord Paraesthesia Peripheral neuropathy Depression Psychosis Dementia ```
64
Management of a low-grade fever (No other symptoms) when giving a blood transfusion
Slow transfusion and prescribe paracetamol | Close monitoring
65
Presentation of an acute Haemolytic transfusion reaction
``` Starts within minutes Fever Chest pain Abdominal pain loin pain DIC Acute renal failure ```
66
Management of an acute Haemolytic transfusion reaction
``` Immediately stop transfusion Return blood and giving set Blood Bank Take blood for FBC, clotting screen and Coombs test ABC Fluid resuscitation Cryoprecipitate or FFP for DIC Transfer to HDU/ITU ```
67
Complications of massive blood transfusion
``` Hypothermia thrombocytopenia hyperkalaemia Hypocalcaemia Deranged clotting ```
68
What is DIC?
Acquired consumptive coagulopathy Widespread inappropriate activation of the clotting cascade. Conversion of fibrinogen To fibrin Multiple thrombi --> End organ ischaemia Intravascular haemolysis Platelet destruction Rapid consumption of clotting factors platelets and fibrinogen
69
Clotting studies in DIC
Prolonged PT | Prolonged APTT
70
Treatment of DIC
Correct cause | Platelets, FFP, cryoprecipitate
71
What is haemophilia A
X-linked recessive disorder of congelation Cannot synthesise factor VIII due to gene mutation Prolonged APTT (Extrinsic clotting Cascade) Normal PT (Intrinsic clotting Cascade)
72
What is haemophilia B
Christmas disease Inability to synthesise factor IX Prolonged to APTT Normal PT
73
Presentation of congenital haemophilia
Presents when child begins to crawl or walk Recurrent painful bleeds into joint and soft tissue Arthropathy and neuropathy
74
Treatment of haemophilia A
Factor VIII concentrate -Regular infusion or when actively bleeding Mild disease can be treated with desmopressin to release factor VIII from internal stores.
75
Treatment of haemophilia B
Factor IX concentrate
76
What causes a global reduction in clotting factor synthesis?
Chronic liver cirrhosis and hepatitis
77
Why is vitamin K essential in clotting factors synthesis
Carboxylation of clotting factors II, VII, IX, X
78
What does vitamin K deficiency do
Causes a hypocoagulable state
79
Common causes of inappropriately high INR While on warfarin
Dosing error, Accidental overdose, Drug interaction - Antibiotics, thyroxine, alcohol, antidepressants, aspirin, amiodarone, quinine
80
What is pernicious anaemia
Autoimmune loss of parietal cells And / or intrinsic factor Prevents absorption of b12
81
What does the schilling test do
Distinguishes pernicious anaemia from intestinal causes of b12 deficiency
82
What causes a macrocytic, megaloblastic anaemia with hypersegmented neutrophils
B12 deficiency
83
Management of b12 deficiency
IM hydroxocobalamin (preparation of b12) 1mg every other day until blood film normal Then 1mg every 3m
84
Management of anaemia secondary to chronic renal failure
Exogenous erythropoietin administration
85
Which haemoglobin chain is not produced in a beta-thalassaemia
The beta-globin chain
86
Presentation of beta-thalassaemia major
``` Presents in 1st year of life Failure to thrive Lethargy Pallor Jaundice Hepatosplenomegaly Frontal bossing Long bone deformity ```
87
Treatment of beta-thalassaemia major
Regular blood transfusions Regular iron chelation therapy - desferrioxamine Or allergenic bone marrow transplant
88
Presentation of beta-thalassaemia minor
Mild anaemia | Usually asymptomatic
89
What are the four variants of alpha-thalassaemia
Asymptomatic - 1 gene corruption Mild hypochromic anaemia - 2 gene corruptions HbH disease - 3 gene corruptions Death in uterine - all 4 genes corrupted
90
What drugs may trigger an autoimmune haemolytic anaemia
Methyldopa Penicillin Cephalosporins Quinine
91
What is the direct Coombs test
Used to test for autoimmune hemolytic anaemia. It detects RBC that are coated with autoantibodies Coombs agent = antibodies which are added to the RBC and adhere to any antibodies on the RBC causing RBC agglutination
92
What is the indirect Coombs test
indirect Coombs' test is done on the plasma. It detects antibodies that are present in the bloodstream and could bind to certain red blood cells and destroy them. Used in prenatal testing of pregnant women and testing blood prior to transfusion.
93
What is multiple myeloma
Multisystem disease | malignant proliferation of plasma cells
94
Investigation findings in multiple myeloma
Monoclonal band on serum Electrophoresis | Free immunoglobulin light chains in urine
95
Presentation of multiple myeloma
``` Lethargy Bone pain Pathological fracture Renal failure Amyloidosis Pancytopenia -due to marrow infiltration ```
96
Diagnosis of multiple myeloma
Two or more of: - Marrow plasmacytosis - serum/urinary immunoglobulin light chains (Bence-Jones protein) - skeletal lesions (osteolytic lesion, pepperpot skull pathological fracture)
97
Management of multiple myeloma
Bone pain control with analgesia, bisphosphonate, orthopaedic intervention Renal failure managed by increased fluid intake Broad-spectrum antibiotics for infection Erythropoietin for anaemia Blood transfusion for pancytopenia
98
Prognosis of multiple myeloma
Death Within 4 years Stem cell transplant offers hope of cure - but 30% mortality from treatment
99
Typical age of presentation of Hodgkin's lymphoma
Bimodal age distribution | Peak onset in third and sixth decades
100
Presentation of Hodgkin's lymphoma
Asymmetrical painless lymphadenopathy Usually as single rubbery lymph-node - cervical or inguinal or axillary Node may be painful after alcohol
101
B symptoms of Hodgkin's lymphoma
Weight loss sweating pruritis general lethargy
102
Diagnosis of Hodgkin's lymphoma
Lymph node biopsy showing Reed-Sternberg cells | CT for spread and staging (Ann Arbor staging system)
103
Management of Hodgkin's lymphoma
Early stage radiotherapy alone | Advanced/bulky disease radiotherapy and chemotherapy
104
What infection is Hodgkin's lymphoma associated with
History of glandular fever
105
What virus is non-Hodgkin's lymphoma associated with
Epstein Barr virus HIV Helicobacter pylori
106
Presentation of non-Hodgkin's lymphoma
Painless lymphadenopathy | +/- Systemic symptoms
107
Diagnosis of non-Hodgkin's lymphoma
Lymph node biopsy
108
Management of non-Hodgkin's lymphoma
CHOP chemotherapy
109
What is Burkitt's lymphoma
Type of non-Hodgkin's lymphoma which frequently involves the jaw Most often seen an African children with EBV
110
What is mycosis fyngoides
A cutaneous T-cell lymphoma
111
What is polycythaemia rubra Vera
Myeloproliferative disorder Mutation of a single pluripotent stem cell Causes excessive erythrocyte production
112
Features of polycythaemia rubra vera
Raised haemoglobin raised red-cell account raised Packed cell volume (Haematocrit) Viscous blood --> Increased risk of thrombosis and paradoxical bleeding
113
Symptoms of polycythaemia rubra Vera
Headache Lethargy Pruritus Pruritus is worse after bathing in warm water
114
Treatment of polycythaemia Rubra Vera
Venesection | Chemotherapy with hydroxyurea
115
Prognosis of polycythaemia rubra Vera
30% develop myelofibrosis | 5% develop acute myeloid leukaemia
116
What is myelofibrosis
A myeloproliferative disorder characterised by replacement of erythropoietic bone marrow with inert fibrotic material.
117
Presentation of myelofibrosis
Bone marrow failure - Pancytopenia | Extramedullary erythropoiesis in the liver and spleen produces massive hepatosplenomegaly
118
Diagnosis of myelofibrosis
Pancytopenia blood film - tear drop poikilocytes Dry bone marrow aspirate Trephine biopsy showed densify fibrosis of bone marrow
119
Management of myelofibrosis
Blood transfusion Chemotherapy Splenectomy
120
A bone marrow biopsy with excess plasma cells occurs in what condition
Multiple myeloma
121
Most common leukaemia in children
Acute lymphoblastic leukaemia (ALL) primarily affects 2-8 yo. equal risk between males and females
122
Are leukaemias more common in males or females
ALL is equally common in both genders all other leukaemias occur more frequently in males
123
Presentation of acute leukaemias
bone marrow failure: Anaemia – tired, SOB on exercise, weakness Bleeding and bruising – due to thrombocytopaenia Infection – result of leukopaenia Bone pain – result of bone marrow infiltration
124
auer rods are pathognomonic For what condition
AML
125
Is the presence of lymphoblasts in the blood more common in ALL or AML
ALL Always present (May or may not be present in AML)
126
Is the presence of bone and joint pain and hepatosplenomegaly more common in ALL or AML
ALL
127
Prognosis of ALL in childhood
Complete remission obtained in almost all patients. 80% alive without recurrence at 5 years. Failure of treatment occurs most commonly occurs in those with: a high blast count a T(9:22) translocation
128
What age group does chronic myeloid leukaemia commonly affect
Almost exclusively an adult disease peak age of incidence is 40-60 years.
129
What disease is characterised by the presence of the Philadelphia chromosome
CML
130
What cells does CML affect
CML affects the myeloid cells | i.e. basophils, neutrophils and eosinophils.
131
What is the natural progression of CML
The natural progression of CML is as follows: chronic phase → aggressive/accelerated phase → blast phase/crisis.
132
What is the most common leukaemia in adults
Chronic lymphocytic leukaemia (CLL) mainly in later life Median survival is 10 years
133
What cell abnormality is responsible for CLL
CLL results from a progressive accumulation of functionally incompetent B lymphocytes.
134
Presentation of CML and CLL
Many Asymptomatic, ``` or SOB due to anaemia, marrow failure immunosuppression, splenic pain, weight loss, fever and sweats, lymphadenopathy hepatosplenomegaly. ``` CML usually presents in the chronic phase CLL is often asymptomatic and discovered incidently.
135
Management of CML
Imatinib ( tyrosine kinase inhibitor) prevents the action of the BCR-ABL fusion protein = the abnormal protein produced by the Ph mutation Continue indefinitely
136
Management of CLL
Chlorambucil first-line therapy | combined with prednisone
137
What is a Myelodysplastic syndromes (MDS)
generally considered to benprecursors to leukaemia. 30% with MDS will develop acute myoblastic leukaemia (AML). generally occur in the elderly. Usually associated pancytopaenia.
138
What is Acute promyelocyte leukaemia (APML)
an uncommon variant of AML involves a specific translocation mutation, t(15:17). almost always coagulopathy - this is often the cause of death.
139
What is a blast crisis
The acute terminal phase of CML. Mortality is very high. blast crisis = > 20-30% blast cells in the blood or bone marrow. Symptoms include: - rapid increase in proportion of blast cells - fever - bone pain - fatigue - increased severity of anaemia, e.g. fatigue - increased severity of thrombocytopaenia, e.g. bleeding and/or bruising - splenomegaly - large clusters of blasts on bone marrow film
140
What is Richter syndrome
where CLL is transformed into an aggressive, large B-cell lymphoma. Epstein-Barr virus may play a role in transformation. Lymph node biopsy is necessary for diagnosis
141
H pylori increases the incidence of what lymphoma
gastric MALT lymphoma
142
What is MALT lymphoma
Mucos associated lymphoid tissue. Most commonly occurs in the stomach. Abnormallly large collection of lymphocytes and macrophages
143
Differentials of cervical lymph node enlargement
Infection - Acute - Infective mononucleosis - Toxoplasmosis - Infected eczema - Cat scratch fever – bartonella. . - Acute childhood exanthema –Includes Measles, rubella and scarlet fever Infection -Chronic - TB. - HIV. - Syphilis. - Sarcoidosis Malignancies - Primary - Hodgkin’s / Non-Hodgkin’s lymphoma - Chronic lymphocytic leukaemia / Acute lymphoblastic leukaemia Malignancies - Secondary - Naropharyngeal / Pharyngeal. - Thyroid - Lung. - Breast. - Stomach Others - Rheumatoid arthirits - Reaction to phenytoin - Kawasaki’s syndrome
144
Post splenectomy prophylaxis rules
Cary a splenectomy card Vaccination against pneumonia, HIB, Neisseria meningitides Prophylactic amoxicillin (erythromycin) until age 15
145
What is the upper age limit for bone marrow transplant
55
146
Complications of bone marrow transplant
Graft vs host disease Pneumonitis Infection Secondary malignancy
147
In CML do patients with or without the Philadelphia chromosome have a worse prognosis
Without Philadelphia = worse prognosis
148
What is tumour lysis syndrome
Seen I. Patients with a lymphoproloferative malignancy following chemotherapy. Massive cell death releases k+, phosphate, uric acid. Phosphate binds Ca2+ Hyperkalaemia, hyperphosphataemia, hyperuricaemia Hypocalcaemia
149
Management of tumour lysis syndrome
IV fluids Allopurinol (lower uric acid level) Renal support Correct electrolyte imbalance
150
What is the most effective way of preventing neutropenic sepsis in post-chemotherapy patients
Hand hygiene | Reverse barrier nursing
151
When do tear drop shaped cells appear on blood film
Myelofibrosis
152
Cause of idopathic thrombocytopenic purpura
Autoantibodies form against platelets | Usually seen after a viral illness e.g. Chicken pox
153
Causes of pancytopenia
Marrow infiltration in multiple myeloma Severe megaloblastic anaemia Aplastic anaemia Hypersplenism
154
What is essential thrombocytopenia
Myeloproliferative disorder | Excess production of defective platelets derived from abnormal megakaryocytes in the bone marrow
155
Symptoms of essential thrombocytopenia
Headache Digital ischaemia Abdominal pain secondary to splenic infarction
156
Causes of warm autoimmune haemolytic anaemia | IgG
``` Idiopathic Leukaemia - CLL Lymphomas - non-Hodgkin's lymphoma Other autoimmune diseases - SLE Drug HIV infection ```
157
Causes of cold autoimmune haemolytic anaemia | IgM
``` Idiopathic Epstein-Barr virus and infectious mononucleosis Mycoplasma pneumoniae, non-Hodgkin's lymphoma CLL Mycoplasma pneumoniae ```

MBBS / MBChB finals (25 decks)

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