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Haem II Flashcards

1
Q

Leukaemia likelihood by age

A

ALL CeLLmates have CoMmon AMbitions

Under 5 and over 45 – acute lymphoblastic leukaemia (ALL)

Over 55 – chronic lymphocytic leukaemia (CeLLmates)

Over 65 – chronic myeloid leukaemia (CoMmon)

Over 75 – acute myeloid leukaemia (AMbitions)

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2
Q

Features of AML

A 
Anaemia 
Neutropenia 
Thrombocytopenia 
Splenomegaly 
Bone pain
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3
Q

Differential diagnosis of petechiae

A 
Leukaemia
Meningococcal septicaemia
Vasculitis
HSP
ITP
Non-accidental injury
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4
Q

What is acute lymphoblastic leukaemia

A

Acute proliferation of a single type of lymphocyte, usually B-lymphocytes

Excessive proliferation of these cells causes them to replace the other cell types being created in the bone marrow, leading to a pancytopenia

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5
Q

Syndrome associated with ALL

A

Downs syndrome

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6
Q

What does a blood film show in ALL

A

Blast cells

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7
Q

Genetic mutation associated with CML

A

Philadelphia chromosome (t(9:22) translocation)

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8
Q

What is CLL

A

chronic proliferation of a single type of well differentiated lymphocyte, usually B-lymphocytes. This usually affects adults over 55 years of age

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9
Q

Presentation of ALL

A

Often it is asymptomatic but it can present with infections, anaemia, bleeding and weight loss.

It can cause warm autoimmune haemolytic anaemia.

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10
Q

What is richter’s transformation

A

CLL can transform into high-grade lymphoma

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11
Q

What does a blood film show in CLL

A

“smear” or “smudge” cells. These occur during the process of preparing the blood film where aged or fragile white blood cells rupture and leave a smudge on the film.

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12
Q

Phases of CML

A

Chronic
Accelerated
Blast

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13
Q

What is the chronic phase of CML

A

The chronic phase can last around 5 years, is often asymptomatic and patients are diagnosed incidentally with a raised white cell count.

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14
Q

What is the accelerated phase of CML

A

Abnormal blast cells take up a high proportion of the cells in the bone marrow and blood (10-20%).

In the accelerated phase patients become more symptomatic, develop anaemia and thrombocytopenia and become immunocompromised.

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15
Q

What is the blast phase of CML

A

Follows the accelerated phase and involves an even high proportion of blast cells and blood (>30%).

This phase has severe symptoms and pancytopenia. It is often fatal.

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16
Q

Cytogenic change in CML

A

The cytogenetic change that is characteristic of CML is the Philadelphia chromosome, which is a translocation of genes between chromosome 9 and 22: it is a t(9:22) translocation.

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17
Q

Most common acute leukaemia in adults

A

AML

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18
Q

What might a blood film show in AML

A

High proportion of blast cells. These blast cells can have rods inside their cytoplasm that are named Auer rods.

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19
Q

Mx of Leukaemia

A

Chemotherapy and steroids

Radiotherapy
Bone marrow transplant
Surgery

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20
Q

Complications of chemotherapy

A 
Failure 
Stunted growth and development in children 
Infections 
Neurotoxicity 
Infertility 
Tumour lysis syndrome
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21
Q

What causes tumour lysis syndrome

A

Caused by the release of uric acid from cells that are being destroyed by chemotherapy

The uric acid can form crystals in the interstitial tissue and tubules of the kidneys and causes acute kidney injury.

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22
Q

Other chemicals released in tumour lysis syndrome

A

Potassium and phosphate are also released so these need to be monitored and treated appropriately.

High phosphate can lead to low calcium, which can have an adverse effect, so calcium is also monitored.

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23
Q

Complications of CLL

A

Anaemia
Hypogammaglubulinaemia(recurrent infections)
Warm autoimmune haemolytic anaemia
Richter’s transformation

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24
Q

Mx of CML

A

Imatinib first line(tyrosine kinase inhibitor)

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25
Mx of tumour lysis syndrome
IV allopurinol or IV rasburicase
26
What is myeloma
cancer of the plasma cells. These are a type of B lymphocyte that produce antibodies Large quantities of a single type of antibody produced
27
What is monoclonal gammopathy of undetermined significance
excess of a single type of antibody or antibody components without other features of myeloma or cancer
28
What is smouldering myeloma
progression of MGUS with higher levels of antibodies or antibody components. It is premalignant and more likely to progress to myeloma than MGUS
29
What is waldenstrom's macroglobulinemia
type of smouldering myeloma where there is excessive IgM specifically
30
Most common type of antibody produced by myeloma
More than 50% of the time this is immunoglobulin type G (IgG)
31
What is the bence jones protein
can be found in the urine of many patients with myeloma and is actually a part (subunit) of the antibody called the light chains.
32
Why is there anaemia in myeloma
plasma cells invade the bone marrow(infiltration) and cause suppression of development of other blood cell lines --> anaemia, neutropenia and thrombocytopenia
33
Features of myeloma bone disease
Skull, spine, long bones and ribs commonly affected Osteolytic lesions - pathological fractures Vertebral fractures Hypercalcaemia
34
What are plasmacytomas
individual tumours made up of the cancerous plasma cells. They can occur in the bones, replacing normal bone tissue or can occur outside bones in the soft tissue of the body.
35
Features of myeloma renal disease
High levels of immunoglobulins (antibodies) can block the flow through the tubules Hypercalcaemia impairs renal function Dehydration Medications used to treat the conditions such as bisphosphonates can be harmful to the kidneys
36
Risk factors for multiple myeloma
``` Older age Male Black African ethnicity Family history Obesity ```
37
Initial ix in multiple myeloma
``` FBC (low white blood cell count in myeloma) Calcium (raised in myeloma) ESR (raised in myeloma) Plasma viscosity (raised in myeloma) ``` urgent serum protein electrophoresis and a urine Bence-Jones protein test.
38
Testing specifically for myeloma - BLIP
B – Bence–Jones protein (request urine electrophoresis) L – Serum‑free Light‑chain assay I – Serum Immunoglobulins P – Serum Protein electrophoresis
39
Imaging for myeloma
Whole body MRI Whole body CT Skeletal survey (xray images of the full skeleton)
40
X-ray signs in myeloma
Punched out lesions Lytic lesions “Raindrop skull” caused by many punched out (lytic) lesions throughout the skull that give the appearance of raindrops splashing on a surface
41
Mx of myeloma
Chemotherapy first one Stem cell transplantation VTE prophylaxis
42
1st line chemotherapy for multiple myeloma
Bortezomid Thalidomide Dexamethasone
43
Mx of myeloma bone disease
Bisphosphonates Radiotherapy Orthopaedic surgery Cement augmentation
44
complications of myeloma
``` Infection Renal failure Anaemia Peripheral neuropathy SCC Hyperviscosity ```
45
Presentation of von willebrand disease
Bleeding gums with brushing Nose bleeds (epistaxis) Heavy menstrual bleeding (menorrhagia) Heavy bleeding during surgical operations
46
Diagnosis of von willebrand disease
Bleeding assessment tools History Family history
47
Mx of von willebrand disease
Only if major bleeding or trauma Desmopression VWF infusion Factor VIII infusion
48
Mx of VMD in women with heavy periods
``` Tranexamic acid Mefanamic acid Norethisterone Combined oral contraceptive pill Mirena coil ``` Hysterectomy in severe cases
49
What is haemophilia A caused by
Deficiency in factor VIII
50
What is haemophilia B caused by
Deficiency in factor IX
51
Inheritance of haemophilia A and B
X linked recessive
52
Presentation of haemophilia A and B
Abnormal bleeding - gums, GI tract, joints(can lead to damage and deformity) Haematuria
53
Mx of haemophilia
Replacement of clotting factors by IV infusion Desmopressin to stimulate VWF release Tranexamic acid
54
Diagnosis of haemophilia
bleeding scores, coagulation factor assays and genetic testing
55
Signs and symptoms of thalassaemia
``` Low MCV Fatigue Pallor Jaundice Gallstones Splenomegaly ```
56
Diagnosis of thalassaemia
FBC Haemoglobin electrophoresis DNA testing
57
What needs to be monitored in thalassaemia
Serum ferritin - iron overload due to faulty RBCs, recurrent transfusions and increased absorption of iron in response to anaemia
58
Mx of alpha thalassaemia
Monitor FBC Blood transfusions Splenectomy Bone marrow transplant(curative)
59
Mx of beta thalassamia
Regular transfusions Iron chelation Splenectomy Bone marrow transplant
60
Complications of sickle cell anaemia
``` Anaemia Stroke Avascular necrosis Pulmonary HTN Priapism ```
61
Renal complication of sickle cell anaemia
CKD
62
Neurological complication of sickle cell anaemia
Stroke
63
Mx of sickle cell anaemia
``` Avoid dehydration Up to date vaccinations Antibiotic prophylaxis(Pen V) Blood transfusion Bone marrow transplant ```
64
Mx of sickle crises
``` Admission Treat infection Keep warm IV fluids Simple analgesia Penile aspiration in priapism ```
65
Presentation of splenic sequestration crisis
Acutely enlarged and painful spleen --> severe anaemia and circulatory collapse
66
Mx of splenic sequestration crisis
supportive with blood transfusions and fluid resuscitation to treat anaemia and shock Splenectomy in recurrent crises
67
what can trigger aplastic crisis in sickle cell anaemia
Parvovirus B19 Leads to significant anaemia
68
Diagnosis of acute chest syndrome in sickle cell anaemia
Fever or respiratory symptoms with | New infiltrates seen on a chest xray
69
Mx of acute chest syndrome in sickle cell anaemia
Antibiotics or antivirals for infections Blood transfusions for anaemia Incentive spirometry using a machine that encourages effective and deep breathing Artificial ventilation with NIV or intubation may be required

General practice (26 decks)

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