Haem II Flashcards
Leukaemia likelihood by age
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)
Features of AML
Anaemia Neutropenia Thrombocytopenia Splenomegaly Bone pain
Differential diagnosis of petechiae
Leukaemia Meningococcal septicaemia Vasculitis HSP ITP Non-accidental injury
What is acute lymphoblastic leukaemia
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
Syndrome associated with ALL
Downs syndrome
What does a blood film show in ALL
Blast cells
Genetic mutation associated with CML
Philadelphia chromosome (t(9:22) translocation)
What is CLL
chronic proliferation of a single type of well differentiated lymphocyte, usually B-lymphocytes. This usually affects adults over 55 years of age
Presentation of ALL
Often it is asymptomatic but it can present with infections, anaemia, bleeding and weight loss.
It can cause warm autoimmune haemolytic anaemia.
What is richter’s transformation
CLL can transform into high-grade lymphoma
What does a blood film show in CLL
“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.
Phases of CML
Chronic
Accelerated
Blast
What is the chronic phase of CML
The chronic phase can last around 5 years, is often asymptomatic and patients are diagnosed incidentally with a raised white cell count.
What is the accelerated phase of CML
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.
What is the blast phase of CML
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.
Cytogenic change in CML
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.
Most common acute leukaemia in adults
AML
What might a blood film show in AML
High proportion of blast cells. These blast cells can have rods inside their cytoplasm that are named Auer rods.
Mx of Leukaemia
Chemotherapy and steroids
Radiotherapy
Bone marrow transplant
Surgery
Complications of chemotherapy
Failure Stunted growth and development in children Infections Neurotoxicity Infertility Tumour lysis syndrome
What causes tumour lysis syndrome
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.
Other chemicals released in tumour lysis syndrome
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.
Complications of CLL
Anaemia
Hypogammaglubulinaemia(recurrent infections)
Warm autoimmune haemolytic anaemia
Richter’s transformation
Mx of CML
Imatinib first line(tyrosine kinase inhibitor)
Mx of tumour lysis syndrome
IV allopurinol or IV rasburicase
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
What is monoclonal gammopathy of undetermined significance
excess of a single type of antibody or antibody components without other features of myeloma or cancer
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
What is waldenstrom’s macroglobulinemia
type of smouldering myeloma where there is excessive IgM specifically
Most common type of antibody produced by myeloma
More than 50% of the time this is immunoglobulin type G (IgG)
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.
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
Features of myeloma bone disease
Skull, spine, long bones and ribs commonly affected
Osteolytic lesions - pathological fractures
Vertebral fractures
Hypercalcaemia
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.
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
Risk factors for multiple myeloma
Older age Male Black African ethnicity Family history Obesity
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.
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
Imaging for myeloma
Whole body MRI
Whole body CT
Skeletal survey (xray images of the full skeleton)
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
Mx of myeloma
Chemotherapy first one
Stem cell transplantation
VTE prophylaxis
1st line chemotherapy for multiple myeloma
Bortezomid
Thalidomide
Dexamethasone
Mx of myeloma bone disease
Bisphosphonates
Radiotherapy
Orthopaedic surgery
Cement augmentation
complications of myeloma
Infection Renal failure Anaemia Peripheral neuropathy SCC Hyperviscosity
Presentation of von willebrand disease
Bleeding gums with brushing
Nose bleeds (epistaxis)
Heavy menstrual bleeding (menorrhagia)
Heavy bleeding during surgical operations
Diagnosis of von willebrand disease
Bleeding assessment tools
History
Family history
Mx of von willebrand disease
Only if major bleeding or trauma
Desmopression
VWF infusion
Factor VIII infusion
Mx of VMD in women with heavy periods
Tranexamic acid Mefanamic acid Norethisterone Combined oral contraceptive pill Mirena coil
Hysterectomy in severe cases
What is haemophilia A caused by
Deficiency in factor VIII
What is haemophilia B caused by
Deficiency in factor IX
Inheritance of haemophilia A and B
X linked recessive
Presentation of haemophilia A and B
Abnormal bleeding - gums, GI tract, joints(can lead to damage and deformity)
Haematuria
Mx of haemophilia
Replacement of clotting factors by IV infusion
Desmopressin to stimulate VWF release
Tranexamic acid
Diagnosis of haemophilia
bleeding scores, coagulation factor assays and genetic testing
Signs and symptoms of thalassaemia
Low MCV Fatigue Pallor Jaundice Gallstones Splenomegaly
Diagnosis of thalassaemia
FBC
Haemoglobin electrophoresis
DNA testing
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
Mx of alpha thalassaemia
Monitor FBC
Blood transfusions
Splenectomy
Bone marrow transplant(curative)
Mx of beta thalassamia
Regular transfusions
Iron chelation
Splenectomy
Bone marrow transplant
Complications of sickle cell anaemia
Anaemia Stroke Avascular necrosis Pulmonary HTN Priapism
Renal complication of sickle cell anaemia
CKD
Neurological complication of sickle cell anaemia
Stroke
Mx of sickle cell anaemia
Avoid dehydration Up to date vaccinations Antibiotic prophylaxis(Pen V) Blood transfusion Bone marrow transplant
Mx of sickle crises
Admission Treat infection Keep warm IV fluids Simple analgesia Penile aspiration in priapism
Presentation of splenic sequestration crisis
Acutely enlarged and painful spleen –> severe anaemia and circulatory collapse
Mx of splenic sequestration crisis
supportive with blood transfusions and fluid resuscitation to treat anaemia and shock
Splenectomy in recurrent crises
what can trigger aplastic crisis in sickle cell anaemia
Parvovirus B19
Leads to significant anaemia
Diagnosis of acute chest syndrome in sickle cell anaemia
Fever or respiratory symptoms with
New infiltrates seen on a chest xray
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
